Norman H. Silverman

All Publications

• In Memory of a Brilliant Pediatric Cardiologist: Dr. Abraham Rudolph. Pediatric cardiology Silverman, N., Teitel, D. 2023

  View details for DOI 10.1007/s00246-023-03189-8

  View details for PubMedID 37212880

• 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

• Functional assessment of atrial wall excursion and foramen ovale flap tracings in 3rd trimester as predictor of short-term hemodynamic stability in congenital heart defects fetuses. Journal of perinatal medicine Sylwestrzak, O., Strzelecka, I., Silverman, N. H., Respondek-Liberska, M. 2022

  Abstract

  OBJECTIVES: Right atrium and left atrium of the fetal heartplay a fundamental role in fetal heart circulatory physiology.METHODS: Excursion of fetal atria walls and tracings of foramen ovale (FO) flap movement were analyzed by M-mode echocardiography by new FO index to determine fetal and neonatal outcome in the first week and month of life in various congenital heart defects. The study group was divided into 3 subgroups: group A: neonates stable after birth (all on I.V. prostaglandin infusion) for at least 7days, group B: neonates unstable after birth (despite I.V. prostaglandin infusion) requiring catheterization procedure or early cardiac surgery<6th day of life and group C: fetuses with in utero demise.RESULTS: The average values of FO index: group A- 32, group B- 20, group C- 12.Schaffe test showed statistical difference of FO index between group A and B (p=0.029) and group A and C (p=0.001), but no difference between group B and C (p=0.24) The FO index of stability was determined by ROC curve analysis. Cut-off point distinguishing between postnatal stability and postnatal instability+in utero demise constituted FO index=25 (specificity 82%; sensitivity 90%).CONCLUSIONS: Prenatal features of atrial M-mode echocardiography shortly before birth might be helpful to predict the short term prognosis of fetal hemodynamic stability or instability shortly after birth regardless of the type of congenital heart defects (CHD).

  View details for DOI 10.1515/jpm-2022-0429

  View details for PubMedID 36437556

• Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study. Journal of the American Heart Association Freud, L. R., McElhinney, D. B., Kalish, B. T., Escobar-Diaz, M. C., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Selamet Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., Tworetzky, W. 2020: e016684

  Abstract

  Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

  View details for DOI 10.1161/JAHA.120.016684

  View details for PubMedID 33076749

• The Natural and Unnatural History of Ventricular Septal Defects Presenting in Infancy: An Echocardiography-Based Review. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Cox, K., Algaze-Yojay, C., Punn, R., Silverman, N. 2020

  Abstract

  BACKGROUND: Ventricular septal defect (VSD), the most common congenital heart defect, accounts for 40% of heart malformations. Despite this prevalence, there remains no consensus on the utility of echocardiography to guide modern-era treatment. In this study, we evaluated patients with isolated VSDs to test the hypothesis that echocardiographic evidence of left ventricular (LV) volume overload and type of VSD are associated with surgical intervention and to identify useful echocardiographic indicators for management of VSDs in infants and children.METHODS: We reviewed 350 patients with VSDs diagnosed during the first year of life. Echocardiographic measurements were made at the time of diagnosis and at the endpoint. The VSD area was calculated using inner edge to inner edge dimensions obtained from two planes and indexed to body surface area. Aortic annulus dimension, left atrium to aortic root ratio, LV end-diastolic diameter, left atrial volume, VSD velocity-time integral, ejection fraction, and pulmonary to systemic blood flow ratio (Qp:Qs) were measured using conventional methods.RESULTS: One hundred seventy-seven muscular (50.5%) and 162 perimembranous (46%) VSDs accounted for the vast majority of defects. Only seven (4%) muscular defects required surgical closure, while 76 (47%) perimembranous defects required surgery. Indexed VSD area, VSD to aortic valve ratio, indexed left atrium volume, LV end-diastolic diameter, VSD velocity-time integral, and Qp:Qs at diagnosis were significantly different between the surgical and nonsurgical groups. Ventricular septal defect area > 50mm2/m2 at initial diagnosis was independently associated with risk for surgery (P=.0055).CONCLUSIONS: Indexed VSD area is an echocardiographic variable that can be easily measured at diagnosis and can provide insight into the likelihood of requiring surgical intervention regardless of the type and location of the defect.

