Scott Ceresnak

All Publications

• Multicenter Study of Survival Benefit of Cardiac Resynchronization Therapy in Pediatric and Congenital Heart Disease. JACC. Clinical electrophysiology Chubb, H., Mah, D. Y., Shah, M., Lin, K. Y., Peng, D. M., Hale, B. W., May, L., Etheridge, S., Goodyer, W., Ceresnak, S. R., Motonaga, K. S., Rosenthal, D. N., Almond, C. S., McElhinney, D. B., Dubin, A. M. 2023

  Abstract

  BACKGROUND: Evidence for the efficacy of cardiac resynchronization therapy (CRT) in pediatric and congenital heart disease (CHD) has been limited to surrogate outcomes.OBJECTIVES: This study aimed to assess the impact of CRT upon the risk of transplantation or death in a retrospective, high-risk, controlled cohort at 5 quaternary referral centers.METHODS: Both CRT patients and control patients were<21 years of age or had CHD; had systemic ventricular ejection fraction<45%; symptomatic heart failure; and significant electrical dyssynchrony (QRS duration z score >3 or single-site ventricular pacing >40%) at enrollment. Patients with CRT were matched with control patients via 1:1 propensity score matching. CRT patients were enrolled at CRT implantation; control patients were enrolled at the outpatient clinical encounter where inclusion criteria were first met. The primary endpoint was transplantation or death.RESULTS: In total, 324 control patients and 167 CRT recipients were identified. Mean follow-up was 4.2 ± 3.7 years. Upon propensity score matching, 139 closely matched pairs were identified (20 baseline indices). Of the 139 matched pairs, 52 (37.0%) control patients and 31 (22.0%) CRT recipients reached the primary endpoint. On both unadjusted and multivariable Cox regression analysis, the risk reduction associated with CRT for the primary endpoint was significant (HR: 0.40; 95%CI: 0.25-0.64; P< 0.001; and HR: 0.44; 95%CI: 0.28-0.71; P=0.001, respectively). On longitudinal assessment, the CRT group had significantly improved systemic ventricular ejection fraction (P< 0.001) and shorter QRS duration (P=0.015), sustained to 5 years.CONCLUSIONS: In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplantation-free survival.

  View details for DOI 10.1016/j.jacep.2023.11.008

  View details for PubMedID 38206260

• Utility of smart watches for identifying arrhythmias in children. Communications medicine Zahedivash, A., Chubb, H., Giacone, H., Boramanand, N. K., Dubin, A. M., Trela, A., Lencioni, E., Motonaga, K. S., Goodyer, W., Navarre, B., Ravi, V., Schmiedmayer, P., Bikia, V., Aalami, O., Ling, X. B., Perez, M., Ceresnak, S. R. 2023; 3 (1): 167

  Abstract

  Arrhythmia symptoms are frequent complaints in children and often require a pediatric cardiology evaluation. Data regarding the clinical utility of wearable technologies are limited in children. We hypothesize that an Apple Watch can capture arrhythmias in children.We present an analysis of patients ≤18 years-of-age who had signs of an arrhythmia documented by an Apple Watch. We include patients evaluated at our center over a 4-year-period and highlight those receiving a formal arrhythmia diagnosis. We evaluate the role of the Apple Watch in arrhythmia diagnosis, the results of other ambulatory cardiac monitoring studies, and findings of any EP studies.We identify 145 electronic-medical-record identifications of Apple Watch, and find arrhythmias confirmed in 41 patients (28%) [mean age 13.8 ± 3.2 years]. The arrythmias include: 36 SVT (88%), 3 VT (7%), 1 heart block (2.5%) and wide 1 complex tachycardia (2.5%). We show that invasive EP study confirmed diagnosis in 34 of the 36 patients (94%) with SVT (2 non-inducible). We find that the Apple Watch helped prompt a workup resulting in a new arrhythmia diagnosis for 29 patients (71%). We note traditional ambulatory cardiac monitors were worn by 35 patients (85%), which did not detect arrhythmias in 10 patients (29%). In 73 patients who used an Apple Watch for recreational or self-directed heart rate monitoring, 18 (25%) sought care due to device findings without any arrhythmias identified.We demonstrate that the Apple Watch can record arrhythmia events in children, including events not identified on traditionally used ambulatory monitors.

  View details for DOI 10.1038/s43856-023-00392-9

  View details for PubMedID 38092993

  View details for PubMedCentralID 4937287

• Correction: Exploring the feasibility of using long-term stored newborn dried blood spots to identify metabolic features for congenital heart disease screening. Biomarker research Ceresnak, S. R., Zhang, Y., Ling, X. B., Su, K. J., Tang, Q., Jin, B., Schilling, J., Chou, C. J., Han, Z., Floyd, B. J., Whitin, J. C., Hwa, K. Y., Sylvester, K. G., Chubb, H., Luo, R. Y., Tian, L., Cohen, H. J., McElhinney, D. B. 2023; 11 (1): 101

  View details for DOI 10.1186/s40364-023-00546-w

  View details for PubMedID 37993911

• Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation. Circulation. Arrhythmia and electrophysiology Giacone, H. M., Chubb, H., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R., Goodyer, W. R., Hanish, D., Trela, A. V., Boramanand, N., Lencioni, E., Boothroyd, D., Graber-Naidich, A., Wright, G., Haeffele, C., Hollander, S. A., McElhinney, D. B., Ma, M., Hanley, F. L., Chen, S. 2023: e011143

  Abstract

  With the advent of more intensive rhythm monitoring strategies, ventricular arrhythmias (VAs) are increasingly detected in Fontan patients. However, the prognostic implications of VA are poorly understood. We assessed the incidence of VA in Fontan patients and the implications on transplant-free survival.Medical records of Fontan patients seen at a single center between 2002 and 2019 were reviewed to identify post-Fontan VA (nonsustained ventricular tachycardia >4 beats or sustained >30 seconds). Patients with preFontan VA were excluded. Hemodynamically unstable VA was defined as malignant VA. The primary outcome was death or heart transplantation. Death with censoring at transplant was a secondary outcome.Of 431 Fontan patients, transplant-free survival was 82% at 15 years post-Fontan with 64 (15%) meeting primary outcome of either death (n=16, 3.7%), at a median 4.6 (0.4-10.2) years post-Fontan, or transplant (n=48, 11%), at a median of 11.1 (5.9-16.2) years post-Fontan. Forty-eight (11%) patients were diagnosed with VA (90% nonsustained ventricular tachycardia, 10% sustained ventricular tachycardia). Malignant VA (n=9, 2.0%) was associated with younger age, worse systolic function, and valvular regurgitation. Risk for VA increased with time from Fontan, 2.4% at 10 years to 19% at 20 years. History of Stage 1 surgery with right ventricular to pulmonary artery conduit and older age at Fontan were significant risk factors for VA. VA was strongly associated with an increased risk of transplant or death (HR, 9.2 [95% CI, 4.5-18.7]; P<0.001), with a transplant-free survival of 48% at 5-year post-VA diagnosis.Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis. Risk factors for VA included older age at Fontan and history of right ventricular to pulmonary artery conduit. A diagnosis of VA in Fontan patients should prompt increased clinical surveillance.

  View details for DOI 10.1161/CIRCEP.122.011143

  View details for PubMedID 37254747

• UTILITY OF THE APPLE WATCH (R) FOR IDENTIFYING ARRHYTHMIAS IN CHILDREN Zahedivash, A., Chubb, H., Giacone, H., Boramanand, N., Dubin, A., Trela, A., Lencioni, E., Motonaga, K., Goodyer, W., Ceresnak, S. R. ELSEVIER SCIENCE INC. 2023: 1563

  View details for Web of Science ID 000990866101575

• Ventricular arrhythmias following transcatheter pulmonary valve replacement with the harmony TPV25 device. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Taylor, A., Yang, J., Dubin, A., Chubb, M. H., Motonaga, K., Goodyer, W., Giacone, H., Peng, L., Romfh, A., McElhinney, D., Ceresnak, S. 2022

  Abstract

  BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Harmony valve (Medtronic, Inc.) was recently approved to treat postoperative native outflow tract pulmonary regurgitation. While the 22mm Harmony valve Early Feasibility Study demonstrated ventricular tachycardia (VT) in only 5% of patients, little is known about ventricular arrhythmias after TPVR with the larger 25mm valve (TPV25).METHODS: A single center review was performed of patients with TPV25 implant from 2020 to 2021. Demographic, cardiac, procedural, and postimplant cardiac telemetry data were collected and compared between patients who did and did not have peri-implant ventricular arrhythmia.RESULTS: Thirty patients underwent TPV25 at a median age of 30 years. On postimplant telemetry, VT events were documented in 12 patients (40%); 11 nonsustained VT (NSVT) (median 3 episodes per patient and 6 beats per episode, maximum 157 episodes) and 1 sustained VT (3%), with Torsades de Pointes secondary to a short coupled premature ventricular contraction (PVC). VT events were associated with annular valve positioning (p<0.001) and increased postimplant PVC burden (p<0.0001), but there was no association between VT and other demongraphic, historical, or procedural factors. The frequency of NSVT events fell from 3/hfrom 0 to 12h postimplant to 0.5/hr from 12 to 24h (p<0.001).CONCLUSION: VT occurred commonly (40%) in the first 24h after TPV25 implant, with self-limited NSVT in 11 of 12 patients and 1 patient with cardiac arrest secondary to Torsades de Pointes. VT only occurred with annular valve positioning. Larger, longer-term studies are needed to determine risk factors for and natural history of post-TPVR VT.

  View details for DOI 10.1002/ccd.30393

  View details for PubMedID 36198126

• Pectoral Nerve Blocks Decrease Postoperative Pain and Opioid Use After Pacemaker or Implantable Cardioverter-Defibrillator Placement in Children. Heart rhythm Yang, J. K., Char, D. S., Motonaga, K. S., Navaratnam, M., Dubin, A. M., Trela, A., Hanisch, D. G., McFadyen, G., Chubb, H., Goodyer, W., Ceresnak, S. R. 2020

  Abstract

  BACKGROUND: Pectoral nerve blocks (PECS) can reduce intra-procedural anesthetic requirements and postoperative pain. Little is known on the utility of PECS in reducing pain and narcotic use after pacemaker (PM) or implantable cardioverter defibrillator (ICD) placement in children.OBJECTIVE: To determine whether PECS can decrease postoperative pain and opioid use after PM or ICD placement in children.METHODS: A single center, retrospective review of pediatric patients undergoing transvenous PM or ICD placement between 2015-2020 was performed. Patients with recent cardiothoracic surgery or neurologic/developmental deficits were excluded. Demographics, procedural variables, postoperative pain, and postoperative opioid usage were compared between patients who underwent PECS and those who underwent conventional local anesthetic (CONTROL).RESULTS: A total of 74 patients underwent PM or ICD placement with 20 patients (27%) undergoing PECS. There were no differences between PECS and CONTROL with regard to age, weight, gender, type of device placed, presence of congenital heart disease, type of anesthesia, procedural time or complication rates. Patients who underwent PECS had lower pain scores at 1, 2, 6, 18, and 24-hours compared to CONTROL. PECS patients had a lower mean cumulative pain score [PECS 1.5 (95%-CI 0.8-2.2) vs CONTROL 3.1 (95%-CI 2.7-3.5); P<0.001] and lower total opioid use [PECS 6.0 MME/m2 (95%-CI 3.4-8.6) vs CONTROL 15.0 MME/m2 (95%-CI 11.8-18.2); P=0.001] over the 24-hours post-implant.CONCLUSIONS: Pectoralis nerve blocks reduce postoperative pain scores and lower total opioid usage after ICD or PM placement. PECS should be considered at the time of transvenous device placement in children.

  View details for DOI 10.1016/j.hrthm.2020.03.009

  View details for PubMedID 32201270

• A proposed approach to the asymptomatic pediatric patient with Wolff-Parkinson-White pattern. HeartRhythm case reports Chubb, H., Ceresnak, S. R. 2020; 6 (1): 2–7

  View details for DOI 10.1016/j.hrcr.2019.09.003

  View details for PubMedID 31956492

• Loss of Ventricular Pre-excitation During Non-invasive Testing Does Not Exclude High-Risk Accessory Pathways: A Multicenter Study of WPW in Children. Heart rhythm Escudero, C. A., Ceresnak, S. R., Collins, K. K., Pass, R. H., Aziz, P. F., Blaufox, A. D., Ortega, M. C., Cannon, B. C., Cohen, M. n., Dechert, B. E., Dubin, A. M., Motonaga, K. S., Epstein, M. R., Erickson, C. C., Fishberger, S. B., Gates, G. J., Capone, C. A., Nappo, L. n., Kertesz, N. J., Kim, J. J., Valdes, S. O., Kubuš, P. n., Law, I. H., Maldonado, J. n., Moore, J. P., Perry, J. C., Sanatani, S. n., Seslar, S. P., Shetty, I. n., Zimmerman, F. J., Skinner, J. R., Marcondes, L. n., Stephenson, E. A., Asakai, H. n., Tanel, R. E., Uzun, O. n., Etheridge, S. P., Janson, C. n. 2020

  Abstract

  Abrupt loss of ventricular pre-excitation on non-invasive evaluation, or non-persistent pre-excitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.To compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted pre-excited atrial fibrillation (RC-AF) in patients with non-persistent and persistent pre-excitation.Patients ≤21 years with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Non-persistent pre-excitation was defined as absence/sudden loss of pre-excitation on ECG, Holter, or exercise test. RC-AF was defined as clinical pre-excited atrial fibrillation with shortest pre-excited R-R interval (SPERRI) ≤250ms. AP effective refractory period (APERP), SPERRI at EPS (EPS-SPERRI), and shortest pre-excited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤250ms.Of 1589 patients, 244 (15%) had non-persistent pre-excitation and 1345 (85%) had persistent pre-excitation. There were no differences in sex (58 vs 60% male, p=0.49) or age (13.3±3.6 vs 13.1±3.9 years, p=0.43) between groups. Though APERP (344±76 vs 312±61ms, p<0.001), and SPPCL (394±123 vs 317±82ms, p<0.001) were longer in non-persistent versus persistent pre-excitation, there was no difference in EPS-SPERRI (331±71 vs 316±73ms, p=0.15). Non-persistent pre-excitation was associated with fewer high-risk APs (13 vs 23%, p<0.001) than persistent pre-excitation. Of 61 patients with SCA or RC-AF, 6 (10%) had non-persistent pre-excitation (3 SCA, 3 RC-AF).Non-persistent pre-excitation was associated with fewer high-risk APs, though it did not exclude risk of SCA or RC-AF in children with WPW.

  View details for DOI 10.1016/j.hrthm.2020.05.035

  View details for PubMedID 32497761

• Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome A Multicenter International Study JACC-CLINICAL ELECTROPHYSIOLOGY Etheridge, S. P., Escudero, C. A., Blaufox, A. D., Law, I. H., Dechert-Crooks, B. E., Stephenson, E. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Skinner, J. R., Marcondes, L. D., Perry, J. C., Collins, K. K., Seslar, S. P., Cabrera, M., Uzun, O., Cannon, B. C., Aziz, P. F., Kubu, P., Tanel, R. E., Valdes, S. O., Sami, S., Kertesz, N. J., Maldonado, J., Erickson, C., Moore, J. P., Asakai, H., Mill, L., Abcede, M., Spector, Z. Z., Menon, S., Shwayder, M., Bradley, D. J., Cohen, M., Sanatani, S. 2018; 4 (4): 433–44

  View details for DOI 10.1016/j.jacep.2017.10.009

  View details for Web of Science ID 000602285700002

• Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy HEART RHYTHM Chen, S., Motonaga, K. S., Hollander, S. A., Almond, C. S., Rosenthal, D. N., Kaufman, B. D., May, L. J., Avasarala, K., Dao, D. T., Dubin, A. M., Ceresnak, S. R. 2016; 13 (6): 1289-1296

  Abstract

  Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined.We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM.A single-center retrospective review of children with DCM was performed. LTEs were defined as documented ventricular tachycardia or fibrillation requiring medical intervention. Three pediatric cardiologists, blinded to clinical events, evaluated ECGs obtained at the time of initial referral. Kaplan-Meier survival and Cox proportional hazards analyses were used to evaluate time to LTEs.A total of 137 patients (mean age 7.8 ± 6.7 years; 75(55%) male patients) with DCM (mean ejection fraction 35% ± 16%) were included; 67 patients (49%) had a corrected JT (JTc) interval of ≥340 ms, 72 (53%) had a corrected QT (QTc) interval of ≥450 ms, and 41 (30%) had abnormal T waves. LTEs occurred in 15 patients at a median of 12 months (interquartile range 3-36 months) after the initial ECG. Patients with LTEs had a longer JTc interval (371 ± 77 ms vs 342 ± 41 ms; P = .02) and a longer QTc interval (488 ± 96 ms vs 453 ± 44 ms; P = .01). In survival analysis, a JTc interval of ≥390 ms (hazard ratio [HR] 4.07; 95% confidence interval [CI] 1.12-14.83; P = .03), a QTc interval of ≥510 ms (HR 6.95; 95% CI 1.53-31.49; P = .01), abnormal T-wave inversion (HR 11.62; 95% CI 2.75-49.00; P = .001), and ST-segment depression (HR 6.91; 95% CI 1.25-38.27; P = .03) were associated with an increased risk of LTEs, even after adjusting for QRS duration and amiodarone use.Repolarization abnormalities are common in children with DCM. Certain ECG repolarization abnormalities, such as significantly prolonged JTc and QTc intervals, may be useful in identifying patients at risk of LTEs.

  View details for DOI 10.1016/j.hrthm.2016.02.014

  View details for Web of Science ID 000376334800016

  View details for PubMedID 26945851

• Pediatric Cardiology Boot Camp: Description and Evaluation of a Novel Intensive Training Program for Pediatric Cardiology Trainees PEDIATRIC CARDIOLOGY Ceresnak, S. R., Axelrod, D. M., Motonaga, K. S., Johnson, E. R., Krawczeski, C. D. 2016; 37 (5): 834-844

  Abstract

  The transition from residency to subspecialty fellowship in a procedurally driven field such as pediatric cardiology is challenging for trainees. We describe and assess the educational value of a pediatric cardiology "boot camp" educational tool designed to help prepare trainees for cardiology fellowship. A two-day intensive training program was provided for pediatric cardiology fellows in July 2015 at a large fellowship training program. Hands-on experiences and simulations were provided in: anatomy, auscultation, echocardiography, catheterization, cardiovascular intensive care (CVICU), electrophysiology (EP), heart failure, and cardiac surgery. Knowledge-based exams as well as surveys were completed by each participant pre-training and post-training. Pre- and post-exam results were compared via paired t tests, and survey results were compared via Wilcoxon rank sum. A total of eight participants were included. After boot camp, there was a significant improvement between pre- and post-exam scores (PRE 54 ± 9 % vs. POST 85 ± 8 %; p ≤ 0.001). On pre-training survey, the most common concerns about starting fellowship included: CVICU emergencies, technical aspects of the catheterization/EP labs, using temporary and permanent pacemakers/implantable cardiac defibrillators (ICDs), and ECG interpretation. Comparing pre- and post-surveys, there was a statistically significant improvement in the participants comfort level in 33 of 36 (92 %) areas of assessment. All participants (8/8, 100 %) strongly agreed that the boot camp was a valuable learning experience and helped to alleviate anxieties about the start of fellowship. A pediatric cardiology boot camp experience at the start of cardiology fellowship can provide a strong foundation and serve as an educational springboard for pediatric cardiology fellows.