  View details for DOI 10.1016/j.echo.2020.01.013

  View details for PubMedID 32249125

• Postoperative Recovery of Left Ventricular Function following Repair of Large Ventricular Septal Defects in Infants. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Adamson, G. T., Arunamata, A., Tacy, T. A., Silverman, N. H., Ma, M., Maskatia, S. A., Punn, R. 2019

  Abstract

  INTRODUCTION: Early postoperative left ventricular (LV) dysfunction is observed following repair of large ventricular septal defects (VSDs), but the frequency and rate of recovery of LV function are unknown. This study aims to characterize the incidence and rate of recovery of postoperative LV dysfunction following repair of large VSDs and to improve understanding of LV mechanics before and after VSD repair.METHODS: Infants who underwent surgical repair of an isolated large VSD were included. Pre- and postoperative echocardiographic images were reviewed, and LV function was assessed by both conventional echocardiography and speckle-tracking strain analyses. Postoperative LV dysfunction was defined as an LV ejection fraction (LVEF)<50% using the 5/6 area-length method. Echocardiograms were reviewed to assess LVEF and LV volume through 1-year follow-up.RESULTS: Of 104 infants evaluated (median age, 0.31 [0.22, 0.56] years), all had normal preoperative LVEF and 39 (38%) had postoperative LV dysfunction. Follow-up echocardiograms were available in 31 (80%) patients, all of whom had LVEF>50% within 9months of surgery. Lower preoperative apical four-chamber longitudinal strain (A4LS) and greater LV end-diastolic volume indexed to body surface area1.38 were independently associated with postoperative LV dysfunction. An absolute preoperative A4LS < 16.7% (area under the curve=0.87; 95% CI, 0.78-0.95; P<.001) was most strongly associated with postoperative dysfunction.CONCLUSIONS: Infants with LV dysfunction following repair of large VSDs recover function within 9months. Preoperative A4LS can be helpful to detect subclinical LV dysfunction in the setting of a large hemodynamically significant VSD and guide postoperative expectations for providers and families.

  View details for DOI 10.1016/j.echo.2019.10.003

  View details for PubMedID 31866322

• Cardiac Segmental Strain Analysis in Pediatric Left Ventricular Noncompaction Cardiomyopathy JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Arunamata, A., Stringer, J., Balasubramanian, S., Tacy, T. A., Silverman, N. H., Punn, R. 2019; 32 (6): 763-+

  View details for DOI 10.1016/j.echo.2019.01.014

  View details for Web of Science ID 000470058700009

• Aortopulmonary window in tetralogy of Fallot with absent conal septum ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Aurigemma, D., Dixon, C., Tucker, S., Davis, C., Silverman, N. 2019; 36 (2): 411–14

  View details for DOI 10.1111/echo.14243

  View details for Web of Science ID 000458874600026

• Cardiac Segmental Strain Analysis in Pediatric Left Ventricular Noncompaction Cardiomyopathy. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Arunamata, A. n., Stringer, J. n., Balasubramanian, S. n., Tacy, T. A., Silverman, N. H., Punn, R. n. 2019

  Abstract

  Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent left ventricular trabeculations, often leading to myocardial dysfunction and death or heart transplantation. Although diagnostic echocardiographic criteria exist for LVNC, segmental speckle-tracking strain patterns have not been described in pediatric LVNC. The objectives of this study were to characterize segmental noncompaction, evaluate segmental speckle-tracking strain, and characterize peak strain values in children with LVNC with adverse clinical outcomes.Echocardiographic noncompaction/compaction ratios and segmental radial, circumferential, and longitudinal strain were measured retrospectively in children with LVNC (January 2000 to June 2013). Segmental strain of the cohort was compared with that in control subjects matched by age and body surface area.One hundred one pediatric patients with LVNC were included (median age 2.8 years; range, 0-19.4 years); 71 patients survived (benign group) and 30 patients did not survive or required heart transplantation (adverse group). The adverse group was younger at diagnosis (P = .03), with lower ejection fractions (P < .0001) compared with the benign group. Both groups demonstrated increased noncompaction/compaction ratios within apical segments, with significantly increased involvement in antero- and inferolateral midpapillary segments in the adverse group (P < .005). Radial, circumferential, and longitudinal strain of nearly all left ventricular segments were significantly decreased in the adverse compared with the benign group and in pediatric LVNC patients compared with control subjects. Global radial (7.4 ± 9.7% vs 19.6 ± 16.5%, P < .0001), circumferential (-8.4 ± 7.0% vs -18.9 ± 6.9%, P < .0001), and longitudinal (-7.1 ± 5.2% vs -11.8 ± 5.3%, P < .0001) strain were all significantly decreased in the adverse group compared with the benign group.Segmental radial, circumferential, and longitudinal strain are decreased in pediatric patients with LVNC compared with control subjects and lowest in those with adverse compared with benign outcomes. LVNC appears to be a global disease affecting deformation in all regions, including compacted myocardial segments.

  View details for PubMedID 30926406

• Aortopulmonary window in tetralogy of fallot with absent conal septum. Echocardiography (Mount Kisco, N.Y.) Aurigemma, D., Dixon, C., Tucker, S., Davis, C., Silverman, N. 2018

  Abstract

  BACKGROUND: Aortopulmonary window is a rare conotruncal defect that is often associated with other congenital heart defects. We present a patient with a previously unreported combination of aortopulmonary window with tetralogy of Fallot with an absent conal septum.CASE PRESENTATION: A term, 2.4kg newborn male infant presented at a community hospital with cyanosis unresponsive to supplemental oxygen. Transthoracic echocardiography demonstrated a conotruncal defect with a large conoventricular ventricular septal defect and an over-riding, dysplastic aortic valve. The main pulmonary artery (MPA) appeared to arise from left facing sinus of the aortic valve, with confluent yet hypoplastic right and left branch pulmonary arteries. There was no evidence of prograde flow into the MPA in systole, though there did appear to be retrograde flow in diastole from the patent ductus. The patient underwent multiple advanced imaging studies, and the diagnosis was not fully elucidated. Postmortem examination demonstrated morphology consistent with Tetralogy of Fallot with the absence of the conal septum. There were two distinct semilunar valves in fibrous continuity with an aortopulmonary window immediately cephalad to the valve.DISCUSSION: The rare combination of defects and the patient's size made the anatomic diagnosis by conventional imaging challenging. However, retrospective review of imaging studies did demonstrate anatomic features seen by direct examination of the specimen.

  View details for PubMedID 30585348

• 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

• Changing Management of the Patent Ductus Arteriosus: Effect on Neonatal Outcomes and Resource Utilization. American journal of perinatology Chock, V. Y., Goel, V. V., Palma, J. P., Luh, T. M., Wang, N. A., Gaskari, S., Punn, R., Silverman, N. H., Benitz, W. E. 2017

  Abstract

  Objective This historical cohort study investigated how a shift toward a more conservative approach of awaiting spontaneous closure of the patent ductus arteriosus (PDA) in preterm infants has affected neonatal outcomes and resource utilization. Methods We retrospectively studied very low birth weight infants diagnosed with a PDA by echocardiogram (ECHO) in 2006-2008 (era 1), when medical or surgical PDA management was emphasized, to those born in 2010-2012 (era 2) when conservative PDA management was encouraged. Multiple regression analyses adjusted for gestational age were performed to assess differences in clinical outcomes and resource utilization between eras. Results More infants in era 2 (35/89, 39%) compared with era 1 (22/120, 18%) had conservative PDA management (p < 0.01). Despite no difference in surgical ligation rate, infants in era 2 had ligation later (median 24 vs. 8 days, p < 0.0001). There was no difference in clinical outcomes between eras, while number of ECHOs per patient was the only resource measure that increased in era 2 (median 3 vs. 2 ECHOs, p = 0.003). Conclusion In an era of more conservative PDA management, no increase in adverse clinical outcomes or significant change in resource utilization was found. Conservative PDA management may be a safe alternative for preterm infants.