  View details for DOI 10.1007/s00246-016-1357-z

  View details for Web of Science ID 000377722400005

  View details for PubMedID 26961569

• The Benefits of 3D Mapping Systems for Ablation of WPW in Children. Journal of cardiovascular electrophysiology Ceresnak, S. R., Pass, R. H. 2015; 26 (10): E10-1

  View details for DOI 10.1111/jce.12706

  View details for PubMedID 25952689

• 50 is the new 70: Short ventriculoatrial times are common in children with atrioventricular reciprocating tachycardia. Heart rhythm Ceresnak, S. R., Doan, L. N., Motonaga, K. S., Avasarala, K., Trela, A. V., Reddy, C. D., Dubin, A. M. 2015; 12 (7): 1541-1547

  Abstract

  One of the basic electrophysiological principles of atrioventricular reciprocating tachycardia (AVRT) is that ventriculoatrial (VA) times during tachycardia are >70 ms. We hypothesized, however, that children may commonly have VA times <70 ms in AVRT.This study sought to determine the incidence and characteristics associated with short-VA AVRT in children.A retrospective single-center review of children with AVRT from 2000 to 2014 was performed. All patients ≤18 years of age with AVRT at electrophysiology study were included. Patients with persistent junctional reciprocating tachycardia, atrioventricular nodal reentry tachycardia, and tachycardia not unequivocally proven to be AVRT were excluded. VA time was defined as the time between earliest ventricular activation and earliest atrial activation in any lead and was confirmed by 2 electrophysiologists. Patients with VA times <70 ms (SHORT-VA) and those with standard VA times ≥70 ms (STD-VA) were compared. Logistic regression analysis identified characteristics of SHORT-VA patients.A total of 495 patients with AVRT were included (mean age 11.7 ± 4.1 years). There were 265 patients (54%) with concealed accessory pathways (APs) and 230 (46%) with Wolff-Parkinson-White syndrome. AP location was left-sided in 301 patients (61%) and right-sided in 194 (39%). The mean VA time in AVRT was 100 ± 33 ms. A total of 63 patients (13%) had VA times <70 ms (SHORT-VA). The shortest VA time during AVRT was 50 ms. There was no difference in age, AV nodal block cycle, or body surface area between SHORT-VA and STD-VA patients, but SHORT-VA patients had lower weight (43 ± 17 vs 51 ± 23 kg, P = .02), lower AV nodal effective refractory period (AVNERP; 269 ± 50 vs 245 ± 52 ms, P < .01), and more left-sided APs (50 [79%] vs 251 [58%]; P < .01]. On multivariate logistic regression, factors associated with SHORT-VA included left-sided AP (odds ratio [OR] 5.79, confidence interval [95% CI] 2.21-15.1, P < .01), shorter AVNERP (OR 0.99, CI 0.98-0.99, P < .01), and lower weight (OR 0.97, CI 0.95-0.99, P < .01).Children with AVRT can frequently have VA times <70 ms, with 50 ms being the shortest VA time. This finding debunks the classic electrophysiology principle that VA times in AVRT must be >70 ms. SHORT-VA AVRT was more common in children with left-sided APs.

  View details for DOI 10.1016/j.hrthm.2015.03.047

  View details for PubMedID 25828598

• Wolff-Parkinson-White syndrome (WPW) and athletes: Darwin at play? JOURNAL OF ELECTROCARDIOLOGY Ceresnak, S. R., Dubin, A. M. 2015; 48 (3): 356-361

  View details for DOI 10.1016/j.jelectrocard.2014.12.019

  View details for Web of Science ID 000354343100012

  View details for PubMedID 25722079

• Success Rates in Pediatric WPW Ablation Are Improved with 3-Dimensional Mapping Systems Compared with Fluoroscopy Alone: A Multicenter Study JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY Ceresnak, S. R., Dubin, A. M., Kim, J. J., Valdes, S. O., Fishberger, S. B., Shetty, I., Zimmerman, F., Tanel, R. E., Epstein, M. R., Motonaga, K. S., Capone, C. A., Nappo, L., Gates, G. J., Pass, R. H. 2015; 26 (4): 412-416

  Abstract

  Three-dimensional mapping (3-D) systems are frequently used for ablation of supraventricular tachycardia. Prior studies have demonstrated radiation dosage reduction with 3-D, but there are no data on whether 3-D improves the efficacy of ablation of Wolff-Parkinson-White syndrome (WPW). We sought to determine if 3-D improves the success rate for ablation of WPW in children.Multicenter retrospective study including patients ≤21 years of age with WPW undergoing ablation from 2008 to 2012. Success rates using the 2 techniques (3-D vs. fluoroscopy alone [FLUORO]) were compared.Six hundred and fifty-one cases were included (58% male, mean age 13 ± 4 years, 366 [56%] 3-D). Baseline characteristics including gender, weight, accessory pathway (AP) location, number of APs, and repeat ablation attempts were similar between the 2 groups (3-D and FLUORO) The 3-D group was slightly younger (12.7 ± 4.0 vs. 13.3 ± 4.0 years; P = 0.04) and less likely to undergo ablation utilizing cryoenergy (38 [10%] vs. 56 [20%]; P < 0.01). The 3-D group had a higher acute success rate of ablation (355 [97%] vs. 260 [91%]; P < 0.01). No differences were seen in recurrence (16 [5%] vs. 26 [9%]; P = 0.09) or complication rates (1 [0.3%] vs. 1 [0.4%]; P = 0.86) between the groups. On multivariable analysis, 3-D was shown to significantly improve success at ablation with an odds ratio of 3.1 (95% CI 1.44-6.72; P < 0.01).Use of 3-D significantly improved success rates for ablation of WPW in children. The increase in acute success associated with 3-D suggests it is an important adjunct for catheter ablation of WPW in children.

  View details for DOI 10.1111/jce.12623

  View details for Web of Science ID 000352814300012

  View details for PubMedID 25600208

• Novel method of signal analysis for ablation of Wolff-Parkinson-White syndrome HEART RHYTHM Ceresnak, S. R., Gates, G. J., Nappo, L., Cohen, H. W., Pass, R. H. 2012; 9 (1): 2-7

  Abstract

  Identification of the site of successful radiofrequency catheter ablation (RFCA) for Wolff-Parkinson-White (WPW) syndrome may be subjective.The purpose of this study was to develop an automated signal analysis program to predict a successful ablation site.Patients who underwent successful RFCA for WPW from 2008- 2010 at our center were analyzed. Inclusion criteria were age <21 years, loss of preexcitation in <5 seconds, and sustained success at 3 months. Exclusion criteria were congenital heart disease and pacing during RFCA. The standard recording system signal was filtered into low frequency (LF 0-≤0.02 Hz) and high frequency (HF >0.02-≤0.45 Hz). Software identified the beginning of the HF signal, LF and R-wave peaks, LF/HF signal amplitude, and area under the HF/LF signals. Successful and unsuccessful (radiofrequency energy applied without accessory pathway block) signals were compared.Thirty patients were analyzed; 16 had both successful and unsuccessful signals, and 14 had a successful ablation with 1 radiofrequency application. Mean age was 13.7 ± 3.1 years, weight 54.9 ± 22.4 kg, and time to accessory pathway ablation 1.7 ± 1.4 seconds. Significant differences were found between successful and unsuccessful signals in area under HF signal, LF amplitude, LF to R time, HF ratio, and HF area × HF ratio. A receiver operating curve of HF area × HF ratio produced an area under the curve of 0.89. An HF area × HF ratio of 3.1 distinguished successful from unsuccessful signals with 100% specificity and 81% sensitivity.Automated signal analysis retrospectively differentiated successful from unsuccessful signals in patients undergoing RFCA for WPW. This software may improve the safety and efficacy of RFCA in children.

  View details for DOI 10.1016/j.hrthm.2011.08.022

  View details for Web of Science ID 000298640400002

  View details for PubMedID 21872561

• Global metabolomics revealed deviations from the metabolic aging clock in colorectal cancer patients. Theranostics Zhang, L., Mo, S., Zhu, X., Chou, C. J., Jin, B., Han, Z., Schilling, J., Liao, W., Thyparambil, S., Luo, R. Y., Whitin, J. C., Tian, L., Nagpal, S., Ceresnak, S. R., Cohen, H. J., McElhinney, D. B., Sylvester, K. G., Gong, Y., Fu, C., Ling, X. B., Peng, J. 2024; 14 (4): 1602-1614

  Abstract

  Background: Markers of aging hold promise in the context of colorectal cancer (CRC) care. Utilizing high-resolution metabolomic profiling, we can unveil distinctive age-related patterns that have the potential to predict early CRC development. Our study aims to unearth a panel of aging markers and delve into the metabolomic alterations associated with aging and CRC. Methods: We assembled a serum cohort comprising 5,649 individuals, consisting of 3,002 healthy volunteers, 715 patients diagnosed with colorectal advanced precancerous lesions (APL), and 1,932 CRC patients, to perform a comprehensive metabolomic analysis. Results: We successfully identified unique age-associated patterns across 42 metabolic pathways. Moreover, we established a metabolic aging clock, comprising 9 key metabolites, using an elastic net regularized regression model that accurately estimates chronological age. Notably, we observed significant chronological disparities among the healthy population, APL patients, and CRC patients. By combining the analysis of circulative carcinoembryonic antigen levels with the categorization of individuals into the "hypo" metabolic aging subgroup, our blood test demonstrates the ability to detect APL and CRC with positive predictive values of 68.4% (64.3%, 72.2%) and 21.4% (17.8%, 25.9%), respectively. Conclusions: This innovative approach utilizing our metabolic aging clock holds significant promise for accurately assessing biological age and enhancing our capacity to detect APL and CRC.

  View details for DOI 10.7150/thno.87303

  View details for PubMedID 38389840

  View details for PubMedCentralID PMC10879879

• Exploring the feasibility of using long-term stored newborn dried blood spots to identify metabolic features for congenital heart disease screening. Biomarker research Ceresnak, S. R., Zhang, Y., Ling, X. B., Su, K. J., Tang, Q., Jin, B., Schilling, J., Chou, C. J., Han, Z., Floyd, B. J., Whitin, J. C., Hwa, K. Y., Sylvester, K. G., Chubb, H., Luo, R. Y., Tian, L., Cohen, H. J., McElhinney, D. B. 2023; 11 (1): 97

  Abstract

  Congenital heart disease (CHD) represents a significant contributor to both morbidity and mortality in neonates and children. There's currently no analogous dried blood spot (DBS) screening for CHD immediately after birth. This study was set to assess the feasibility of using DBS to identify reliable metabolite biomarkers with clinical relevance, with the aim to screen and classify CHD utilizing the DBS. We assembled a cohort of DBS datasets from the California Department of Public Health (CDPH) Biobank, encompassing both normal controls and three pre-defined CHD categories. A DBS-based quantitative metabolomics method was developed using liquid chromatography with tandem mass spectrometry (LC-MS/MS). We conducted a correlation analysis comparing the absolute quantitated metabolite concentration in DBS against the CDPH NBS records to verify the reliability of metabolic profiling. For hydrophilic and hydrophobic metabolites, we executed significant pathway and metabolite analyses respectively. Logistic and LightGBM models were established to aid in CHD discrimination and classification. Consistent and reliable quantification of metabolites were demonstrated in DBS samples stored for up to 15 years. We discerned dysregulated metabolic pathways in CHD patients, including deviations in lipid and energy metabolism, as well as oxidative stress pathways. Furthermore, we identified three metabolites and twelve metabolites as potential biomarkers for CHD assessment and subtypes classifying. This study is the first to confirm the feasibility of validating metabolite profiling results using long-term stored DBS samples. Our findings highlight the potential clinical applications of our DBS-based methods for CHD screening and subtype classification.

  View details for DOI 10.1186/s40364-023-00536-y

  View details for PubMedID 37957758

  View details for PubMedCentralID PMC10644604

• The development and efficacy of a paediatric cardiology fellowship online preparatory course. Cardiology in the young Motonaga, K. S., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J. A., Silverman, N. H., Hanley, F. L., Axelrod, D. M., Krawczeski, C. D., Arunamata, A., Kwiatkowski, D. M., Ceresnak, S. R. 2022: 1-6

  Abstract

  BACKGROUND: The transition from residency to paediatric cardiology fellowship is challenging due to the new knowledge and technical skills required. Online learning can be an effective didactic modality that can be widely accessed by trainees. We sought to evaluate the effectiveness of a paediatric cardiology Fellowship Online Preparatory Course prior to the start of fellowship.METHODS: The Online Preparatory Course contained 18 online learning modules covering basic concepts in anatomy, auscultation, echocardiography, catheterisation, cardiovascular intensive care, electrophysiology, pulmonary hypertension, heart failure, and cardiac surgery. Each online learning module included an instructional video with pre-and post-video tests. Participants completed pre- and post-Online Preparatory Course knowledge-based exams and surveys. Pre- and post-Online Preparatory Course survey and knowledge-based examination results were compared via Wilcoxon sign and paired t-tests.RESULTS: 151 incoming paediatric cardiology fellows from programmes across the USA participated in the 3 months prior to starting fellowship training between 2017 and 2019. There was significant improvement between pre- and post-video test scores for all 18 online learning modules. There was also significant improvement between pre- and post-Online Preparatory Course exam scores (PRE 43.6 ± 11% versus POST 60.3 ± 10%, p < 0.001). Comparing pre- and post-Online Preparatory Course surveys, there was a statistically significant improvement in the participants' comfort level in 35 of 36 (97%) assessment areas. Nearly all participants (98%) agreed or strongly agreed that the Online Preparatory Course was a valuable learning experience and helped alleviate some anxieties (77% agreed or strongly agreed) related to starting fellowship.CONCLUSION: An Online Preparatory Course prior to starting fellowship can provide a foundation of knowledge, decrease anxiety, and serve as an effective educational springboard for paediatric cardiology fellows.

  View details for DOI 10.1017/S1047951122003626

  View details for PubMedID 36440543

• Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration. JACC. Clinical electrophysiology Howard, T. S., Chiang, D. Y., Ceresnak, S. R., Ladouceur, V. B., Whitehill, R. D., Czosek, R. J., Knilans, T. K., Ahnfeldt, A. M., Borresen, M. L., Jaeggi, E., Udupa, S., Gow, R., Moore, J. P., Galloti, R. G., Mah, D. Y., Kim, J. J., Valdes, S. O., Milewicz, D. M., Miyake, C. Y. 2022

  Abstract

  BACKGROUND: Atrial standstill (AS) is a rare condition characterized by absence of electrical activity within the atria. Studies to date have been limited.OBJECTIVES: The authors sought to describe the clinical characteristics, genetics, and outcomes of patients with AS.METHODS: This was a retrospective multicenter study of patients<18 years at AS diagnosis, defined as absence of atrial activity documented during an electrophysiology study, device placement, or noninvasive rhythm tracings and confirmed by echocardiogram. Patients with acquired disorders were excluded. Clinical details and genetic variants were recorded and analyzed.RESULTS: Twenty patients were diagnosed at a median age of 6.6 years (IQR: 2.9-10.8 years). Arrhythmias included 16(80%) with atrial/supraventricular arrhythmias and 8 (40%) with ventricular tachycardia, including 4 with cardiac arrests. A type 1 Brugada pattern was documented in 4. Pacemakers were implanted in 18 (90%). Although atrial leads were attempted in 15, only 4 achieved pacing at implantation. During a median follow-up of 6.9 years (IQR: 1.2-13.3 years), 7 (35%) had thromboembolic events. Of these, none had atrial pacing, 6 were not on anticoagulation, and 1 was on aspirin. Genetic testing identified SCN5A variants in 13 patients (65%). Analyses suggest SCN5A loss-of-function may be one mechanism driving AS. Ventricular arrhythmias and cardiac arrest were more commonly seen in patients with biallelic SCN5A variants.CONCLUSIONS: AS may be associated with loss-of-function SCN5A variants. Patients demonstrate atrial and ventricular arrhythmias, and may present challenges during device placement. Patients without the capacity for atrial pacing are at risk for thromboembolic events and warrant anticoagulation.

  View details for DOI 10.1016/j.jacep.2022.08.022

  View details for PubMedID 36435694

• Single center blind testing of a US multi-center validated diagnostic algorithm for Kawasaki disease in Taiwan. Frontiers in immunology Kuo, H. C., Hao, S., Jin, B., Chou, C. J., Han, Z., Chang, L. S., Huang, Y. H., Hwa, K., Whitin, J. C., Sylvester, K. G., Reddy, C. D., Chubb, H., Ceresnak, S. R., Kanegaye, J. T., Tremoulet, A. H., Burns, J. C., McElhinney, D., Cohen, H. J., Ling, X. B. 2022; 13: 1031387

  Abstract

  Kawasaki disease (KD) is the leading cause of acquired heart disease in children. The major challenge in KD diagnosis is that it shares clinical signs with other childhood febrile control (FC) subjects. We sought to determine if our algorithmic approach applied to a Taiwan cohort.A single center (Chang Gung Memorial Hospital in Taiwan) cohort of patients suspected with acute KD were prospectively enrolled by local KD specialists for KD analysis. Our previously single-center developed computer-based two-step algorithm was further tested by a five-center validation in US. This first blinded multi-center trial validated our approach, with sufficient sensitivity and positive predictive value, to identify most patients with KD diagnosed at centers across the US. This study involved 418 KDs and 259 FCs from the Chang Gung Memorial Hospital in Taiwan.Our diagnostic algorithm retained sensitivity (379 of 418; 90.7%), specificity (223 of 259; 86.1%), PPV (379 of 409; 92.7%), and NPV (223 of 247; 90.3%) comparable to previous US 2016 single center and US 2020 fiver center results. Only 4.7% (15 of 418) of KD and 2.3% (6 of 259) of FC patients were identified as indeterminate. The algorithm identified 18 of 50 (36%) KD patients who presented 2 or 3 principal criteria. Of 418 KD patients, 157 were infants younger than one year and 89.2% (140 of 157) were classified correctly. Of the 44 patients with KD who had coronary artery abnormalities, our diagnostic algorithm correctly identified 43 (97.7%) including all patients with dilated coronary artery but one who found to resolve in 8 weeks.This work demonstrates the applicability of our algorithmic approach and diagnostic portability in Taiwan.

  View details for DOI 10.3389/fimmu.2022.1031387

  View details for PubMedID 36263040

  View details for PubMedCentralID PMC9575935

• VENTRICULAR ARRHYTHMIAS FOLLOWING TRANSCATHETER PULMONARY VALVE REPLACEMENT WITH THE HARMONY(C) TPV 25 DEVICE Taylor, A., Yang, J., Dubin, A., Chubb, M., Motonaga, K., Goodyer, W., Giacone, H., Peng, L. F., Romfh, A. W., McElhinney, D. B., Ceresnak, S. R. ELSEVIER SCIENCE INC. 2022: 1362

  View details for Web of Science ID 000781026601461

• Ultra-Rapid Nanopore Whole Genome Genetic Diagnosis of Dilated Cardiomyopathy in an Adolescent With Cardiogenic Shock. Circulation. Genomic and precision medicine Gorzynski, J. E., Goenka, S. D., Shafin, K., Jensen, T. D., Fisk, D. G., Grove, M. E., Spiteri, E., Pesout, T., Monlong, J., Bernstein, J. A., Ceresnak, S., Chang, P., Christle, J. W., Chubb, H., Dunn, K., Garalde, D. R., Guillory, J., Ruzhnikov, M. R., Wright, C., Wusthoff, C. J., Xiong, K., Hollander, S. A., Berry, G. J., Jain, M., Sedlazeck, F. J., Carroll, A., Paten, B., Ashley, E. A. 2022: CIRCGEN121003591

  View details for DOI 10.1161/CIRCGEN.121.003591

  View details for PubMedID 35133172

• Ultrarapid Nanopore Genome Sequencing in a Critical Care Setting. The New England journal of medicine Gorzynski, J. E., Goenka, S. D., Shafin, K., Jensen, T. D., Fisk, D. G., Grove, M. E., Spiteri, E., Pesout, T., Monlong, J., Baid, G., Bernstein, J. A., Ceresnak, S., Chang, P. C., Christle, J. W., Chubb, H., Dalton, K. P., Dunn, K., Garalde, D. R., Guillory, J., Knowles, J. W., Kolesnikov, A., Ma, M., Moscarello, T., Nattestad, M., Perez, M., Ruzhnikov, M. R., Samadi, M., Setia, A., Wright, C., Wusthoff, C. J., Xiong, K., Zhu, T., Jain, M., Sedlazeck, F. J., Carroll, A., Paten, B., Ashley, E. A. 2022

  View details for DOI 10.1056/NEJMc2112090

  View details for PubMedID 35020984

• Implantable Cardioverter Defibrillators in Infants and Toddlers: Indications, Placement, Programming, and Outcomes. Circulation. Arrhythmia and electrophysiology Zahedivash, A., Hanisch, D., Dubin, A. M., Trela, A., Chubb, H., Motonaga, K., Goodyer, W., Maeda, K., Reinhartz, O., Ma, M., Martin, E., Ceresnak, S. 2022: CIRCEP121010557

  Abstract

  Limited data exist regarding implantable cardioverter defibrillator (ICD) usage in infants and toddlers. This study evaluates ICD placement indications, procedural techniques, programming strategies, and outcomes of ICDs in infants and toddlers.This is a single-center retrospective review of all patients ≤3 years old who received an ICD from 2009 to 2021.Fifteen patients received an ICD at an age of 1.2 years (interquartile range [IQR], 0.1-2.4; 12 [80%] women; weight, 8.2 kg [IQR, 4.2-12.6]) and were followed for a median of 4.28 years (IQR, 1.40-5.53) or 64.2 patient-years. ICDs were placed for secondary prevention in 12 patients (80%). Diagnoses included 8 long-QT syndromes (53%), 4 idiopathic ventricular tachycardias/ventricular fibrillations (VFs; 27%), 1 recurrent ventricular tachycardia with cardiomyopathy (7%), 1 VF with left ventricular noncompaction (7%), and 1 catecholaminergic polymorphic ventricular tachycardia (7%). All implants were epicardial, with a coil in the pericardial space. Intraoperative defibrillation safety testing was attempted in 11 patients (73%), with VF induced in 8 (53%). Successful restoration of sinus rhythm was achieved in all tested patients with a median of 9 (IQR, 7.3-11.3) J or 0.90 (IQR, 0.68-1.04) J/kg. Complications consisted of 1 postoperative chylothorax and 3 episodes of feeding intolerance. VF detection was programmed to 250 (IQR, 240-250) ms with first shock delivering 10 (IQR, 5-15) J or 1.1 (IQR, 0.8-1.4) J/kg. Three patients (20%) received appropriate shocks for ventricular tachycardia/VF. No patient received an inappropriate shock. There were 2 (13%) ventricular lead fractures (at 2.6 and 4.2 years post-implant), 1 (7%) pocket-site infection, and 2 (13%) generator exchanges. All patients were alive, and 1 patient (7%) received a heart transplant.ICDs can be safely and effectively placed for sudden death prevention in infants and toddlers with good midterm outcomes.

  View details for DOI 10.1161/CIRCEP.121.010557

  View details for PubMedID 35089800

• Identifying an Appropriate Endpoint for Cryoablation in Children with Atrioventricular Nodal Reentry Tachycardia: Is Residual Slow Pathway Conduction Associated with Recurrence? Heart rhythm Zook, N., DeBruler, K., Ceresnak, S., Motonaga, K., Goodyer, W., Trela, A., Dubin, A., Chubb, H. 2021

  Abstract

  BACKGROUND: Cryoablation is increasingly used to treat atrioventricular nodal reentry tachycardia (AVNRT) due to its safety profile. However, cryoablation may have higher recurrence than radiofrequency ablation (RFA) and the optimal procedural endpoint remains undefined.OBJECTIVE: The purpose of this study was to identify the association of cryoablation procedural endpoints with post-procedural AVNRT recurrence.METHODS: We performed a single-center, retrospective analysis of pediatric patients following successful first-time cryoablation for AVNRT between 1/1/2011 and 12/31/2019. Pre-ablation inducibility of AVNRT was recorded. Procedural endpoints, including slow pathway (SP) conduction (presence of jump or echo beats) with and without isoproterenol, were identified. Recurrence established from clinical notes and/or direct patient contact.RESULTS: Of 256 patients, 147(57%) were assessed on isoproterenol pre-cryoablation, and 171(47%) were assessed on isoproterenol post-cryoablation. Mean cryolesion time was 2586±1434 seconds. Following ablation, 104(41%) had some evidence of residual SP conduction. With median follow up time of 1.9[0.7-3.7] years, recurrence occurred in 14(5%) patients. Complete elimination of SP conduction (with and without isoproterenol) had a HR for recurrence of 1.26(95% CI 0.42-3.8, P=.68) on univariate analysis and 1.39(95% CI 0.36-5.4, P=.63) on multivariate analysis (including demographics, ablation time, 8mm cryocatheter and baseline inducibility).CONCLUSION: The observed AVNRT recurrence rate after cryoablation was comparable to RFA. The presence of residual SP conduction was not associated with recurrence. This suggests that jump or single echo beat may be an acceptable endpoint in AVNRT cryoablation.

  View details for DOI 10.1016/j.hrthm.2021.09.031

  View details for PubMedID 34601128

• IMPLANTABLE CARDIOVERTER-DEFIBRILLATORS IN INFANTS AND TODDLERS: INDICATIONS, PLACEMENT, PROGRAMMING AND OUTCOMES Zahedivash, A., Hanisch, D., Dubin, A. M., Trela, A. V., Chubb, H., Motonaga, K., Goodyer, W., Maeda, K., Reinhartz, O., Ceresnak, S. ELSEVIER SCIENCE INC. 2021: 470

  View details for Web of Science ID 000647487500470

• Subcutaneous Implantable Cardioverter-Defibrillators in Pediatrics and Congenital Heart Disease: A Pediatric and Congenital Electrophysiology Society Multicenter Review. JACC. Clinical electrophysiology von Alvensleben, J. C., Dechert, B., Bradley, D. J., Fish, F. A., Moore, J. P., Pilcher, T. A., Escudero, C., Ceresnak, S. R., Kwok, S. Y., Balaji, S., Aziz, P. F., Papagiannis, J., Cortez, D., Garnreiter, J., Kean, A., Schafer, M., Collins, K. K. 2020; 6 (14): 1752–61

  Abstract

  OBJECTIVES: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease.BACKGROUND: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease.METHODS: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360days, inappropriate shocks, and delivery of appropriate therapy were assessed.RESULTS: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30days and 14.7% at 360days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%.CONCLUSIONS: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.

  View details for DOI 10.1016/j.jacep.2020.07.010

  View details for PubMedID 33357571

• Impact of Cardiac Resynchronization Therapy on Heart Transplant-Free Survival in Pediatric and Congenital Heart Disease Patients. Circulation. Arrhythmia and electrophysiology Chubb, H., Rosenthal, D. N., Almond, C. S., Ceresnak, S. R., Motonaga, K. S., Arunamata, A. A., Long, J., Trela, A. V., Hanisch, D., McElhinney, D. B., Dubin, A. M. 2020

  Abstract

  Background - Cardiac resynchronization therapy (CRT) studies in pediatric and/or congenital heart disease (CHD) patients have shown an improvement in ejection fraction and heart failure symptoms. However, a survival benefit of CRT in this population has not been established. This study aimed to evaluate the impact of CRT upon heart transplant-free survival in pediatric and CHD patients, using a propensity score-matched analysis. Methods - This single-center study compared CRT patients (implant date 2004-2017) and controls, matched by 1:1 propensity-score matching (PSM) using 21 comprehensive baseline indices for risk stratification. CRT patients were <21 years or had CHD; had systemic ventricular ejection fraction <;45%; symptomatic heart failure; and had significant electrical dyssynchrony, all prior to CRT implant. Controls were screened from non-selective imaging and ECG databases. Controls were retrospectively enrolled when they achieved the same inclusion criteria at an outpatient clinical encounter, within the same time period. Results - Of 133 patients who received CRT during the study period, 84 met all study inclusion criteria. 133 controls met all criteria at an outpatient encounter. Following PSM, 63 matched CRT-control pairs were identified with no significant difference between groups across all baseline indices. Heart transplant or death occurred in 12 (19%) PSM-CRT subjects and 37 (59%) PSM-controls with a median follow-up of 2.7 years (quartiles 0.8-6.1 years). CRT was associated with markedly reduced risk of heart transplant or death (hazard ratio 0.24 [95% CI 0.12-0.46], p<0.001). There was no CRT procedural mortality and one system infection at 54 months post-implant. Conclusions - In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplant-free survival.

  View details for DOI 10.1161/CIRCEP.119.007925

  View details for PubMedID 32202126

• Can the 12-lead ECG distinguish RVOT from aortic cusp PVCs in pediatric patients? Pacing and clinical electrophysiology : PACE Clark, B. C., Ceresnak, S. R., Pass, R. H., Nappo, L., Sumihara, K., Dubin, A. M., Motonaga, K., Moak, J. P. 2020

  Abstract

  BACKGROUND: The ability to differentiate right ventricular outflow tract (RVOT) from coronary cusp (CC) site of origin (SOO) by 12-lead ECG in pediatric patients may impact efficacy and procedural time. The objective of this study was to predict RVOT versus CC SOO by ECG in pediatric patients.METHODS: Pediatric patients (<21 years) without structural heart disease with RVOT or CC premature ventricular contraction (PVC) ablations performed (2014 - 2018) were evaluated through multi-institution retrospective review. Demographics, ECG PVC parameters, ablation site, recurrence and repeat procedures were collected.RESULTS: 37 patients were evaluated (mean age 14.6 years, weight 60.6kg): 11 CC and 26 RVOT PVC SOO. CC PVCs were less likely to exhibit left bundle branch block (64vs. 100%, p=0.005), had larger R-wave amplitude in V1 (0.27vs. 0.11mV, p=0.03), larger R/S ratio in V1 (0.37vs. 0.09, p=0.003), and had precordial transition in V3 or earlier (73vs. 15%, p=0.002). A composite score was created with the following variables: isodiphasic or positive QRS in V1, R/S ratio in V1>0.05, S wave in V1<0.9mV and precordial transition at or before V3. Composite score ≥ 2 was associated with a CC SOO (OR 42.0, p=0.001, AUC 0.86).CONCLUSIONS: 12-lead ECG of PVCs from the CC was associated with larger V1 R-wave amplitude, larger R/S ratio in V1 and precordial transition at or before V3. A composite score may help predict PVC/VT arising from the CC. This article is protected by copyright. All rights reserved.

  View details for DOI 10.1111/pace.13885

  View details for PubMedID 32040211

• High-throughput quantitation of serological ceramides/dihydroceramides by LC/MS/MS: Pregnancy baseline biomarkers and potential metabolic messengers. Journal of pharmaceutical and biomedical analysis Huang, Q. n., Hao, S. n., Yao, X. n., You, J. n., Li, X. n., Lai, D. n., Han, C. n., Schilling, J. n., Hwa, K. Y., Thyparambil, S. n., Whitin, J. n., Cohen, H. J., Chubb, H. n., Ceresnak, S. R., McElhinney, D. B., Wong, R. J., Shaw, G. M., Stevenson, D. K., Sylvester, K. G., Ling, X. B. 2020; 192: 113639

  Abstract

  Ceramides and dihydroceramides are sphingolipids that present in abundance at the cellular membrane of eukaryotes. Although their metabolic dysregulation has been implicated in many diseases, our knowledge about circulating ceramide changes during the pregnancy remains limited. In this study, we present the development and validation of a high-throughput liquid chromatography-tandem mass spectrometric method for simultaneous quantification of 16 ceramides and 10 dihydroceramides in human serum within 5 min. by using stable isotope-labeled ceramides as internal standards. This method employs a protein precipitation method for high throughput sample preparation, reverse phase isocratic elusion for chromatographic separation, and Multiple Reaction Monitoring for mass spectrometric detection. To qualify for clinical applications, our assay has been validated against the FDA guidelines for Lower Limit of Quantitation (1 nM), linearity (R2>0.99), precision (imprecision<15 %), accuracy (inaccuracy<15 %), extraction recovery (>90 %), stability (>85 %), and carryover (<0.01 %). With enhanced sensitivity and specificity from this method, we have, for the first time, determined the serological levels of ceramides and dihydroceramides to reveal unique temporal gestational patterns. Our approach could have value in providing insights into disorders of pregnancy.

  View details for DOI 10.1016/j.jpba.2020.113639

  View details for PubMedID 33017796

• Kinetics of SARS-CoV-2 positivity of infected and recovered patients from a single center. Scientific reports Huang, J. n., Zheng, L. n., Li, Z. n., Hao, S. n., Ye, F. n., Chen, J. n., Gans, H. A., Yao, X. n., Liao, J. n., Wang, S. n., Zeng, M. n., Qiu, L. n., Li, C. n., Whitin, J. C., Tian, L. n., Chubb, H. n., Hwa, K. Y., Ceresnak, S. R., Zhang, W. n., Lu, Y. n., Maldonado, Y. A., McElhinney, D. B., Sylvester, K. G., Cohen, H. J., Liu, L. n., Ling, X. B. 2020; 10 (1): 18629

  Abstract

  Recurrence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive detection in infected but recovered individuals has been reported. Patients who have recovered from coronavirus disease 2019 (COVID-19) could profoundly impact the health care system. We sought to define the kinetics and relevance of PCR-positive recurrence during recovery from acute COVID-19 to better understand risks for prolonged infectivity and reinfection. A series of 414 patients with confirmed SARS-Cov-2 infection, at The Second Affiliated Hospital of Southern University of Science and Technology in Shenzhen, China from January 11 to April 23, 2020. Statistical analyses were performed of the clinical, laboratory, radiologic image, medical treatment, and clinical course of admission/quarantine/readmission data, and a recurrence predictive algorithm was developed. 16.7% recovered patients with PCR positive recurring one to three times, despite being in strict quarantine. Younger patients with mild pulmonary respiratory syndrome had higher risk of PCR positivity recurrence. The recurrence prediction model had an area under the ROC curve of 0.786. This case series provides characteristics of patients with recurrent SARS-CoV-2 positivity. Use of a prediction algorithm may identify patients at high risk of recurrent SARS-CoV-2 positivity and help to establish protocols for health policy.

  View details for DOI 10.1038/s41598-020-75629-x

  View details for PubMedID 33122706

• Structural Abnormalities on Cardiac Magnetic Resonance Imaging in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia. JACC. Clinical electrophysiology Gerber, D. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Bussineau, M. n., Dunn, K. n., Caleshu, C. n., Shoemaker, M. B., Lubitz, S. A., Perez, M. V. 2020; 6 (6): 741–42

  View details for DOI 10.1016/j.jacep.2020.03.006

  View details for PubMedID 32553227

• An exploratory assessment of pediatric patient and parent needs after implantable cardioverter defibrillator implant. Pacing and clinical electrophysiology : PACE Schneider, L. M., Wong, J. J., Trela, A. n., Hanisch, D. n., Shaw, R. J., Sears, S. F., Motonaga, K. S., Ceresnak, S. R., Hood, K. K., Dubin, A. M. 2020

  Abstract

  Placement of an ICD is often accompanied by psychological adjustment issues in pediatric patients and their parents. While anxiety, depression, and lowered QOL have been seen in these patients, no studies have investigated patient or parent reported needs. This exploratory study describes the needs of pediatric ICD patients and parents and assesses whether patient factors of age, gender, depression, and anxiety are associated with specific needs.ICD patients ages 8-21 and their parents completed a needs analysis survey assessing various domains of functioning. Patients also completed self-reported measures of depression and anxiety.Thirty-two patients (28% female) and their parents (72% mothers) completed the survey. Patients' most frequently endorsed needs involved educational issues: understanding their cardiac event/diagnosis (34%), medications (34%), and how the ICD would change their lifestyle (31%). Parents' most frequently endorsed needs involved family issues; almost half of parents (47%) were concerned about their children's frustration with their overprotectiveness and 28% were concerned with their child feeling depressed or anxious. Patients who reported feeling overprotected (12.1 ± 3.4 vs 17.4 ± 3.5 years, p = .001) were significantly younger than those who did not. Experiencing peer issues was more frequently endorsed by females than males (33% of females vs 4% of males, p = .026).ICD patients and parents endorsed markedly different needs. Patients focused on understanding their ICD, while parents were more focused on their children's emotional needs. Novel ways of educating patients about their device and clinic based screenings of emotional functioning may serve to meet these needs. This article is protected by copyright. All rights reserved.

  View details for DOI 10.1111/pace.13876

  View details for PubMedID 31971265

• Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study. The international journal of cardiovascular imaging Hagdorn, Q. A., Beurskens, N. E., Gorter, T. M., Eshuis, G. n., Hillege, H. L., Lui, G. K., Ceresnak, S. R., Chan, F. P., van Melle, J. P., Berger, R. M., Willems, T. P. 2020

  Abstract

  Purpose Substantial differences between sexes exist with respect to cardiovascular diseases, including congenital heart disease. Nevertheless, clinical decisions in the long-term follow-up of patients with repaired tetralogy of Fallot (rTOF) are currently based on unisex thresholds for cardiac magnetic resonance (CMR) measurements. This study aimed to assess whether sex differences exist in cardiac adaptation to hemodynamic loading conditions in patients with rTOF. Methods and Results This cross-sectional, two-center, combined pediatric and adult cohort included 320 rTOF patients (163 males, 51%) who underwent routine CMR. Despite similar age (median and interquartile range [m + IQR] 23.4 [15.2-34.4] years), surgical history, and hemodynamic loading, males with rTOF demonstrated higher biventricular CMR-derived volumes and masses, indexed for body surface area, compared to females (e.g. m + IQR right ventricular (RV) end-diastolic volume: males 123 [100-151] mL/m2, females 114 [94-131] mL/m2, P = 0.007). Sex-specific Z-scores of biventricular volumes and masses were similar for males and females. RV volumes and masses correlated with hemodynamic loading, but these relations did not differ between sexes. Biventricular ejection fraction (EF) appeared to be lower in male patients, compared to female patients (e.g. m + IQR RVEF: males 48 [43-54]%, females 52 [46-57]%, P < 0.001). Conclusion Indexed ventricular volumes and masses are higher in males with rTOF, compared to females, similar to the healthy population. RV hypertrophy and dilatation correlated to loading conditions similarly for both sexes. However, under comparable loading conditions, males demonstrated more severe functional impairment. These results indicate that sex-differences should no longer be ignored in treatment strategies, including timing of pulmonary valve replacement.