  View details for DOI 10.1055/s-0037-1601442

  View details for PubMedID 28376547

• Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia AMERICAN JOURNAL OF CARDIOLOGY Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2017; 119 (1): 106-111

  Abstract

  In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

  View details for DOI 10.1016/j.amjcard.2016.09.022

  View details for Web of Science ID 000391246900017

• Tricuspid Atresia with Non-compaction: An Early Experience with Implications for Surgical Palliation. Pediatric cardiology Nguyen, H. H., Khan, R. n., Silverman, N. H., Singh, G. K. 2017; 38 (3): 495–505

  Abstract

  Left ventricle non-compaction (LVNC) has worse outcomes when associated with congenital heart defects (CHD). The co-occurrence and outcomes of LVNC with tricuspid atresia (TA) are not well described. Our study aims to determine the prevalence of LVNC with functionally single ventricle due to TA, and to describe the early outcomes of surgical palliation. A retrospective database search for patients (n = 167,566) and echocardiograms (n = 44,053) was performed in order to collect clinical, echocardiographic, and hemodynamic data of pediatric patients with TA and LVNC at St. Louis Children's Hospital, Missouri, USA, from January 1, 2008, to August 31, 2013. The prevalence of TA and LVNC was 0.015 and 0.08%, respectively. Eight patients with TA had LVNC (32%, group 1). Seventeen patients had only TA (68%, group 2). Five patients from group 1 and 8 patients from group 2 were surgically palliated with the Fontan procedure. They exhibited similar remodeling indices, and hemodynamics (median LV end-diastolic pressure ≤10 mmHg; median mean pulmonary artery pressure ≤15 mmHg) that allowed for completion of the Fontan procedure. All Fontan patients were in New York Heart Association class I after a 3-year (median) follow-up period. Our data show that TA with LVNC patients, who have acceptable cardiac remodeling indices, LV systolic function, and hemodynamics (LVEDP ≤ 10 mmHg, mean pulmonary artery pressure ≤ 15 mmHg) can have successful completion of the Fontan procedure and positive early outcomes.

  View details for PubMedID 27942762

• Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia. American journal of cardiology Selamet Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2016

  Abstract

  In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

  View details for DOI 10.1016/j.amjcard.2016.09.022

  View details for PubMedID 27793395

• Decompressing vein and bilateral superior venae cavae in a patient with hypoplastic left heart syndrome. Echocardiography (Mount Kisco, N.Y.) Stauffer, K. J., Arunamata, A., Vasanawala, S. S., Behera, S. K., Kipps, A. K., Silverman, N. H. 2016; 33 (9): 1428-1431

  Abstract

  The levoatrial cardinal vein (LACV), first described in 1926, acts as a decompressing vessel for pulmonary venous return in cases of severe left-sided obstruction with an intact or significantly restrictive atrial septum. The LACV and the persistent left superior vena cava (LSVC) are thought to share similar embryologic origins. To challenge this notion, we present a unique case of a neonate with hypoplastic left heart syndrome, cor triatriatum, and a decompressing LACV in the presence of bilateral superior venae cavae.

  View details for DOI 10.1111/echo.13292

  View details for PubMedID 27641733

• Discordant Fetal and Post-Natal Diagnosis Can We Do Better? JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Silverman, N. H., Moon-Grady, A. J. 2016; 68 (9): 931–33

  View details for DOI 10.1016/j.jacc.2016.06.023

  View details for Web of Science ID 000382312900008

  View details for PubMedID 27561767

• Postnatal Outcome of Fetal Left Ventricular Hypertrabeculation/Noncompaction PEDIATRIC CARDIOLOGY Stoellberger, C., Wegner, C., Benatar, A., Chin, T. K., Dangel, J., Majoor-Krakauer, D., Mondal, T. K., Sivanandam, S., Silverman, N. H., van Waning, J., Finsterer, J. 2016; 37 (5): 919-924