  View details for DOI 10.1007/s10554-020-01900-x

  View details for PubMedID 32472300

• "If at first you don't succeed": repeat ablations in young patients with supraventricular tachycardia. Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing Rochelson, E., Clark, B. C., Janson, C. M., Ceresnak, S. R., Nappo, L., Pass, R. H. 2019

  Abstract

  PURPOSE: Success rates for catheter ablation of supraventricular tachycardia (SVT) in the young exceed 90%. While studies have described reasons for initial ablation failure, less is known about outcomes of repeat ablation attempts. The purpose of this study was to report acute and mid-term success rates for second ablation attempts in young patients, as well as to analyze factors that may affect these outcomes.METHODS: Retrospective single-center study of all patients undergoing a second ablation attempt for WPW (Wolff-Parkinson-White) or SVT from 2008 to 2017. Inclusion criteria are all patients <21years old at the time of their first ablation who underwent a second ablation attempt. An intention to treat analysis was performed.RESULTS: Fifty-five patients met inclusion criteria, with a median age of 15years (IQR 12-16). The most common arrhythmia mechanisms at repeat procedure were single accessory pathways (n=32, 58%) and AVNRT (n=14, 25%). Six patients (11%) were found to have a different SVT mechanism than at initial ablation. Acute success at repeat ablation was achieved in 48 patients (87%). At mid-term follow-up (10.5months, IQR 0.6-25), four patients (8% of acute successes) experienced SVT recurrence. The overall success rate of repeat ablations, accounting for acute and mid-term failures, was 80%.CONCLUSIONS: In this report of 55 young patients who underwent repeat ablation for WPW and/or SVT, acute and mid-term success rates were 87% and 80%, respectively. These data may help inform decision-making when caring for patients with persistent or recurrent SVT after an initial ablation attempt.

  View details for DOI 10.1007/s10840-019-00672-2

  View details for PubMedID 31834569

• QTc Prolongation and Risk of Torsades de Pointes in Hospitalized Pediatric Oncology Patients. The Journal of pediatrics Lim, T. R., Rangaswami, A. A., Dubin, A. M., Kapphahn, K. I., Sakarovitch, C., Long, J., Motonaga, K. S., Trela, T., Ceresnak, S. R. 2019

  Abstract

  OBJECTIVE: To evaluate the prevalence of torsades de pointes and to identify risk factors associated with QTc prolongation of ≥500milliseconds in hospitalized pediatric oncology patients. A QTc prolongation of ≥500milliseconds is associated with higher mortality in hospitalized adults but has not been demonstrated in pediatrics.STUDY DESIGN: A single-center, retrospective review of all hospitalized oncology patients ≤21years of age was performed from 2014 to 2016. Patients with long/short QT syndrome or a QRS interval of ≥120ms were excluded. Rapid response events were reviewed to determine the prevalence of torsades. In patients with ECGs for review, data were compared between patients with a QTc of <500 and≥500ms via logistic regression.RESULTS: There were 1934 hospitalized patients included. Rapid response events occurred in 90 patients (4.7%) with 2 torsades events (0.1%). There were 1412 electrocardiograms performed in 287 unique patients (10.6±6.3years of age; 43% female). The mean QTc was 448±31ms; 25 patients (8.7%) had ≥1 ECG with a QTc of ≥500ms. The prevalence of torsades was greater in patients with a QTc of ≥500ms (8% vs 0%; P<.01). In multivariate analysis, factors associated with a QTc of ≥500ms included female sex, (OR 2.95) and ≥2 QT-prolonging medications (OR, 2.95).CONCLUSIONS: The prevalence of torsades in hospitalized pediatric oncology patients was low (0.1%), although the risk was significantly greater in patients with a QTc of ≥500ms. Routine monitoring of electrocardiograms and electrolytes is essential in patients with risk factors predisposing to QTc prolongation.

  View details for DOI 10.1016/j.jpeds.2019.10.018

  View details for PubMedID 31761428

• Broad Genetic Testing in a Clinical Setting Uncovers a High Prevalence of Titin Loss-of-Function Variants in Very Early-Onset Atrial Fibrillation. Circulation. Genomic and precision medicine Goodyer, W. R., Dunn, K., Caleshu, C., Jackson, M., Wylie, J., Moscarello, T., Platt, J., Reuter, C., Smith, A., Trela, A., Ceresnak, S. R., Motonaga, K. S., Ashley, E., Yang, P., Dubin, A. M., Perez, M. 2019

  Abstract

  Atrial fibrillation (AF) is the most common sustained arrhythmia, affecting approximately 34 million worldwide. The pathophysiology of AF remains incompletely understood but is clearly complex with multiple underlying genetic, physiologic and environmental factors. Very early-onset AF (vEAF) (defined here as onset <45 years and without significant comorbidities), while rare (only ~0.5-3% of AF cases), is highly heritable, with a greater prevalence of rare variants in genes previously associated with AF. Patients with vEAF, therefore, represent an ideal population for discovering novel genes involved in the underlying genetic basis of AF. Notably, the Framingham study showed that patients with AF without comorbidities have a three-fold higher risk for heart failure. Conversely, several forms of inherited cardiomyopathy have been strongly associated with AF suggestive of a shared etiology.

  View details for DOI 10.1161/CIRCGEN.119.002713

  View details for PubMedID 31638414

• Management of Asymptomatic Wolff-Parkinson-White Pattern by Pediatric Electrophysiologists. The Journal of pediatrics Chubb, H., Campbell, R. M., Motonaga, K. S., Ceresnak, S. R., Dubin, A. M. 2019

  Abstract

  OBJECTIVE: To determine the present-day approach of pediatric cardiac electrophysiologists to asymptomatic Wolff-Parkinson-White (WPW) pattern and to contrast to both published consensus statements and a similar survey.STUDY DESIGN: A questionnaire was sent to 266 Pediatric and Congenital Electrophysiology Society physician members in 25 countries; 21 questions from the 2003 survey were repeated, with new questions added regarding risk stratification and decision making.RESULTS: We received 113 responses from 13 countries, with responders having extensive electrophysiology experience (median 15years [IQR 8.5-25years]). Only 12 (11%) believed that intermittent pre-excitation and 37 (33%) that sudden loss of pre-excitation on exercise test were sufficient evidence of accessory pathway safety to avoid an invasive electrophysiology study. Optimal weight for electrophysiology study was 20kg (IQR 18-22.5kg), and 61% and 58% would then ablate all right-sided or left-sided accessory pathways, respectively, regardless of electrophysiological properties, whereas only 23% would ablate all septal accessory pathways (P<.001). Compared with 2003, respondents were more likely to consider inducible arrhythmia (77% vs 26%, P<.001) as sufficient indication alone for ablation.CONCLUSIONS: In the context of recent literature regarding the reliability of risk-stratification tools, most operators are now performing electrophysiology study for asymptomatic Wolff-Parkinson-White regardless of noninvasive findings. Many will then proceed to default ablation of all accessory pathways distant from critical conduction structures.

  View details for DOI 10.1016/j.jpeds.2019.05.058

  View details for PubMedID 31235382

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring CONGENITAL HEART DISEASE Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019; 14 (3): 410–18

  View details for DOI 10.1111/chd.12736

  View details for Web of Science ID 000471070900013

• A novel pacing maneuver to verify the postpacing interval minus the tachycardia cycle length while adjusting for decremental conduction: Using "dual-chamber entrainment" for improved supraventricular tachycardia discrimination HEART RHYTHM Kaiser, D. W., Nasir, J. M., Liem, L., Brodt, C., Motonaga, K. S., Ceresnak, S. R., Turakhia, M. P., Dubin, A. M. 2019; 16 (5): 717–23

  View details for DOI 10.1016/j.hrthm.2018.11.021

  View details for Web of Science ID 000466096200013

• Incidence of Echocardiographic Abnormalities Following Pediatric SVT Ablation: Comparison of Cases Utilizing Fluoroscopy Alone to Cases with Adjunctive 3D Electroanatomic Mapping PEDIATRIC CARDIOLOGY Gartenberg, A. J., Pass, R. H., Ceresnak, S., Nappo, L., Janson, C. M. 2019; 40 (3): 497–503

  View details for DOI 10.1007/s00246-018-1999-0

  View details for Web of Science ID 000461348000006

• Year in Review in Cardiac Electrophysiology. Circulation. Arrhythmia and electrophysiology Tzou, W. S., Hussein, A. A., Madhavan, M., Viswanathan, M. N., Steinberg, B. A., Ceresnak, S. R., Davis, D. R., Park, D. S., Wang, P. J., Kapa, S. 2019; 12 (2): e007142

  View details for PubMedID 30744401

• Year in Review in Cardiac Electrophysiology CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY Tzou, W. S., Hussein, A. A., Madhavan, M., Viswanathan, M. N., Steinberg, B. A., Ceresnak, S. R., Davis, D. R., Park, D. S., Wang, P. J., Kapa, S. 2019; 12 (2)

  View details for DOI 10.1161/CIRCEP.118.007142

  View details for Web of Science ID 000459028300007

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring. Congenital heart disease Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019

  Abstract

  BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

  View details for PubMedID 30604934

• A proposed method for the calculation of age-dependent QRS duration z-scores. Journal of electrocardiology Chubb, H. n., Ceresnak, S. R., Motonaga, K. S., Dubin, A. M. 2019; 58: 132–34

  Abstract

  There are currently no published algorithms for calculation of age-dependent QRS duration z-scores. The absence of a standardized measure has limited researchers' abilities to compare ECG measurements of electrical synchrony between subjects of different ages or longitudinally over time.Four existing studies of normal ECG measurements (total 19,062 subjects) were used to estimate age and sex-dependent means and standard deviations.Weighted means and standard deviations were best estimated by cubic functions to create z-score algorithms.Nomograms and algorithms for QRS duration z-scores may be estimated to compare ECG findings in both children and adults.

  View details for DOI 10.1016/j.jelectrocard.2019.12.004

  View details for PubMedID 31846856

• A Prospective Assessment of Optimal Mechanical Ventilation Parameters for Pediatric Catheter Ablation PEDIATRIC CARDIOLOGY Janson, C. M., Ceresnak, S. R., Choi, J. M., Dubin, A. M., Motonaga, K. S., Mann, G. E., Kahana, M., Fitz-James, I., Wise-Faberowski, L., Kamra, K., Nappo, L., Trela, A., Pass, R. H. 2019; 40 (1): 126–32

  Abstract

  Catheter stability, an important factor in ablation success, is affected by ventilation. Optimal ventilation strategies for pediatric catheter ablation are not known. We hypothesized that small tidal volume and positive end-expiratory pressure are associated with reduced ablation catheter movement at annular positions. Subjects aged 5-25 years undergoing ablation for supraventricular tachycardia (SVT) or WPW at two centers from March 2015 to September 2016 were prospectively enrolled and randomized to receive mechanical ventilation with either positive end-expiratory pressure of 5 cm H2O (PEEP) or 0 cm H2O (ZEEP). Movement of the ablation catheter tip at standard annular positions was measured using 3D electroanatomic mapping systems under two conditions: small tidal volume (STV) (3-5 mL/kg) or large TV (LTV) (6-8 mL/kg). 58 subjects (mean age 13.8 years) were enrolled for a total of 266 separate observations of catheter movement. STV ventilation was associated with significantly reduced catheter movement, compared to LTV at all positions (right posteroseptal: 2.5 ± 1.4 vs. 5.2 ± 3.1 mm, p < 0.0001; right lateral: 2.7 ± 1.6 vs. 6.3 ± 3.5 mm, p < 0.0001; left lateral: 1.8 ± 1.0 vs. 4.3 ± 1.9 mm, p < 0.0001). The presence or absence of PEEP had no effect on catheter movement. In multivariable analysis, STV was associated with a 3.1-mm reduction in movement (95% CI 2.6-3.5, p < 0.0001), adjusting for end-expiratory pressure, annular location, and patient size. We conclude that STV ventilation is associated with reduced ablation catheter movement compared to a LTV strategy, independent of PEEP and annular position.

  View details for PubMedID 30178187

• Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING Beurskens, N. G., Hagdorn, Q. J., Gorter, T. M., Berger, R. F., Vermeulen, K. M., van Melle, J. P., Ebels, T. E., Lui, G. K., Ceresnak, S. R., Chan, F. P., Willems, T. P. 2019; 35 (1): 143–51

  Abstract

  Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9-6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p < 0.001) independently predicted ATA. RV systolic dysfunction, hypertrophy and dilatation on CMR, together with QRS prolongation, and obesity are predictive of VT in TOF patients. Older age at TOF repair and obesity were associated with the occurrence of ATA.

  View details for PubMedID 30094564

• Validation of a novel automated signal analysis tool for ablation of Wolff-Parkinson-White Syndrome. PloS one Ceresnak, S. R., Pass, R. H., Dubin, A. M., Yang, L., Motonaga, K. S., Hedlin, H., Avasarala, K., Trela, A., McElhinney, D. B., Janson, C., Nappo, L., Ling, X. B., Gates, G. J. 2019; 14 (6): e0217282

  Abstract

  BACKGROUND: In previous pilot work we demonstrated that a novel automated signal analysis tool could accurately identify successful ablation sites during Wolff-Parkinson-White (WPW) ablation at a single center.OBJECTIVE: We sought to validate and refine this signal analysis tool in a larger multi-center cohort of children with WPW.METHODS: A retrospective review was performed of signal data from children with WPW who underwent ablation at two pediatric arrhythmia centers from 2008-2015. All patients with WPW ≤ 21 years who underwent invasive electrophysiology study and ablation with ablation signals available for review were included. Signals were excluded if temperature or power delivery was inadequate or lesion time was < 5 seconds. Ablation lesions were reviewed for each patient. Signals were classified as successful if there was loss of antegrade and retrograde accessory pathway (AP) conduction or unsuccessful if ablation did not eliminate AP conduction. Custom signal analysis software analyzed intracardiac electrograms for amplitudes, high and low frequency components, integrated area, and signal timing components to create a signal score. We validated the previously published signal score threshold 3.1 in this larger, more diverse cohort and explored additional scoring options. Logistic regression with lasso regularization using Youden's index criterion and a cost-benefit criterion to identify thresholds was considered as a refinement to this score.RESULTS: 347 signals (141 successful, 206 unsuccessful) in 144 pts were analyzed [mean age 13.2 ± 3.9 years, 96 (67%) male, 66 (45%) left sided APs]. The software correctly identified the signals as successful or unsuccessful in 276/347 (80%) at a threshold of 3.1. The performance of other thresholds did not significantly improve the predictive ability. A signal score threshold of 3.1 provided the following diagnostic accuracy for distinguishing a successful from unsuccessful signal: sensitivity 83%, specificity 77%, PPV 71%, NPV 87%.CONCLUSIONS: An automated signal analysis software tool reliably distinguished successful versus unsuccessful ablation electrograms in children with WPW when validated in a large, diverse cohort. Refining the tools using an alternative threshold and statistical method did not improve the original signal score at a threshold of 3.1. This software was effective across two centers and multiple operators and may be an effective tool for ablation of WPW.

  View details for DOI 10.1371/journal.pone.0217282

  View details for PubMedID 31242221

• A Novel Pacing Maneuver to Verify the Post-Pacing Interval Minus the Tachycardia Cycle Length While Adjusting for Decremental Conduction: Using 'Dual Chamber Entrainment' for Improved Supraventricular Tachycardia Discrimination. Heart rhythm Kaiser, D. W., Nasir, J. M., Liem, L. B., Brodt, C., Motonaga, K. S., Ceresnak, S. R., Turakhia, M. P., Dubin, A. M. 2018

  Abstract

  BACKGROUND: The post-pacing interval (PPI) minus the tachycardia cycle length (TCL) is frequently used to investigate tachycardias. However, a variety of issues (e.g. failure to entrain, decremental conduction, and oscillating TCLs) can make interpretation of the PPI-TCL challenging.OBJECTIVES: To investigate a novel maneuver to confirm the PPI-TCL value without using either the ventricular PPI or the TCL interval. To assess the ability of this maneuver to identify decremental conduction and differentiate supraventricular tachycardias.METHODS: We analyzed 77 intracardiac recordings from patients [age 25±20 years, 40 female] who underwent catheter ablation of AVNRT or orthodromic reciprocating tachycardia (ORT) with a concealed pathway. We calculated the PPI-TCL, the AH-corrected PPI-TCL, and estimated the PPI-TCL using "dual chamber entrainment" calculated as: [PPIV-TCL=Stim(AoV)+Stim(VoA)-PPIA].RESULTS: The PPI-TCL calculated by dual chamber entrainment highly correlated with the observed and AH-corrected PPI-TCL [R2=0.79 and 0.96, respectively, p<0.001]. A dual chamber entrainment PPI-TCL value of 80ms correctly differentiated all AVNRT from septal ORT cases, whereas the standard PPI-TCL and AH-corrected PPI-TCL methods were incorrect in 14% and 6% of cases, respectively. Dual chamber entrainment identified 3±10ms of additional decremental conduction beyond AH-prolongation, including four pathways with significant (>10ms) decrement.CONCLUSION: Dual chamber entrainment estimates the PPI-TCL value without using either the ventricular PPI or the TCL interval. This maneuver adjusts for all decremental conduction, including within concealed pathways, where a dual chamber entrainment PPI-TCL value >80ms favors AVNRT over ORT. This maneuver can be used to verify the observed PPI-TCL value in challenging cases.

  View details for PubMedID 30465902

• "If at First You Don't Succeed": Repeat Ablations in Young Patients With Supraventricular Tachycardia Rochelson, E., Clark, B. C., Janson, C. M., Ceresnak, S. R., Nappo, L., Pass, R. H. LIPPINCOTT WILLIAMS & WILKINS. 2018

  View details for Web of Science ID 000528619405276

• Incidence of Echocardiographic Abnormalities Following Pediatric SVT Ablation: Comparison of Cases Utilizing Fluoroscopy Alone to Cases with Adjunctive 3D Electroanatomic Mapping. Pediatric cardiology Gartenberg, A. J., Pass, R. H., Ceresnak, S., Nappo, L., Janson, C. M. 2018

  Abstract

  There are few data on the incidence of echocardiographic (echo) abnormalities following catheter ablation in children in the era of 3D mapping. Wide practice variation exists regarding routine post-ablation echo. We hypothesized a low incidence of clinically significant echo abnormalities following SVT ablation in otherwise healthy children. Single center data from 2009 to 2015 were reviewed; routine post-ablation echo was standard practice. Cases were categorized as utilizing fluoroscopy alone (FLUORO) or 3D mapping with a low fluoroscopic protocol (CARTO3). Congenital heart disease was excluded. Outcomes of interest included new valvular abnormalities, pericardial effusions, and wall motion abnormalities. Findings were compared to baseline studies when available and classified as normal/unchanged, clinically insignificant, or clinically significant. Outcomes were compared between FLUORO and CARTO3 groups. Of 347 ablations, 319 (92%) underwent post-procedural echo: 57% male; 55% FLUORO; mean age 13.4±3.6years. The most common ablation target was an accessory pathway (AP) in 66% (n=144 WPW, 66 concealed), followed by AVNRT in 32% (n=102). Radiofrequency (RF) energy was utilized in 82% (n=262). Post-ablation echos were normal in 81% (n=259). Clinically insignificant findings were seen in 18% (n=58), most commonly trivial-small pericardial effusions in 11% (n=34). Two significant findings required additional follow-up or treatment. There were no cases of wall motion abnormalities or clinically significant effusions. There were no differences in frequency of echo abnormalities between the FLUORO and CARTO3 groups. Clinically significant echocardiographic abnormalities are rare following SVT ablation in children with structurally normal hearts, independent of the use of 3D mapping.

  View details for PubMedID 30315340

• Utility of serial 12-lead electrocardiograms in children with Marfan syndrome CARDIOLOGY IN THE YOUNG Arunamata, A. A., Nguyen, C. T., Ceresnak, S. R., Dubin, A. M., Olson, I. L., Murphy, D. J., Tierney, E. 2018; 28 (8): 1009–13

  View details for DOI 10.1017/S1047951118000707

  View details for Web of Science ID 000439937200005

• Antibiotic Prophylaxis Practices in Pediatric Cardiac Implantable Electronic Device Procedures: A Survey of the Pediatric And Congenital Electrophysiology Society (PACES) PEDIATRIC CARDIOLOGY Chen, S. Y., Ceresnak, S. R., Motonaga, K. S., Trela, A., Hanisch, D., Dubin, A. M. 2018; 39 (6): 1129–33

  View details for DOI 10.1007/s00246-018-1865-0

  View details for Web of Science ID 000441721000009

• Utility of serial 12-lead electrocardiograms in children with Marfan syndrome. Cardiology in the young Arunamata, A. A., Nguyen, C. T., Ceresnak, S. R., Dubin, A. M., Olson, I. L., Murphy, D. J., Selamet Tierney, E. S. 2018: 1–5

  Abstract

  OBJECTIVES: The goal of this study was to assess the utility of serial electrocardiograms in routine follow-up of paediatric Marfan patients.METHODS: Children ⩽18 years who met the revised Ghent criteria for Marfan syndrome and received a 12-lead electrocardiogram and echocardiogram within a 3-month period were included. Controls were matched by age, body surface area, gender, race, and ethnicity, and consisted of patients assessed in clinic with a normal cardiac evaluation. Demographic, clinical, echocardiographic, and electrocardiographic data were collected.RESULTS: A total of 45 Marfan patients (10.8 [2.4-17.1] years) and 37 controls (12.8 [1.3-17.1] years) were included. Left atrial enlargement and left ventricular hypertrophy were more frequently present on 12-lead electrocardiogram of Marfan patients compared with controls (12 (27%) versus 0 (0%), p<0.001; and 8 (18%) versus 0 (0%), p=0.008, respectively); however, only two patients with left atrial enlargement on 12-lead electrocardiogram were confirmed to have left atrial enlargement by echocardiogram, and one patient had mild left ventricular hypertrophy by echocardiogram, not appreciated on 12-lead electrocardiogram. QTc interval was longer in Marfan patients compared with controls (427±16 versus 417±22 ms, p=0.03), with four Marfan patients demonstrating borderline prolonged QTc intervals for gender.CONCLUSIONS: While Marfan patients exhibited a higher frequency of left atrial enlargement and left ventricular hypertrophy on 12-lead electrocardiograms compared with controls, these findings were not supported by echocardiography. Serial 12-lead electrocardiograms in routine follow-up of asymptomatic paediatric Marfan patients may be more appropriate for a subgroup of Marfan patients only, specifically those with prolonged QTc interval at their baseline visit.

  View details for PubMedID 29972109

• Year in Review in Cardiac Electrophysiology. Circulation. Arrhythmia and electrophysiology Kapa, S., Davis, D. R., Park, D. S., Steinberg, B. A., Viswanathan, M. N., Tzou, W., Madhavan, M., Ceresnak, S. R., Wang, P. J. 2018; 11 (7): e006648

  View details for PubMedID 30012874

• Year in Review in Cardiac Electrophysiology CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY Kapa, S., Davis, D. R., Park, D. S., Steinberg, B. A., Viswanathan, M. N., Tzou, W., Madhavan, M., Ceresnak, S. R., Wang, P. J. 2018; 11 (7)

  View details for DOI 10.1161/CIRCEP.118.006648

  View details for Web of Science ID 000438922000016

• The "hidden" concealed left-sided accessory pathway: An uncommon cause of SVT in young people PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Pass, R. H., Liberman, L., Silver, E. S., Janson, C. M., Blaufox, A. D., Nappo, L., Ceresnak, S. R. 2018; 41 (4): 368–71

  Abstract

  Concealed left-sided accessory pathways (CLAP) are a cause of supraventricular tachycardia (SVT) in the young. Most are mapped with right ventricular (RV) apical/outflow pacing. Rarely, alternative means of mapping are required. We review our experience from three pediatric electrophysiology (EP) centers with a rare form of "hidden" CLAP.All patients <21 years undergoing EP study from 2008 to 2014 with a "hidden" CLAP (defined as an accessory pathway [AP] for which RV pacing at cycle lengths [CL] stable for mapping did not demonstrate eccentric retrograde conduction) were included.preexcitation. Demographic, procedural, and follow-up data were collected.A total of 23 patients met the criteria (median age, 14.3 years [range 7-21], weight, 51 kg [31-99]). 21 (96%) had SVT and one AFIB (4%). APs were adenosine sensitive in 7/20 patients (35%) and VA conduction was decremental in six (26%). CLAP conduction was demonstrable with orthodromic reentrant tachycardia in all patients, with RV extrastimulus testing in seven (30%) and with rapid RV pacing (

  View details for PubMedID 29327439

• Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study. JACC. Clinical electrophysiology Etheridge, S. P., Escudero, C. A., Blaufox, A. D., Law, I. H., Dechert-Crooks, B. E., Stephenson, E. A., Dubin, A. M., Ceresnak, S. R., Motonaga, K. S., Skinner, J. R., Marcondes, L. D., Perry, J. C., Collins, K. K., Seslar, S. P., Cabrera, M., Uzun, O., Cannon, B. C., Aziz, P. F., Kubus, P., Tanel, R. E., Valdes, S. O., Sami, S., Kertesz, N. J., Maldonado, J., Erickson, C., Moore, J. P., Asakai, H., Mill, L., Abcede, M., Spector, Z. Z., Menon, S., Shwayder, M., Bradley, D. J., Cohen, M. I., Sanatani, S. 2018; 4 (4): 433–44

  Abstract

  OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population.BACKGROUND: Children with WPW syndrome are at risk of sudden death.METHODS: This retrospective multicenter pediatric study identified 912 subjects≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrialfibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects.RESULTS: Case subjects (n= 96) were older and less likely than subjects (n= 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia.CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

  View details for PubMedID 30067481

• Antibiotic Prophylaxis Practices in Pediatric Cardiac Implantable Electronic Device Procedures: A Survey of the Pediatric And Congenital Electrophysiology Society (PACES). Pediatric cardiology Chen, S. Y., Ceresnak, S. R., Motonaga, K. S., Trela, A., Hanisch, D., Dubin, A. M. 2018

  Abstract

  Cardiac implantable electronic device (CIED) infections are associated with significant morbidity in the pediatric device population, with a tenfold higher risk of infection in children compared to adults. The 2010 American Heart Association (AHA) guidelines recommend a single dose of systemic antibiotic (ABX) prophylaxis prior to CIED implantation and no post-operative (OP) ABX. However, there is limited data regarding adherence to this recommendation among the pediatric community. To assess current clinical practices for CIED ABX prophylaxis in pediatrics; whether the AHA guidelines are being followed; and if not, the reasons for non-adherence. An anonymous web-based survey was sent to physician members of the Pediatric And Congenital Electrophysiology Society regarding ABX prophylaxis for new CIED implants and reoperations. 75 (25%) members responded. Only 7% of respondents follow the 2010 AHA guidelines. While all respondents give pre-OP IV ABX, 64% routinely treat patients with 24-h post-OP IV ABX with additional oral or IV therapy. 69% of respondents are cognizant of the guidelines but 88% of those cognizant do not follow the guidelines for a variety of reasons including lack of data and different substrate (pediatric patients). 79% stated that pediatric-specific data would be required for them to change their practice and follow the published guidelines. The majority of pediatric EP physicians who responded to this survey do not follow the current AHA guidelines on ABX prophylaxis and administer post-OP ABX. Most pediatric EP physicians believe that the increased risk of infection in children merits additional ABX.

  View details for PubMedID 29564522

• THE DEVELOPMENT AND EFFICACY OF A PEDIATRIC CARDIOLOGY FELLOWSHIP ONLINE PREPARATORY COURSE Motonaga, K., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J., Silverman, N., Hanley, F., Axelrod, D., Krawczeski, C., Ceresnak, S. ELSEVIER SCIENCE INC. 2018: 2622

  View details for DOI 10.1016/S0735-1097(18)33163-2

  View details for Web of Science ID 000429659705072

• Bridge to success: A better method of cryoablation for atrioventricular nodal reentrant tachycardia in children HEART RHYTHM Reddy, C. D., Ceresnak, S. R., Motonaga, K. S., Avasarala, K., Feller, C., Trela, A., Hanisch, D., Dubin, A. M. 2017; 14 (11): 1649–54

  Abstract

  Cryoablation for atrioventricular nodal reentrant tachycardia (AVNRT) is associated with higher recurrence rates than radiofrequency ablation (RFA). Junctional tachycardia marks procedural success with RFA, but no such indicator exists for cryoablation.The purpose of this study as to determine the impact of voltage mapping plus longer ablation lesions on midterm success of cryoablation for children with AVNRT.We performed a single-center retrospective analysis of pediatric patients with AVNRT who underwent cryoablation from 2011 to 2015. Patients ablated using a standard electroanatomic approach (control) were compared with patients ablated using voltage mapping (voltage group). In the voltage group, EnSite NavX navigation and visualization technology (St Jude Medical, St Paul, MN) was used to develop a "bridge" of lower voltage gradients (0.3-0.8 mV) of the posteroseptal right atrium to guide cryoablation. Kaplan-Meier analysis was used to determine freedom from recurrence of supraventricular tachycardia.In all, 122 patients were included (71 voltage, 51 control). There was no difference between groups regarding age, sex, or catheter-tip size. Short-term success was similar in both groups (98.5% voltage vs 92% control; P = .159), but recurrence rates were lower in the voltage group (0% vs 11%, P = .006). Follow-up time was shorter in the voltage group (15 ± 7 months vs 22 ± 17 months, P < .05). The 1-year freedom from recurrence was lower in the voltage group (100% vs 91.5%, P <.05). Ablation times were longer in the voltage group (43.7 ± 20.9 minutes vs 34.3 ± 20.5 minutes, P = .01), but overall procedure times were shorter in the voltage group (157 ± 40 minutes vs 198 ± 133 minutes; P = .018). No significant complication was seen in either group.Voltage gradient mapping and longer lesion time can decrease recurrence rates in pediatric patients with AVNRT.

  View details for PubMedID 28716699

• A multicenter review of ablation in the aortic cusps in young people. Pacing and clinical electrophysiology : PACE Nguyen, M. B., Ceresnak, S. R., Janson, C. M., Fishberger, S. B., Love, B. A., Blaufox, A. D., Motonaga, K. S., Dubin, A. M., Nappo, L., Pass, R. H. 2017

  Abstract

  Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients.A retrospective, descriptive study of aortic cusp ablation in five pediatric electrophysiology centers from 2008 to 2014 was performed. All patients <21 years of age who underwent ablation in the aortic cusps were included. Factors analyzed included patient demographics, procedural details, outcomes, and complications.Thirteen patients met inclusion criteria (median age 16 years [range 10-20.5] and median body surface area 1.58 m(2) [range 1.12-2.33]). Substrates for ablation included: nine premature ventricular contractions or sustained ventricular tachycardia (69%), two concealed anteroseptal accessory pathways (APs) (15%), one Wolff-Parkinson-White with an anteroseptal AP (8%), and one ectopic atrial tachycardia (8%). Three-dimensional electroanatomic mapping in combination with fluoroscopy was used in 12/13 (92%) patients. Standard 4-mm-tip radiofrequency (RF) current was used in 11/13 (85%) and low-power irrigated-tip RF in 2/13 (15%). Angiography was used in 13/13 and intracardiac echocardiography was additionally utilized in 3/13 (23%). Ablation locations included: eight noncoronary (62%), three left (23%), and two right (15%) cusps. Ablation was acutely successful in all patients. At median follow-up of 20 months, there was one recurrence of PVCs (8%). There were no ablation-related complications and no valvular injuries observed.Arrhythmias originating from the coronary cusps in this series were successfully and safely ablated in young people without injury to the coronary arteries or the aortic valve.

  View details for DOI 10.1111/pace.13126

  View details for PubMedID 28568013

• Ventricular pacing in single ventricles-A bad combination. Heart rhythm Bulic, A., Zimmerman, F. J., Ceresnak, S. R., Shetty, I., Motonaga, K. S., Freter, A., Trela, A. V., Hanisch, D., Russo, L., Avasarala, K., Dubin, A. M. 2017; 14 (6): 853-857

  Abstract

  Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population.To determine the longitudinal effect of VP in SV patients.SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology. Patients were excluded if a prepacing echocardiogram was not available. Echocardiogram and clinical parameters were compared at baseline (prepacing) and at last follow-up in the paced group, and in controls when they were at ages similar to those of their paced-group matches.Twenty-two paced and 53 control patients from 2 institutions were followed for similar durations (6.6±5 years vs 7.6±7.6 years; P = .59). There was no difference between groups regarding baseline ventricular function or the presence of moderate-to-severe atrioventricular valvar regurgitation (AVVR). Paced patients were more likely to develop moderate-to-severe systolic dysfunction (68% vs 15%; P < .01) and AVVR (55% vs 8%; P < .001) and require heart failure medications (65% vs 21%; P < .001). Chronic VP was also associated with a higher risk of transplantation or death (odds ratio, 4.9; 95% confidence interval, 1.05-22.7; P = .04).SV patients requiring chronic VP are at higher risk of developing moderate-to-severe ventricular dysfunction and AVVR with an increased risk of death or transplantation compared with controls. New strategies to either limit VP or improve synchronization in this vulnerable population is imperative.

  View details for DOI 10.1016/j.hrthm.2017.03.035

  View details for PubMedID 28528723

• Advances in Pediatric Cardiology Boot Camp: Boot Camp Training Promotes Fellowship Readiness and Enables Retention of Knowledge. Pediatric cardiology Ceresnak, S. R., Axelrod, D. M., Sacks, L. D., Motonaga, K. S., Johnson, E. R., Krawczeski, C. D. 2017

  Abstract

  We previously demonstrated that a pediatric cardiology boot camp can improve knowledge acquisition and decrease anxiety for trainees. We sought to determine if boot camp participants entered fellowship with a knowledge advantage over fellows who did not attend and if there was moderate-term retention of that knowledge. A 2-day training program was provided for incoming pediatric cardiology fellows from eight fellowship programs in April 2016. Hands-on, immersive experiences and simulations were provided in all major areas of pediatric cardiology. Knowledge-based examinations were completed by each participant prior to boot camp (PRE), immediately post-training (POST), and prior to the start of fellowship in June 2016 (F/U). A control group of fellows who did not attend boot camp also completed an examination prior to fellowship (CTRL). Comparisons of scores were made for individual participants and between participants and controls. A total of 16 participants and 16 control subjects were included. Baseline exam scores were similar between participants and controls (PRE 47 ± 11% vs. CTRL 52 ± 10%; p = 0.22). Participants' knowledge improved with boot camp training (PRE 47 ± 11% vs. POST 70 ± 8%; p < 0.001) and there was excellent moderate-term retention of the information taught at boot camp (PRE 47 ± 11% vs. F/U 71 ± 8%; p < 0.001). Testing done at the beginning of fellowship demonstrated significantly better scores in participants versus controls (F/U 71 ± 8% vs. CTRL 52 ± 10%; p < 0.001). Boot camp participants demonstrated a significant improvement in basic cardiology knowledge after the training program and had excellent moderate-term retention of that knowledge. Participants began fellowship with a larger fund of knowledge than those fellows who did not attend.

  View details for DOI 10.1007/s00246-016-1560-y

  View details for PubMedID 28161811

• Tachycardia cycle and atrioventricular nodal conduction properties in children with supraventricular tachycardia. Pacing and clinical electrophysiology : PACE Mills, M., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R. 2017; 40 (6): 745–47

  View details for PubMedID 28383202

• Response to the Letter to the Editor: Arrhythmias in the pediatric intensive care unit: a prospective study of the rates and predictors of arrhythmias in children without underlying cardiac disease CARDIOLOGY IN THE YOUNG Cassel-Choudhury, G. N., Aydin, S. I., Ushay, H. M., Ceresnak, S. R. 2017; 27 (1): 205-206

  View details for DOI 10.1017/S1047951116001475

  View details for Web of Science ID 000398104500032

• Early somatic mosaicism is a rare cause of long-QT syndrome PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA Priest, J. R., Gawad, C., Kahlig, K. M., Yu, J. K., O'Hara, T., Boyle, P. M., Rajamani, S., Clark, M. J., Garcia, S. T., Ceresnak, S., Harris, J., Boyle, S., Dewey, F. E., Malloy-Walton, L., Dunn, K., Grove, M., Perez, M. V., Neff, N. F., Chen, R., Maeda, K., Dubin, A., Belardinelli, L., West, J., Antolik, C., Macaya, D., Quertermous, T., Trayanova, N. A., Quake, S. R., Ashley, E. A. 2016; 113 (41): 11555-11560

  Abstract

  Somatic mosaicism, the occurrence and propagation of genetic variation in cell lineages after fertilization, is increasingly recognized to play a causal role in a variety of human diseases. We investigated the case of life-threatening arrhythmia in a 10-day-old infant with long QT syndrome (LQTS). Rapid genome sequencing suggested a variant in the sodium channel NaV1.5 encoded by SCN5A, NM_000335:c.5284G > T predicting p.(V1762L), but read depth was insufficient to be diagnostic. Exome sequencing of the trio confirmed read ratios inconsistent with Mendelian inheritance only in the proband. Genotyping of single circulating leukocytes demonstrated the mutation in the genomes of 8% of patient cells, and RNA sequencing of cardiac tissue from the infant confirmed the expression of the mutant allele at mosaic ratios. Heterologous expression of the mutant channel revealed significantly delayed sodium current with a dominant negative effect. To investigate the mechanism by which mosaicism might cause arrhythmia, we built a finite element simulation model incorporating Purkinje fiber activation. This model confirmed the pathogenic consequences of cardiac cellular mosaicism and, under the presenting conditions of this case, recapitulated 2:1 AV block and arrhythmia. To investigate the extent to which mosaicism might explain undiagnosed arrhythmia, we studied 7,500 affected probands undergoing commercial gene-panel testing. Four individuals with pathogenic variants arising from early somatic mutation events were found. Here we establish cardiac mosaicism as a causal mechanism for LQTS and present methods by which the general phenomenon, likely to be relevant for all genetic diseases, can be detected through single-cell analysis and next-generation sequencing.