  Abstract

  Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Prenatal diagnosis of LVHT can be established by fetal echocardiography. A review of 106 published cases showed that 46 cases with prenatally diagnosed LVHT were alive 0.5-120 months after birth. Since the course of cases with prenatally LVHT after publication is unknown, we aimed to collect follow-up-information. Information regarding vital status, cardiac and extracardiac morbidity was gathered by contacting the authors of the 46 cases. Fourteen of the 28 authors answered and gave information about 18 cases (six females, seven males, five gender-unknown, age 18 months to 10 years, mean follow-up 60 months). No differences were found between the 18 cases with follow-up and the 28 cases without follow-up regarding age, gender, cardiac or extracardiac comorbidities, and interventions. Three of the 18 cases had died subsequently from heart failure, osteosarcoma, and enterocolitis, respectively. Mutations or chromosomal abnormalities were found in six of the seven examined patients, extracardiac abnormalities in nine patients. Three patients received a pacemaker because of complete AV block, and two patients underwent heart transplantation. Cardiac surgical or interventional procedures were carried out in four patients. None suffered from malignant arrhythmias or had a cardioverter-defibrillator implanted. Based on the limited information, there are indications that cases with fetal diagnosis of LVHT have a continuing morbidity and mortality, even if they receive appropriate care. Since fetal LVHT is frequently associated with genetic abnormalities, further research about survival and underlying genetic causes is needed.

  View details for DOI 10.1007/s00246-016-1369-8

  View details for Web of Science ID 000377722400015

  View details for PubMedID 27033242

• Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD. Cardiology in the young Madriago, E. J., Punn, R., Geeter, N., Silverman, N. H. 2016; 26 (2): 263-268

  Abstract

  Trans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown.We retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings.The diagnoses most commonly requiring further repair and subsequent imaging were mitral valve disease (20.9%), tricuspid valve disease (16.0%), atrioventricular canal defects (12.0%), and pulmonary valve disease (14.1%). The vast majority of those requiring immediate return to bypass benefited by avoiding subsequent operations and longer lengths of hospital stay. A total of 14 patients (0.8%) who received routine imaging required further surgical repair within 1 week, usually due to disease that developed over ensuing days. Patients who had second post-operative trans-oesophageal echocardiograms in the operating room rarely required re-operations, confirming the benefit of routine intra-operative imaging.This study represents a large single institutional review of intra-operative trans-oesophageal echocardiography, and confirms its applicability in the surgical repair of patients with CHD. Routine imaging accurately identifies patients requiring further intervention, does not confer additional risk of mortality or prolonged length of hospital stay, and prevents subsequent operations and associated sequelae in a substantial subset of patients. This study demonstrates the utility of echocardiography in intra-operative monitoring of surgical repair and highlights patients who are most likely to require return to bypass, as well as the co-morbidities of such manipulations.

  View details for DOI 10.1017/S1047951115000098

  View details for PubMedID 25730612

• Near-fatal neonatal coronary ischaemia associated with intermittent aortic regurgitation: successful surgical treatment CARDIOLOGY IN THE YOUNG Likes, M. L., Silverman, N. H., Albers, E. L., Choy, R., Bhat, A., McMullan, D. M. 2015; 25 (8): 1531-1535

  Abstract

  An infant presented with features suggestive of an anomalous left coronary artery was found to have normal origins of both coronary arteries. Echocardiography during episodes of ischaemia showed marked aortic regurgitation with retrograde coronary flow. The left coronary leaflet was mildly hypoplastic. Surgical re-suspension of this leaflet prevented aortic regurgitation and the patient had no further symptoms and recovered cardiac function.

  View details for DOI 10.1017/S1047951115002024

  View details for Web of Science ID 000367196500013

  View details for PubMedID 26675600

• Echocardiographic presentation of anomalous origin of the left coronary artery from the pulmonary artery CARDIOLOGY IN THE YOUNG Silverman, N. H. 2015; 25 (8): 1512-1523

  Abstract

  In the 1970s, diagnosing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was often uncertain using imaging alone; however, with the advances in high-frequency transducers, advanced image processing, and other ultrasound modalities such as Doppler colour flow imaging, tissue Doppler imaging, and speckle tracking to asses regional wall motion abnormalities, modern echocardiography now permits accurate diagnosis of ALCAPA with greater certainty. Although many consider ultrasound to be the only imaging test necessary if there is a question as to the diagnosis, other imaging modalities such as MRI, CT, and cardiac catheterisation with angiography remain valuable complementary tests, especially in older patients.