  View details for DOI 10.1073/pnas.1607187113

  View details for PubMedID 27681629

• Is There a Difference in Tachycardia Cycle Length during SVT in Children with AVRT and AVNRT? Pacing and clinical electrophysiology : PACE Mills, M. F., Motonaga, K. S., Trela, A., Dubin, A. M., Avasarala, K., Ceresnak, S. R. 2016

  Abstract

  There are limited adult data suggesting the tachycardia cycle length (TCL) of atrioventricular reentry tachycardia (AVRT) is shorter than atrioventricular nodal reentry tachycardia (AVNRT), though little data exist in children. We sought to determine if there is a difference in TCL between AVRT and AVNRT in children.A single-center retrospective review of children with supraventricular tachycardia (SVT) from 2000 to 2015 was performed.Age ≤ 18 years, invasive electrophysiology study (EPS) confirming AVRT or AVNRT.Atypical AVNRT, congenital heart disease, antiarrhythmic medication use at time of EPS. Data were compared between patients with AVRT and AVNRT via t-test, χ(2) test, and linear regression.A total of 835 patients were included (12 ± 4 years, 52 ± 31 kg, TCL 321 ± 55 ms), 539 (65%) with AVRT (270 Wolff-Parkinson-White, 269 concealed pathways) and 296 (35%) with AVNRT. Patients with AVRT were younger (11.7 ± 4.1 years vs 13.0 ± 3.6 years, P < 0.001) and smaller (49 ± 22 kg vs 57 ± 43 kg, P < 0.001). In the baseline state, the TCL was shorter in AVRT than AVRNT (329 ± 51 ms vs 340 ± 60 ms, P = 0.04). In patients requiring isoproterenol to induce SVT, there was no difference in TCL (290 ± 49 ms vs 297 ± 49 ms, P = 0.26). When controlling for age, there was no difference in TCL between AVRT and AVNRT at baseline or on isoproterenol. The regression equation for TCL in the baseline state was TCL = 290 + 4 (age), indicating the TCL will increase by 4 ms above a baseline of 290 ms for each year of life.When controlling for age, there is no difference in the TCL between AVRT and AVNRT in children. Age, not tachycardia mechanism, is the most significant factor in predicting TCL.

  View details for DOI 10.1111/pace.12950

  View details for PubMedID 27653639

• IS THERE A DIFFERENCE IN TACHYCARDIA CYCLE LENGTH DURING SUPRAVENTRICULAR TACHYCARDIA IN CHILDREN WITH ATRIO-VENTRICULAR RECIPROCATING TACHYCARDIA AND ATRIO-VENTRICULAR NODAL RE-ENTRY TACHYCARDIA? Mills, M., Motonaga, K., Trela, A., Dubin, A., Avasarala, K., Ceresnak, S. ELSEVIER SCIENCE INC. 2016: 848

  View details for DOI 10.1016/S0735-1097(16)30849-X

  View details for Web of Science ID 000375188701692

• Unusual Outflow Tract Ventricular Tachycardia. Cardiac electrophysiology clinics Motonaga, K. S., Ceresnak, S. R., Hsia, H. H. 2016; 8 (1): 79-88

  Abstract

  Distinguishing premature ventricular contractions/ventricular tachycardia from the right ventricular outflow tract versus the left ventricular outflow tract can be difficult by electrocardiogram findings alone. A thorough understanding of the outflow tract anatomy and a systematic and meticulous approach to mapping of the ventricular outflow regions and great vessels increases the success rate and decreases the risk of damage to adjacent structures and the conduction system. The use of multimodality imaging, particularly real-time intracardiac echocardiographic guidance, is essential for defining anatomy, ensuring adequate catheter contact, and minimizing risks.

  View details for DOI 10.1016/j.ccep.2015.10.032

  View details for PubMedID 26920175

• A Pilot Study Assessing ECG versus ECHO Ventriculoventricular Optimization in Pediatric Resynchronization Patients. Journal of cardiovascular electrophysiology Punn, R., Hanisch, D., Motonaga, K. S., Rosenthal, D. N., Ceresnak, S. R., Dubin, A. M. 2016; 27 (2): 210-216

  Abstract

  Cardiac resynchronization therapy indications and management are well described in adults. Echocardiography (ECHO) has been used to optimize mechanical synchrony in these patients; however, there are issues with reproducibility and time intensity. Pediatric patients add challenges, with diverse substrates and limited capacity for cooperation. Electrocardiographic (ECG) methods to assess electrical synchrony are expeditious but have not been extensively studied in children. We sought to compare ECHO and ECG CRT optimization in children.Prospective, pediatric, single-center cross-over trial comparing ECHO and ECG optimization with CRT. Patients were assigned to undergo either ECHO or ECG optimization, followed for 6 months, and crossed-over to the other assignment for another 6 months. ECHO pulsed-wave tissue Doppler and 12-lead ECG were obtained for 5 VV delays. ECG optimization was defined as the shortest QRSD and ECHO optimization as the lowest dyssynchrony index. ECHOs/ECGs were interpreted by readers blinded to optimization technique. After each 6 month period, these data were collected: ejection fraction, velocimetry-derived cardiac index, quality of life, ECHO-derived stroke distance, M-mode dyssynchrony, study cost, and time. Outcomes for each optimization method were compared.From June 2012 to December 2013, 19 patients enrolled. Mean age was 9.1 ± 4.3 years; 14 (74%) had structural heart disease. The mean time for optimization was shorter using ECG than ECHO (9 ± 1 min vs. 68 ± 13 min, P < 0.01). Mean cost for charges was $4,400 ± 700 less for ECG. No other outcome differed between groups.ECHO optimization of synchrony was not superior to ECG optimization in this pilot study. ECG optimization required less time and cost than ECHO optimization.

  View details for DOI 10.1111/jce.12863

  View details for PubMedID 26515428

• Tricking CARTO: Cryoablation of Supraventricular Tachycardia in Children with Minimal Radiation Exposure Using the CARTO3 System PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Ceresnak, S. R., Nappo, L., Janson, C. M., Pass, R. H. 2016; 39 (1): 36-41

  Abstract

  CARTO3 is frequently used during ablation but is not designed to allow visualization of non-CARTO3 ablation catheters. We describe how cryoablation catheters can be visualized and recorded using CARTO3 with minimal fluoroscopy (FLUORO) usage.Retrospective review of patients ≤21 years undergoing cryoablation with CARTO3 from 2010 to 2013 for ablation of supraventricular tachycardia. After mapping with a Navistar catheter, the Navistar was removed and a cryocatheter was utilized. The cryocatheter was connected to the pin box via a jumper cable and the pin box was connected to the CARTO3 patient interface unit. Locations of ablation attempts with the cryocatheter were recorded with the "Create Snapshot" tool. Clinical characteristics and radiation doses were compared between patients undergoing cryoablation (cryoenergy [CRYO]) to an age- and diagnosis-matched control group (CONTROL) undergoing RF ablation.A total of 174 ablations were performed and 14 patients underwent cryoablation (CRYO, 13.3 ± 4.7 years, weight 42 ± 14 kg). Indications for cryoablation were: five atrioventricular nodal reentry tachycardia (36%), four ectopic atrial tachycardia (29%), three concealed accessory pathways (21%), and two Wolff-Parkinson-White syndromes (14%). Acute success was achieved in all patients (100%) with no complications and one recurrence (7%). The site of successful cryoablation was successfully recorded on the CARTO3 system in all cases. Radiation doses were low and not different from an age-, era-, and diagnosis-matched control group undergoing RF ablation (CRYO 3.2 ± 0.8 mGy vs CONTROL 1.6 ± 0.4 mGy, P = 0.07).Though a "closed" system, CARTO3 can be "tricked" to allow for the use of cryoablation, allowing clear catheter visualization, mapping, and recording of ablation lesions with minimal FLUORO usage.

  View details for DOI 10.1111/pace.12754

  View details for Web of Science ID 000368085000006

• Tricking CARTO: Cryoablation of Supraventricular Tachycardia in Children with Minimal Radiation Exposure Using the CARTO3 System. Pacing and clinical electrophysiology : PACE Ceresnak, S. R., Nappo, L., Janson, C. M., Pass, R. H. 2016; 39 (1): 36-41

  Abstract

  CARTO3 is frequently used during ablation but is not designed to allow visualization of non-CARTO3 ablation catheters. We describe how cryoablation catheters can be visualized and recorded using CARTO3 with minimal fluoroscopy (FLUORO) usage.Retrospective review of patients ≤21 years undergoing cryoablation with CARTO3 from 2010 to 2013 for ablation of supraventricular tachycardia. After mapping with a Navistar catheter, the Navistar was removed and a cryocatheter was utilized. The cryocatheter was connected to the pin box via a jumper cable and the pin box was connected to the CARTO3 patient interface unit. Locations of ablation attempts with the cryocatheter were recorded with the "Create Snapshot" tool. Clinical characteristics and radiation doses were compared between patients undergoing cryoablation (cryoenergy [CRYO]) to an age- and diagnosis-matched control group (CONTROL) undergoing RF ablation.A total of 174 ablations were performed and 14 patients underwent cryoablation (CRYO, 13.3 ± 4.7 years, weight 42 ± 14 kg). Indications for cryoablation were: five atrioventricular nodal reentry tachycardia (36%), four ectopic atrial tachycardia (29%), three concealed accessory pathways (21%), and two Wolff-Parkinson-White syndromes (14%). Acute success was achieved in all patients (100%) with no complications and one recurrence (7%). The site of successful cryoablation was successfully recorded on the CARTO3 system in all cases. Radiation doses were low and not different from an age-, era-, and diagnosis-matched control group undergoing RF ablation (CRYO 3.2 ± 0.8 mGy vs CONTROL 1.6 ± 0.4 mGy, P = 0.07).Though a "closed" system, CARTO3 can be "tricked" to allow for the use of cryoablation, allowing clear catheter visualization, mapping, and recording of ablation lesions with minimal FLUORO usage.

  View details for DOI 10.1111/pace.12754

  View details for PubMedID 26412504

• Three-Catheter Technique for Ablation of Left-Sided Accessory Pathways in Wolff-Parkinson-White is Less Expensive and Equally Successful When Compared to a Five-Catheter Technique PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Capone, C. A., Ceresnak, S. R., Nappo, L., Gates, G. J., Schechter, C. B., Pass, R. H. 2015; 38 (12): 1405-1411

  Abstract

  To compare the efficacy, safety, and cost-effectiveness of a three-catheter approach with a conventional five-catheter approach for the mapping and ablation of supraventricular tachycardia in pediatric patients with Wolff-Parkinson-White Syndrome (WPW) and concealed accessory pathways (APs).A retrospective review from 2008 to 2012 of patients less than 21 years with WPW who underwent a three-catheter radiofrequency (RF) ablation of a left-sided AP (ablation, right ventricular [RV] apical, and coronary sinus [CS] decapolar catheters) was performed. The three-catheter group was compared to a control group who underwent a standard five-catheter (ablation, RV apical, CS decapolar, His catheter, and right atrial catheter) ablation for the treatment of left-sided WPW or concealed AP. Demographics, ablation outcomes, and costs were compared between groups.Twenty-eight patients met inclusion criteria with 28 control patients. The groups did not differ in gender, age, weight, or body surface area. Locations of the AP on the mitral annulus were similar between the groups. All patients were ablated via transseptal approach. Note that 28 of 28 in the three-catheter group (100%) and 27 of 28 (96%) controls were acutely successfully ablated (P = 0.31). No complications were encountered. There was no difference in procedural time, time to loss of AP conduction, or number of RF applications. Use of the three-catheter technique resulted in a total savings of $2,465/case, which includes the $680 savings from using fewer catheters as well as the savings from a shortened procedure time.Ablation in patients with WPW and a left-sided AP can be performed using three catheters with similar efficacy and safety while offering significant cost savings compared to a conventional five-catheter approach.

  View details for DOI 10.1111/pace.12742

  View details for Web of Science ID 000368084500006

• Three-Catheter Technique for Ablation of Left-Sided Accessory Pathways in Wolff-Parkinson-White is Less Expensive and Equally Successful When Compared to a Five-Catheter Technique. Pacing and clinical electrophysiology : PACE Capone, C. A., Ceresnak, S. R., Nappo, L., Gates, G. J., Schechter, C. B., Pass, R. H. 2015; 38 (12): 1405-11

  Abstract

  To compare the efficacy, safety, and cost-effectiveness of a three-catheter approach with a conventional five-catheter approach for the mapping and ablation of supraventricular tachycardia in pediatric patients with Wolff-Parkinson-White Syndrome (WPW) and concealed accessory pathways (APs).A retrospective review from 2008 to 2012 of patients less than 21 years with WPW who underwent a three-catheter radiofrequency (RF) ablation of a left-sided AP (ablation, right ventricular [RV] apical, and coronary sinus [CS] decapolar catheters) was performed. The three-catheter group was compared to a control group who underwent a standard five-catheter (ablation, RV apical, CS decapolar, His catheter, and right atrial catheter) ablation for the treatment of left-sided WPW or concealed AP. Demographics, ablation outcomes, and costs were compared between groups.Twenty-eight patients met inclusion criteria with 28 control patients. The groups did not differ in gender, age, weight, or body surface area. Locations of the AP on the mitral annulus were similar between the groups. All patients were ablated via transseptal approach. Note that 28 of 28 in the three-catheter group (100%) and 27 of 28 (96%) controls were acutely successfully ablated (P = 0.31). No complications were encountered. There was no difference in procedural time, time to loss of AP conduction, or number of RF applications. Use of the three-catheter technique resulted in a total savings of $2,465/case, which includes the $680 savings from using fewer catheters as well as the savings from a shortened procedure time.Ablation in patients with WPW and a left-sided AP can be performed using three catheters with similar efficacy and safety while offering significant cost savings compared to a conventional five-catheter approach.

  View details for DOI 10.1111/pace.12742

  View details for PubMedID 26400468

• Right-sided subcutaneous implantable cardioverter-defibrillator placement in a patient with dextrocardia, tetralogy of Fallot, and conduction disease. HeartRhythm case reports Ceresnak, S. R., Motonaga, K. S., Rogers, I. S., Viswanathan, M. N. 2015; 1 (4): 186-189

  View details for DOI 10.1016/j.hrcr.2015.02.001

  View details for PubMedID 28491545

• Reducing patient radiation exposure during paediatric SVT ablations: use of CARTO (R) 3 in concert with "ALARA" principles profoundly lowers total dose CARDIOLOGY IN THE YOUNG Pass, R. H., Gates, G. G., Gellis, L. A., Nappo, L., Ceresnak, S. R. 2015; 25 (5): 963-968

  Abstract

  "ALARA - As Low As Reasonably Achievable" protocols reduce patient radiation dose. Addition of electroanatomical mapping may further reduce dose.From 6/11 to 4/12, a novel ALARA protocol was utilised for all patients undergoing supraventricular tachycardia ablation, including low frame rates (2-3 frames/second), low fluoro dose/frame (6-18 nGy/frame), and other techniques to reduce fluoroscopy (ALARA). From 6/12 to 3/13, use of CARTO® 3 (C3) with "fast anatomical mapping" (ALARA+C3) was added to the ALARA protocol. Intravascular echo was not utilised. Demographics, procedural, and radiation data were analysed and compared between the two protocols.A total of 75 patients were included: 42 ALARA patients, and 33 ALARA+C3 patients. Patient demographics were similar between the two groups. The acute success rate in ALARA was 95%, and 100% in ALARA+C3; no catheterisation-related complications were observed. Procedural time was 125.7 minutes in the ALARA group versus 131.4 in ALARA+C3 (p=0.36). Radiation doses were significantly lower in the ALARA+C3 group with a mean air Kerma in ALARA+C3 of 13.1±28.3 mGy (SD) compared with 93.8±112 mGy in ALARA (p<0.001). Mean dose area product was 92.2±179 uGym2 in ALARA+C3 compared with 584±687 uGym2 in ALARA (p<0.001). Of the 33 subjects (42%) in the ALARA+C3 group, 14 received ⩽1 mGy exposure. The ALARA+C3 dosages are the lowest reported for a combined electroanatomical-fluoroscopy technique.Addition of CARTO® 3 to ALARA protocols markedly reduced radiation exposure to young people undergoing supraventricular tachycardia ablation while allowing for equivalent procedural efficacy and safety.

  View details for DOI 10.1017/S1047951114001474

  View details for Web of Science ID 000355822400018

  View details for PubMedID 25155609

• Diminished exercise capacity and chronotropic incompetence in pediatric patients with congenital complete heart block and chronic right ventricular pacing. Heart rhythm Motonaga, K. S., Punn, R., Axelrod, D. M., Ceresnak, S. R., Hanisch, D., Kazmucha, J. A., Dubin, A. M. 2015; 12 (3): 560-565

  Abstract

  Chronic right ventricular (RV) pacing has been associated with decreased exercise capacity and left ventricular (LV) function in adults with congenital complete atrioventricular block (CCAVB), but not in children.The purpose of this study was to evaluate the exercise capacity and LV function in pediatric patients with CCAVB receiving chronic RV pacing.We prospectively evaluated pediatric patients with isolated CCAVB receiving atrial synchronous RV pacing for at least 5 years. Supine bicycle ergometry was performed, and LV ejection fraction (EF) was evaluated by echocardiography.Ten CCAVB subjects and 31 controls were matched for age, gender, and body surface area. CCAVB subjects had normal resting EF (63.1% ± 4.0%) and had been paced for 7.9 ± 1.4 years. Exercise testing demonstrated reduced functional capacity in CCAVB patients compared to controls with a lower VO2peak (26.0 ± 6.6 mL/kg/min vs 39.9 ± 7.0 mL/kg/min, P <.001), anaerobic threshold (15.6 ± 3.9 mL/kg/min vs 18.8 ± 2.7 mL/kg/min, P = .007), and oxygen uptake efficiency slope (1210 ± 406 vs 1841 ± 452, P <.001). Maximum heart rate (165 ± 8 bpm vs 185 ± 9 bpm, P <.001) and systolic blood pressure (159 ± 17 mm Hg vs 185 ± 12 mm Hg, P <.019) also were reduced in CCAVB patients despite maximal effort (respiratory exchange ratio 1.2 ± 0.1). EF was augmented with exercise in controls but not in CCAVB patients (13.2% ± 9.3% vs 0.2% ± 4.8% increase, P <.001).Clinically asymptomatic children with chronic RV pacing due to CCAVB have significant reductions in functional capacity accompanied by chronotropic incompetence and inability to augment EF with exercise.

  View details for DOI 10.1016/j.hrthm.2014.11.036

  View details for PubMedID 25433143

• The Prevalence of Arrhythmias, Predictors for Arrhythmias, and Safety of Exercise Stress Testing in Children PEDIATRIC CARDIOLOGY Ghosh, R. M., Gates, G. J., Walsh, C. A., Schiller, M. S., Pass, R. H., Ceresnak, S. R. 2015; 36 (3): 584-590

  Abstract

  Exercise testing is commonly performed in children for evaluation of cardiac disease. Few data exist, however, on the prevalence, types of arrhythmias, predictors for arrhythmias, and safety of exercise testing in children. A retrospective review of all patients ≤21 years undergoing exercise testing at our center from 2008 to 2012 was performed. Patients with clinically relevant arrhythmias were compared to those not experiencing a significant arrhythmia. 1,037 tests were performed in 916 patients. The mean age was 14 ± 4 years, 537 (55 %) were male, 281 (27 %) had congenital heart disease, 178 (17 %) had a history of a prior arrhythmia, and 17 (2 %) had a pacemaker or ICD. 291 (28 %) patients had a rhythm disturbance during the procedure. Clinically important arrhythmias were noted in 34 (3 %) patients and included: 19 (1.8 %) increasing ectopy with exercise, 5 (0.5 %) VT, 5 (0.5 %) second degree AV block, 3 (0.3 %) SVT, and 2 (0.2 %) AFIB. On multivariate logistic regression, variables associated with the development of clinically relevant arrhythmias included severe left ventricular (LV) dysfunction on echo (OR 1.99, CI 1.20-3.30) and prior history of a documented arrhythmia (OR 2.94, CI 1.25-6.88). There were no adverse events related to testing with no patient requiring cardioversion, defibrillation, or acute anti-arrhythmic therapy. A total of 28 % of children developed a rhythm disturbance during exercise testing and 3 % were clinically important. Severe LV dysfunction and a history of documented arrhythmia were associated with the development of a clinically important arrhythmia.

  View details for DOI 10.1007/s00246-014-1053-9

  View details for Web of Science ID 000350034000019

  View details for PubMedID 25384613

• Ventricular lead redundancy to prevent cardiovascular events and sudden death from lead fracture in pacemaker-dependent children. Heart rhythm Ceresnak, S. R., Perera, J. L., Motonaga, K. S., Avasarala, K., Malloy-Walton, L., Hanisch, D., Punn, R., Maeda, K., Reddy, V. M., Doan, L. N., Kirby, K., Dubin, A. M. 2015; 12 (1): 111-116

  Abstract

  Children requiring a permanent epicardial pacemaker(PM) traditionally have a single lead placed on the right ventricle. Lead failure in pacemaker dependent(PMD) children, however, can result in cardiovascular events(CVE) and death.To determine if redundant ventricular lead systems(RVLS) can safeguard against CVE and death in PMD children.Single-center study of PMD patients undergoing placement of RVLS from 2002-2013. Patients ≤ 21 years of age who were PMD were included. Patients with biventricular systems(BiV) systems placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle(SiV).769 patients underwent PM/ICD placement with 76 BiV implants and there were 49 PMD patients(6%). 13 patients underwent implantation of a RVLS. There was no difference between the RVLS group(n=13) and SiV PMD control group(n=24) with regard to age(RVLS 9.5±5.8 vs. SiV 9.4±6.7 years; p=0.52), weight(RVLS 38.2±32.6 vs. SiV 35.2±29.3 kg; p=0.62), indication for pacing, procedural complications or time to follow-up. There were 2 lead fractures (17%) in the RVLS group(mean follow-up 3.8±2.9 years) with no deaths or presentations with CVE. The SiV control group had 3 lead fractures (13%)(mean follow-up 2.8±2.9 years), with no deaths, but all 3 patients presented with CVE and required emergent PM placement.RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVEs in the event of lead failure in PMD patients.

  View details for DOI 10.1016/j.hrthm.2014.09.056

  View details for PubMedID 25277988

• Improving ECG Services at a Children's Hospital: Implementation of a Digital ECG System. International journal of pediatrics Osei, F. A., Gates, G. J., Choi, S. J., Hsu, D. T., Pass, R. H., Ceresnak, S. R. 2015; 2015: 697149-?

  Abstract

  Background. The use of digital ECG software and services is becoming common. We hypothesized that the introduction of a completely digital ECG system would increase the volume of ECGs interpreted at our children's hospital. Methods. As part of a hospital wide quality improvement initiative, a digital ECG service (MUSE, GE) was implemented at the Children's Hospital at Montefiore in June 2012. The total volume of ECGs performed in the first 6 months of the digital ECG era was compared to 18 months of the predigital era. Predigital and postdigital data were compared via t-tests. Results. The mean ECGs interpreted per month were 53 ± 16 in the predigital era and 216 ± 37 in the postdigital era (p < 0.001), a fourfold increase in ECG volume after introduction of the digital system. There was no significant change in inpatient or outpatient service volume during that time. The mean billing time decreased from 21 ± 27 days in the postdigital era to 12 ± 5 days in the postdigital era (p < 0.001). Conclusion. Implementation of a digital ECG system increased the volume of ECGs officially interpreted and reported.

  View details for DOI 10.1155/2015/697149

  View details for PubMedID 26451150

  View details for PubMedCentralID PMC4584242

• Arrhythmias in the paediatric intensive care unit: a prospective study of the rates and predictors of arrhythmias in children without underlying cardiac disease. Cardiology in the young Cassel-Choudhury, G. N., Aydin, S. I., Toedt-Pingel, I., Ushay, H. M., Killinger, J. S., Cohen, H. W., Ceresnak, S. R. 2014: 1-9

  Abstract

  Arrhythmias are common in patients admitted to the paediatric intensive care unit. We sought to identify the rates of occurrence and types of arrhythmias, and determine whether an arrhythmia was associated with illness severity and paediatric intensive care unit length of stay.This is a prospective, observational study of all patients admitted to the paediatric intensive care unit at the Children's Hospital at Montefiore from March to June 2012. Patients with cardiac disease or admitted for the treatment of primary arrhythmias were excluded. Clinical and laboratory data were collected and telemetry was reviewed daily. Tachyarrhythmias were identified as supraventricular tachycardia, ventricular tachycardia, and arrhythmias causing haemodynamic compromise or for which an intervention was performed.A total of 278 patients met the inclusion criteria and were analysed. There were 97 incidences of arrhythmia in 53 patients (19%) and six tachyarrhythmias (2%). The most common types of arrhythmias were junctional rhythm (38%), premature atrial contractions (24%), and premature ventricular contractions (22%). Tachyarrhythmias included three supraventricular tachycardia (50%) and three ventricular tachycardia (50%). Of the six tachyarrhythmias, four were related to placement or migration of central venous lines and two occurred during aminophylline infusion. Patients with an arrhythmia had longer duration of mechanical ventilation and paediatric intensive care unit stay (p<0.001). In multivariate analysis, central venous lines (odds ratio 3.1; 95% confidence interval 1.3-7.2, p=0.009) and aminophylline use (odds ratio 5.1; 95% confidence interval 1.7-14.9, p=0.003) were independent predictors for arrhythmias.Arrhythmias were common in paediatric intensive care unit patients (19%), although tachyarrhythmias occurred rarely (2%). Central venous lines and use of aminophylline were identified as two clinical factors that may be associated with development of an arrhythmia.

  View details for DOI 10.1017/S1047951114002339

  View details for PubMedID 25434920

• Catecholaminergic polymorphic ventricular tachycardia in a child with Brugada pattern on ECG: One patient with two diseases? HEART RHYTHM Janson, C. M., Ceresnak, S. R., Chung, W. K., Pass, R. H. 2014; 11 (11): 2101-2104

  View details for DOI 10.1016/j.hrthm.2014.07.010

  View details for Web of Science ID 000344480000037

• Catecholaminergic polymorphic ventricular tachycardia in a child with Brugada pattern on ECG: one patient with two diseases? Heart rhythm Janson, C. M., Ceresnak, S. R., Chung, W. K., Pass, R. H. 2014; 11 (11): 2101-2104

  View details for DOI 10.1016/j.hrthm.2014.07.010

  View details for PubMedID 25016147

• Molecular diagnosis of long QT syndrome at 10 days of life by rapid whole genome sequencing HEART RHYTHM Priest, J. R., Ceresnak, S. R., Dewey, F. E., Malloy-Walton, L. E., Dunn, K., Grove, M. E., Perez, M. V., Maeda, K., Dubin, A. M., Ashley, E. A. 2014; 11 (10): 1707-1713

  Abstract

  The advent of clinical next generation sequencing is rapidly changing the landscape of rare disease medicine. Molecular diagnosis of long QT syndrome (LQTS) can impact clinical management, including risk stratification and selection of pharmacotherapy based on the type of ion channel affected, but results from current gene panel testing requires 4 to 16 weeks before return to clinicians.A term female infant presented with 2:1 atrioventricular block and ventricular arrhythmias consistent with perinatal LQTS, requiring aggressive treatment including epicardial pacemaker, and cardioverter-defibrillator implantation and sympathectomy on day of life two. We sought to provide a rapid molecular diagnosis for optimization of treatment strategies.We performed CLIA-certified rapid whole genome sequencing (WGS) with a speed-optimized bioinformatics platform to achieve molecular diagnosis at 10 days of life.We detected a known pathogenic variant in KCNH2 that was demonstrated to be paternally inherited by followup genotyping. The unbiased assessment of the entire catalog of human genes provided by whole genome sequencing revealed a maternally inherited variant of unknown significance in a novel gene.Rapid clinical WGS provides faster and more comprehensive diagnostic information by 10 days of life than standard gene-panel testing. In selected clinical scenarios such as perinatal LQTS, rapid WGS may be able to provide more timely and clinically actionable information than a standard commercial test.

  View details for DOI 10.1016/j.hrthm.20l4.06.030

  View details for Web of Science ID 000343112200012

• Molecular diagnosis of long QT syndrome at 10 days of life by rapid whole genome sequencing. Heart rhythm Priest, J. R., Ceresnak, S. R., Dewey, F. E., Malloy-Walton, L. E., Dunn, K., Grove, M. E., Perez, M. V., Maeda, K., Dubin, A. M., Ashley, E. A. 2014; 11 (10): 1707-1713

  Abstract

  The advent of clinical next generation sequencing is rapidly changing the landscape of rare disease medicine. Molecular diagnosis of long QT syndrome (LQTS) can impact clinical management, including risk stratification and selection of pharmacotherapy based on the type of ion channel affected, but results from current gene panel testing requires 4 to 16 weeks before return to clinicians.A term female infant presented with 2:1 atrioventricular block and ventricular arrhythmias consistent with perinatal LQTS, requiring aggressive treatment including epicardial pacemaker, and cardioverter-defibrillator implantation and sympathectomy on day of life two. We sought to provide a rapid molecular diagnosis for optimization of treatment strategies.We performed CLIA-certified rapid whole genome sequencing (WGS) with a speed-optimized bioinformatics platform to achieve molecular diagnosis at 10 days of life.We detected a known pathogenic variant in KCNH2 that was demonstrated to be paternally inherited by followup genotyping. The unbiased assessment of the entire catalog of human genes provided by whole genome sequencing revealed a maternally inherited variant of unknown significance in a novel gene.Rapid clinical WGS provides faster and more comprehensive diagnostic information by 10 days of life than standard gene-panel testing. In selected clinical scenarios such as perinatal LQTS, rapid WGS may be able to provide more timely and clinically actionable information than a standard commercial test.

  View details for DOI 10.1016/j.hrthm.2014.06.030

  View details for PubMedID 24973560

• Effects of Ventilation and Catheter Position on Catheter Movement on the Tricuspid Annulus during Ablation in Children. Pacing and clinical electrophysiology : PACE Ceresnak, S. R., Kahana, M., Zucker, H. A., Mann, G., Nappo, L., Pass, R. H. 2014; 37 (8): 1051-1057

  Abstract

  There are little data on the effect of catheter position and mechanical ventilation on ablation catheter stability during electrophysiology study in children. We sought to determine the magnitude of catheter movement with mechanical ventilation, the effect of ventilation maneuvers on catheter movement, and to compare the degree of movement observed between the right lateral (RL) and right posteroseptal (RPS) regions.From June 2012 to June 2013, patients ≤21 years of age undergoing ablation for supraventricular tachycardia with CARTO® 3 (Biosense Webster, Diamond Bar, CA, USA) were included. During mapping the ablation catheter was placed in the RPS and RL regions and the magnitude of catheter movement (mm) was measured using CARTO® 3. Measurements were made during routine ventilation and with a maximal inspiration maneuver between end-expiration (ENDEX) and peak-inspiration (PEAKINS).Twenty-one patients were included: 12 males (57%), age 13 ± 3 years, weight 55 ± 14 kg. Indications for ablation were: 10 Wolff-Parkinson-White, seven atrioventricular node re-entry tachycardia, four concealed accessory pathway. Mechanical ventilation was used in all cases. The magnitude of catheter movement was 3.6 ± 1.7 mm with routine ventilation and 6.2 ± 4.1 mm between ENDEX and PEAKINS (P ≤ 0.01). Catheter movement was greater in the RL compared to the RPS region with routine ventilation (RL 4.3 ± 1.6 vs RPS 3.0 ± 1.5; P < 0.01) and between ENDEX and PEAKINS (RL 8.3 ± 4.7 vs RPS 4.0 ± 1.7; P < 0.01).Ventilation and catheter position both have significant impact on the degree of catheter movement during ablation. Movement was greatest in the RL position. This may partially explain the lower success rates of ablation in the RL region.

  View details for DOI 10.1111/pace.12393

  View details for PubMedID 24666025

• Ablating the anteroseptal accessory pathway-ablation via the right internal jugular vein may improve safety and efficacy JOURNAL OF INTERVENTIONAL CARDIAC ELECTROPHYSIOLOGY Dilorenzo, M. P., Pass, R. H., Nappo, L., Ceresnak, S. R. 2012; 35 (3): 293-299

  Abstract

  Ablation of anteroseptal accessory pathways have historically been associated with lower success rates and a higher risk of AV nodal injury due to close proximity to the compact AV node. We describe the technique of ablation of anteroseptal APs via the right internal jugular vein (RIJV).A retrospective analysis of all patients undergoing EP study and ablation at the Children's Hospital at Montefiore from 2008 to 2011 for SVT and/or WPW was performed. All patients less than 21 years of age who underwent ablation from the RIJV for either WPW or a concealed accessory pathway located in the anteroseptal region were included.A total of 16 patients met inclusion criteria and were the subject of this analysis. Twelve patients had WPW (75%) and four had a concealed AP (25%). Ablation was acutely successful in 94% of patients (15/16). In one patient, ablation was deferred due to close proximity to the compact AV node and risk of AV nodal injury. Radiofrequency (RF) was used in 14 patients and cryoenergy in two patients. There was no difference in WB CL pre- and post-ablation (p = 0.19). There were no complications encountered. At a mean follow-up of 15 ± 12 months, there were no recurrences.Ablation of APs in the right anteroseptal region can safely and effectively be performed via the RIJV with a success rate of 94%. This technique should be considered for ablation of APs located in the anterior septum.

  View details for DOI 10.1007/s10840-012-9699-9

  View details for Web of Science ID 000311313000007

  View details for PubMedID 22869385

• Characteristics of ventricular tachycardia arising from the inflow region of the right ventricle JOURNAL OF ELECTROCARDIOLOGY Ceresnak, S. R., Pass, R. H., Krumerman, A. K., Kim, S. G., Nappo, L., Fisher, J. D. 2012; 45 (4): 385-390

  Abstract

  Ventricular tachycardia (VT) arising from the right ventricular inflow (RVI) region is uncommon. There is minimal literature on the clinical and electrocardiographic characteristics of RVI VT.A retrospective analysis of patients with RVI VT who underwent electrophysiology study between 2006 and 2011 was performed. Patients with structural heart disease (including arrhythmogenic right ventricular dysplasia) were excluded.Seventy patients underwent an electrophysiology study for VT arising from the right ventricle during the study period. Nine patients (13%) met the inclusion criteria for RVI VT and were the subject of this analysis. The median age was 46 years (range, 14-71), and VT cycle length was 295 milliseconds (range, 279-400 milliseconds). All VTs had an left bundle-branch block morphology. An inferiorly directed QRS axis was noted in 7 (78%) of 9 patients and a left superior axis in 2 (22%) of 9 patients. A QS or rS pattern was noted in all patients in aVR and V(1). A transition from S to R wave occurred in V(3) to V(5) in all patients, with 78% of the patients transitioning in V(4) or V(5). Ablation was attempted in 8 (89%) of 9 patients and was successful in 6 (67%) of 9 patients. Ablation was limited in all unsuccessful patients due to the proximity to the His and risk of complete heart block.Electrocardiographic findings of a left bundle-branch block with a normal QRS axis, QS or rS patterns in aVR and V(1), and late S to R transition (V(4)/V(5)) are commonly found in RVI VT. Because of the proximity to the His, ablation of RVI VT may be more challenging than that of right ventricular outflow tract VT.

  View details for DOI 10.1016/j.jelectrocard.2012.03.009

  View details for Web of Science ID 000306153000010

  View details for PubMedID 22554461

• Clinical and Electrophysiologic Characteristics of Antidromic Tachycardia in Children with Wolff-Parkinson-White Syndrome PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Ceresnak, S. R., Tanel, R. E., Pass, R. H., Liberman, L., Collins, K. K., Van Hare, G. F., Gates, G. J., Dubin, A. M. 2012; 35 (4): 480-488

  Abstract

  Antidromic reciprocating tachycardia (ART) is a rare form of wide complex tachycardia in children with Wolff-Parkinson-White syndrome (WPW). The incidence and electrophysiologic characteristics of ART in children with WPW have not been well described.A multicenter retrospective analysis of all patients with WPW undergoing electrophysiology (EP) study from 1990 to 2009 was performed. Patients with clinical or inducible ART were included.A total of 1,147 patients with WPW underwent EP study and 30 patients had ART (2.6%) and were the subject of this analysis. The mean age was 16±3 years, weight was 65±16 kg, and tachycardia cycle length was 305±55 ms. There were two patients (7%) with congenital heart disease (both with Ebstein's anomaly). Four patients (13%) had more than one accessory pathway (AP). The location of the AP was left sided in 53% of patients and right sided in 47%, with septal location and left lateral pathways most commonly involved. AP conduction was found to be high risk in 17 patients (57%). Ablation was not attempted in two patients (7%) due to proximity to the HIS and risk of heart block. Ablation was acutely successful in 93% of the patients in whom it was attempted.ART is a rare finding in children undergoing EP study. Over half of the patients with ART were found to be high risk and multiple AP were uncommon. Unlike the adult population, ART occurred commonly with septal APs.

  View details for DOI 10.1111/j.1540-8159.2011.03317.x

  View details for Web of Science ID 000302540300024

  View details for PubMedID 22324823

• Late Onset Ictal Asystole in Refractory Epilepsy PEDIATRIC NEUROLOGY Beal, J. C., Sogawa, Y., Ceresnak, S. R., Mahgerefteh, J., Moshe, S. L. 2011; 45 (4): 253-255

  Abstract

  Ictal asystole is a cardiac phenomenon associated with epileptic seizures, and may play a role in sudden unexplained death in epilepsy. We present a 17-year-old boy with chronic intractable epilepsy and a vagus nerve stimulator who developed ictal asystole many years after the onset of epilepsy. The asystole was not linked to the vagus nerve stimulator, and ultimately necessitated the placement of a cardiac pacemaker. A cardiac pacemaker and vagus nerve stimulator can be safely used simultaneously after careful testing during placement. The onset of asystolic events many years after the onset of epilepsy suggests that repeated seizures may exert long-term effects on cardiac function.

  View details for DOI 10.1016/j.pediatrneurol.2011.07.005

  View details for Web of Science ID 000295244900009

  View details for PubMedID 21907888

• Electrocardiograms Transmitted via Facsimile May Not Allow Accurate Interval Interpretation PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Farooqi, K. M., Ceresnak, S. R., Freeman, K., Pass, R. H. 2011; 34 (10): 1283-1287

  Abstract

  Electrocardiograms (ECGs) are sent via facsimile by pediatricians and psychiatrists to cardiologists for assessment. The validity of this method of transmission has not been established. ECGs were collected from 100 consecutive patients from the cardiology clinic of the Children's Hospital at Montefiore. The ECGs were faxed and also electronically faxed (efax) and printed. Two electrophysiologists (EP1 and EP2) interpreted the intervals on original, faxed, and efaxed ECGs and intervals (RR, PR, and QT) were compared. A three-way analysis of variance to examine differences between raters, among ECG intervals, and among methods (repeated factor) was performed. Because no interaction terms were significant, a Duncan's multiple range test was used to evaluate where differences occurred among the three intervals and three methods, given these main effects were significant. The difference between raters EP1 and EP2 was not significant (P = 0.6681). Although the interval measurements of the faxed and efaxed ECGs were not significantly different from each other (P > 0.05), each was significantly different from the original across all three ECG intervals and both raters (P = 0.0138). The RR interval yielded mean (SD) values for the original, faxed, and efaxed methods of 0.6986 seconds (0.2074), 0.6646 seconds (0.1938), and 0.6838 seconds (0.1935), respectively. For the QT interval, the mean (SD) values for the original, faxed, and efaxed methods were 0.3370 seconds (0.0524), 0.32134 seconds (0.0466), and 0.3284 seconds (0.0515), respectively. ECG transmission via facsimile or efax may introduce significant distortion of the intervals. Alternative means of sending ECGs for interpretation should be considered.

  View details for DOI 10.1111/j.1540-8159.2011.03158.x

  View details for Web of Science ID 000296051400016

  View details for PubMedID 21995491

• Ex Vivo Cryoablation of Wolff-Parkinson-White in a Donor Heart Prior to Pediatric Heart Transplantation AMERICAN JOURNAL OF TRANSPLANTATION Ceresnak, S. R., Hsu, D. T., Lamour, J. M., Weinstein, S., Pass, R. H. 2011; 11 (9): 1986-1988

  Abstract

  This report describes the use of a donor heart with ventricular pre-excitation for pediatric orthotopic heart transplantation and the successful surgical cryoablation of the donor heart prior to transplantation. The issues related to the preoperative evaluation and surgical management of the donor heart with Wolff-Parkinson White syndrome are discussed.

  View details for DOI 10.1111/j.1600-6143.2011.03657.x

  View details for Web of Science ID 000294360400029

  View details for PubMedID 21794085

• Wolff-Parkinson-White Syndrome and Isoproterenol Testing in Children A Valid Adjunct to Predict Risk? CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY Pass, R. H., Ceresnak, S. R. 2011; 4 (1): 8-10

  View details for DOI 10.1161/CIRCEP.110.961227

  View details for Web of Science ID 000287356000004

  View details for PubMedID 21325214

• Predictors for hemodynamic improvement with temporary pacing after pediatric cardiac surgery JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Ceresnak, S. R., Pass, R. H., Starc, T. J., Hordof, A. J., Bonney, W. J., Mosca, R. S., Liberman, L. 2011; 141 (1): 183-187

  Abstract

  Temporary epicardial pacing wires are commonly placed during pediatric cardiac surgery. Data are sparse on postoperative pacing in this population. The objective of this study was to determine the frequency of use and identify predictors for the use of temporary epicardial pacing wires.Perioperative data were prospectively collected on all patients who underwent cardiac surgery at our institution (n = 162).A total of 117 (72%) patients had temporary epicardial pacing wires placed. Postoperatively, 23 (20%) of 117 patients had hemodynamic improvement with the use of temporary epicardial pacing wires. Indications for pacing were slow junctional rhythm (11/23 [48%]), junctional ectopic tachycardia (7/23 [31%]), pace termination of supraventricular tachycardia (3/23 [13%]) and atrial flutter (1/23 [4%]), and complete heart block (1/23 [4%]). By using univariate analysis, single-ventricle anatomy, heterotaxy, the Fontan procedure, use of circulatory arrest, intraoperative arrhythmia, pacing in the operating room, and use of vasoactive medications were predictors for hemodynamic improvement with the use of temporary epicardial pacing wires (P < .05). On multivariate analysis, the Fontan procedure, circulatory arrest, and intraoperative arrhythmias were independent predictors (P < .01). When excluding all patients with any of these 3 risk factors, only 2% were paced. Patients with clinically significant pacing had longer chest tube drainage (P < .01) and intensive care unit length of stay (P < .01). There were no complications associated with temporary epicardial pacing wires.The Fontan procedure, use of circulatory arrest, and intraoperative arrhythmias were associated with hemodynamic improvement with postoperative pacing and might represent indications for empiric intraoperative placement of temporary epicardial pacing wires. Patients without these risk factors were less likely to require pacing. Temporary epicardial pacing wires were safe and useful in the management of arrhythmias after pediatric cardiac surgery.

  View details for DOI 10.1016/j.jtcvs.2010.03.048

  View details for Web of Science ID 000285407500032

  View details for PubMedID 20656300

• Electrophysiology Issues and Heart Failure in Congenital Heart Disease HEART FAILURE IN CONGENITAL HEART DISEASE: FROM FETUS TO ADULT Ceresnak, S. R., Dubin, A. M., Shaddy, R. E. 2011: 155–72

  View details for DOI 10.1007/978-1-84996-480-7_8

  View details for Web of Science ID 000286092100008

• Are wide complex tachycardia algorithms applicable in children and patients with congenital heart disease? JOURNAL OF ELECTROCARDIOLOGY Ceresnak, S. R., Liberman, L., Avasarala, K., Tanel, R., Motonaga, K. S., Dubin, A. M. 2010; 43 (6): 694-700

  Abstract

  Several algorithms have been developed to help determine the etiology of wide complex tachycardias (WCTs) in adults. Sensitivity and specificity for differentiating supraventricular tachycardia (SVT) with aberration from ventricular tachycardia (VT) in adults have been demonstrated to be as high as 98% and 97%. These algorithms have not been tested in the pediatric population. We hypothesize that these algorithms have lower diagnostic accuracy in children and patients with congenital heart disease.A retrospective review of the pediatric electrophysiology database at Stanford from 2001 to 2008 was performed. All children with WCT, a 12-lead electrocardiogram (ECG) available for review, and an electrophysiology study confirming the etiology of the rhythm were included. Patients with a paced rhythm were excluded. The ECGs were analyzed by 2 electrophysiologists blinded to the diagnosis according to the algorithms described in Brugada et al,(2) and Vereckei et al.(5) Additional ECG findings were recorded by each electrophysiologist.A total of 65 WCT ECGs in 58 patients were identified. Supraventricular tachycardia was noted in 62% (40/65) and VT in 38% (25/65) of the ECGs. The mean age was 13.5 years (SD ± 5.1), the mean weight was 51.8 kg (SD ± 22.4), and 48% (31/65) were male. The mean tachycardia cycle length was 340 milliseconds (SD ± 95). Congenital heart disease (CHD) was present in 37% (24/65) of patients (7 tetralogy of Fallot, 6 Ebstein's, 4 double-outlet right ventricle, 3 complex CHD, 2 d-transposition of great arteries, 1 status-post orthotopic heart transplantation, 1 ventricular septal defect). The Brugada algorithm correctly predicted the diagnosis 69% (45/65) of the time, the Vereckei algorithm correctly predicted the diagnosis 66% (43/65) of the time, and the blinded reviewer correctly predicted the diagnosis 78% (51/65) of the time. There was no difference in the efficacy of the algorithms in patients with CHD vs those with structurally normal hearts. The findings of left superior axis deviation (P < .01) and a notch in the QRS downstroke of V(1) or V(2) (P < .01) were more common in VT than SVT, whereas a positive QRS deflection in V(1) (P = .03) was more commonly present in SVT than VT.The Brugada and Vereckei algorithms have lower diagnostic accuracy in the pediatric population and in patients with congenital heart disease than in the adult population. Left superior axis deviation and a notch in the QRS downstroke were more commonly associated with VT, whereas a positive QRS deflection in V(1) was more commonly associated with SVT in this population.

  View details for DOI 10.1016/j.jelectrocard.2010.02.008

  View details for Web of Science ID 000284514700039

  View details for PubMedID 20382398

• Cryoablation with an 8-mm Tip Catheter for Pediatric Atrioventricular Nodal Reentrant Tachycardia Is Safe and Efficacious with a Low Incidence of Recurrence PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Silver, E. S., Silva, J. N., Ceresnak, S. R., Chiesa, N. A., Rhee, E. K., Dubin, A. M., Avasarala, K., Van Hare, G. F., Collins, K. K. 2010; 33 (6): 681-686

  Abstract

  Cryoablation with 4- and 6-mm tip ablation catheters has been demonstrated to be safe and effective in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients, albeit with a higher rate of clinical recurrence. Limited information is available regarding efficacy, mid-term outcomes, and complications related to the use of the 8-mm Freezor Max Cryoablation catheter (Medtronic, Minneapolis, MN, USA) in pediatric patients.We performed a retrospective review of all pediatric patients with normal cardiac anatomy who underwent an ablation procedure for treatment of AVNRT using the 8-mm tip Cryoablation catheter at three large pediatric academic arrhythmia centers.Cryoablation with an 8-mm tip catheter was performed in 77 patients for treatment of AVNRT (female n = 40 [52%], age 14.8 +/- 2.2 years, weight 62.0 +/- 13.9 kg). Initial procedural success was achieved in 69 patients (69/76, 91%). Transient second- or third-degree atrioventricular (AV) block was noted in five patients (6.5%). There was no permanent AV block. Of the patients successfully ablated with Cryotherapy, there were two recurrences (2/70, 2.8%) over a follow-up of 11.6 +/- 3.3 months.Cryoablation with an 8-mm tip ablation catheter is both safe and effective with a low risk of recurrence for the treatment of AVNRT in pediatric patients.

  View details for DOI 10.1111/j.1540-8159.2010.02706.x

  View details for Web of Science ID 000278818200006

  View details for PubMedID 20230479

• Survival of Transvenous ICD Leads in Young Patients PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Bonney, W. J., Spotnitz, H. M., Liberman, L., Silver, E. S., Ceresnak, S. R., Hordof, A. J., Pass, R. H. 2010; 33 (2): 186-191

  Abstract

  In adults, transvenous implantable cardioverter defibrillator (ICD) lead failure rates are significant, and their occurrence increases with time from implant. There are limited data in children. The goal of this study was to assess lead survival in young patients undergoing ICD implantation at a single center.Records of patients under 21 years old with transvenous ICD leads implanted at our center from June 1997 to August 2007 were retrospectively reviewed. Age, weight, height, diagnosis, lead and generator model, venous access technique, generator position, pacing thresholds, lead impedance, and R wave size were recorded. "Lead failure" was defined as any lead problem requiring surgical intervention to restore proper function to the ICD system.Seventy-one transvenous leads were included (70 patients). Average age at implant was 14.8 years (range 5.7-19.5). All the devices were implanted by a single operator (HMS). Venous access was obtained via cephalic cutdown in 66/71. Mean follow-up time was 2.8 years (range 0.2-7.8 years, median 2.3 years). There were no infections requiring explantation. There were four lead failures. Three were lead fractures, occurring 12, 13, and 19 months after implant. The fourth lead failed when an arrhythmia was not appropriately detected, and a second dedicated rate-sensing lead was thus implanted. Univariate analysis did not identify any variable to be a significant predictor of lead failure. Kaplan-Meier survival analysis demonstrated 5-year lead survival at 89.6%.ICD lead survival in children, when performed by an experienced operator, is similar to that found in adults.

  View details for DOI 10.1111/j.1540-8159.2009.02600.x

  View details for Web of Science ID 000273901500013

  View details for PubMedID 20002886

• An epicardial pacing safety net: an alternative technique for pacing in the young CARDIOLOGY IN THE YOUNG Ceresnak, S. R., Liberman, L., Chen, J. M., Hordof, A. J., Lamberti, J. J., Bonney, W. J., Pass, R. H. 2009; 19 (3): 228-232

  Abstract

  Epicardial pacing is the standard approach for permanent pacing in small children and patients with functionally univentricular physiology. The longevity of epicardial leads, however, is compromised by increased occurrences of exit block and lead fractures. We report our experience with a technique of placing a second ventricular lead, and attaching it to the atrial port of a dual chamber pacemaker to prevent the need for early re-operation in the event of failure of the primary epicardial lead. A retrospective review showed that, over the period from 2001 through 2007, epicardial ventricular pacemakers had been placed in 88 patients. In 6 of these, we had placed 2 ventricular leads, their median weight being 8.0 kilograms, with a range from 4.2 to 31.8 kilograms. Fracture of a lead occurred in 1 of the patients (17%) 8 months after placement, requiring reprogramming to pace from the atrial port. This possibility avoided the need for repeated emergent surgery. At a median follow-up of 1.5 years, with a range from 0.3 to 4.4 years, there have been no complications. During the same time period, overall failure of epicardial leads at our institution was 13%. Placement of a second ventricular epicardial pacing lead, attached to the atrial port of a dual chamber pacemaker, therefore, may provide a safe and effective means of ventricular pacing in the setting of epicardial lead failure, and may obviate the need for repeat, potentially urgent, pacemaker surgery.

  View details for DOI 10.1017/S1047951109003710

  View details for Web of Science ID 000266792000003

  View details for PubMedID 19272204

• Elevated Impedance During Cardioversion in Neonates with Atrial Flutter PEDIATRIC CARDIOLOGY Ceresnak, S. R., Starc, T. J., Hordof, A. J., Pass, R. H., Bonney, W. J., Liberman, L. 2009; 30 (4): 436-440

  Abstract

  Direct-current cardioversion is a common treatment modality for acute termination of atrial flutter in neonates. Studies in children have demonstrated that cardioversion is often successful with as little as 0.25-0.5 J/kg with the current biphasic devices. We hypothesize that during cardioversion of atrial flutter in neonates, however, the impedance may be high and more energy may be required for successful cardioversion. A retrospective chart review of our institutional experience from 2005 through 2008 was performed. Neonates with atrial flutter requiring cardioversion who had strips available for review were included. Six patients met the inclusion criteria. The median age at the time of cardioversion was 2.6 h (range, 1.3-336 h) and the mean weight was 3.22 +/- 0.4 kg (SD). The mean electrical impedance of the successful shocks was elevated, at 234 +/- 136 Omega. The mean energy delivered for successful cardioversion was 0.9 +/- 0.3 J/kg, and the current was 1 A in all patients. In conclusion, the shock impedance was elevated in the neonates studied during cardioversion of atrial flutter. Low current was sufficient for successful cardioversion. Further studies are needed in this specific population.

  View details for DOI 10.1007/s00246-009-9413-6

  View details for Web of Science ID 000266167600008

  View details for PubMedID 19365665

• Verapamil sensitive ventricular tachycardia associated with a cardiac hemangioma in the right ventricular outflow tract. Indian pacing and electrophysiology journal Bonney, W. J., Ceresnak, S. R., Ira, S., Hordof, A., Liberman, L. 2009; 9 (6): 355-359

  Abstract

  Primary tumors of the heart are rare, but they are often associated with refractory arrhythmias. Vascular tumors of the heart comprise a small minority of primary cardiac tumors. In patients with structurally normal hearts, ventricular tachycardia (VT) originating from the right ventricular outflow tract (RVOT) can be sensitive to adenosine, vagal maneuvers, and calcium channel blockers. In this report, we describe a case of ventricular tachycardia originating from within a hemangioma in the RVOT that was ultimately controlled with verapamil.

  View details for PubMedID 19898659

• The palliative arterial switch procedure for single ventricles: Are these patients suitable Fontan candidates? ANNALS OF THORACIC SURGERY Ceresnak, S. R., Quaegebeur, J. M., Pass, R. H., Hordof, A. J., Liberman, L. 2008; 86 (2): 583-587

  Abstract

  The traditional first stage of palliation for functional single-ventricle lesions with transposition of the great vessels and systemic outflow tract obstruction has been either the Norwood or Sano procedure or the Damus-Kaye-Stansel procedure. There is limited literature on the use of an arterial switch procedure as the initial staged palliation for functional single ventricles in this setting. This study is an examination of our institutional experience with performing the palliative switch procedure with examination of suitability for Fontan completion and midterm outcome in these patients.This is a retrospective review of our institutional experience from October 1991 through August 2006 on single-ventricle patients with transposition of the great vessels and systemic outflow tract obstruction who underwent a palliative arterial switch procedure.Nine patients underwent an initial palliative switch procedure. Six of 9 patients underwent completion of palliation with the Fontan procedure and are alive and well. One patient is well and is awaiting the next stage of palliation (78%). There was 1 early operative death (11%) and 1 late death (11%). There was 1 case of recoarctation (11%).The palliative switch procedure appears a reasonable surgical option for patients with functional single-ventricle lesions, transposition of the great vessels, and systemic outflow tract obstruction. Patients who have undergone a palliative switch procedure are suitable candidates for completion of palliation with the Fontan procedure.

  View details for DOI 10.1016/j.athoracsur.2008.04.022

  View details for Web of Science ID 000257767100034

  View details for PubMedID 18640337

• Coronary Artery Fistulas: A Review of the Literature and Presentation of Two Cases of Coronary Fistulas with Drainage into the Left Atrium Congenital Heart Disease Ceresnak, MD, S. R., Gray, MD, R. G., Altmann, MD, K., Chen, MD, J. M., Glickstein, MD, J. S., Hellenbrand, MD, W. E. 2007; 2 (3): 208-213

  View details for DOI 10.1111/j.1747-0803.2007.00100.x

• Amino acid profile of escaped feed protein after rumen incubation and their intestinal digestibility ARCHIVES OF ANIMAL NUTRITION Ceresnakova, Z., Sommer, A., Chrenkova, M., Dolesova, P. 2002; 56 (6): 409-418

  Abstract

  The crude protein content and amino acid profile of seven feedstuffs (linseed meal, maize gluten meal, rapeseed meal, rapeseed meal protected, soybean meal, fullfat soybean extruded and sunflower meal) were determined before and after ruminal incubation for 16 h in three bulls with large rumen cannulas. The intestinal disappearance of amino acids was measured using mobile bag technique. Ruminal incubation affected amino acid profile of all experimental feedstuffs. Crude protein degradation varied from 29.3% for maize gluten meal to 86.4% for rapeseed meal. A tendency towards increased disappearance was observed for glutamic acid, histidine, lysine and proline and decreased disappearance for branched-chain amino acids. The intestinal crude protein digestibility was higher than > 80%, except rapeseed meal (66.4%) and sunflower meal (77.8%). The least digestible individual amino acids were methionine and isoleucine in rapeseed meal, histidine and methionine in rapeseed meal protected and arginine in sunflower meal. In general, the lowest amino acid digestibilities were found in feedstuffs with the highest fibre content. The feedstuffs show that they have different potential for supplying of limiting amino acids. Of particular value are the feedstuffs with low crude protein degradability in the rumen and high intestinal digestibility of amino acids.

  View details for DOI 10.1080/0003942921000019135

  View details for Web of Science ID 000180621800003

  View details for PubMedID 12553691