  View details for DOI 10.1017/S1047951115002565

  View details for Web of Science ID 000367196500011

  View details for PubMedID 26675598

• Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study CIRCULATION Freud, L. R., Escobar-Diaz, M. C., Kalish, B. T., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., McElhinney, D. B., Tworetzky, W. 2015; 132 (6): 481-489

  Abstract

  Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001).In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

  View details for DOI 10.1161/CIRCULATIONAHA.115.015839

  View details for Web of Science ID 000359666500003

  View details for PubMedID 26059011

• Echocardiography of congenital mitral valve disorders: echocardiographic-morphological comparisons CARDIOLOGY IN THE YOUNG Silverman, N. H. 2014; 24 (6): 1030-1048

  Abstract

  I surveyed our echocardiographic database of the years between 1998 and 2012 for congenital abnormalities of the mitral valve in patients over 14 years. A total of 249 patients with mitral valve abnormalities were identified. Abnormalities included clefts in the mitral valve in 58 patients, double orifice of the mitral valve in 19, mitral stenosis with two papillary muscles in 72, and mitral stenosis with one papillary muscle in 51 patients. Supravalvar rings were found in 35 patients with a single papillary muscle, and mitral stenoses with two papillary muscles were found in 22 patients. Mitral prolapse occurred in 44 patients and mitral valvar straddle in five patients. The patients were evaluated by all modalities of ultrasound available over the course of time. Although some lesions were isolated, there were many lesions in which more than one mitral deformity presented in the same patient. The patients are presented showing anatomical correlation with autopsy specimens, some of which came from the patients in this series, and others matched to show correlative anatomy. These lesions remain rare as a group and continue to have high morbidity and mortality.

  View details for DOI 10.1017/S1047951114002157

  View details for Web of Science ID 000348580300005

  View details for PubMedID 25647377

• Evaluation of Risk Factors for Prediction of Outcome in Fetal Spectrum of Atrioventricular Septal Defects CONGENITAL HEART DISEASE Bader, R. S., Punn, R., Silverman, N. H. 2014; 9 (4): 286-293

  Abstract

  Atrioventricular septal defects (AVSD) are very commonly diagnosed in utero. Heterotaxy/chromosomal abnormalities frequently coexist with AVSD. However, outcomes of fetal AVSD are not precisely known. We attempted to define mortality risk factors in AVSD.We retrospectively searched our database, electronic records, and echocardiograms with diagnosis of fetal AVSD from 2003 to 2012. We investigated the following risk factors: atrial situs, heart rate/rhythm, ventricular dominance/morphology, atrioventricular valve regurgitation, cardiothoracic ratio, ejection fraction, and extracardiac anomalies.Forty-five fetuses with a median gestational age of 28 weeks (17.5-37.1) were determined to have AVSD during the 10 years, of which 12 were either lost to follow-up (6) or underwent termination (6). There were 16 deaths (48%); two died in utero. Isomerism was identified in 17 of 45 (37%) fetuses (11 left atrial, 6 right atrial isomerism) and chromosomal abnormalities were identified in 12 (27%). Twenty-eight of 33 fetuses, not lost to follow-up or terminated, had extracardiac anomalies which had associated increased mortality (57% vs. 0%, P = .04). Heart block (75% vs. 43%, P = .12), left ventricular noncompaction (80% vs. 43%, P = .17), and isomerism (63% vs. 41%, P = .28) were associated with mortality but without statistical significance. Twenty-five of 45 (56%) had unbalanced AVSD. Positional abnormalities of the great arteries or semilunar valve stenosis were present in 20/45 (44%) while venous anomalies were present in 16/45 (36%). Presence of ventricular dominance, atrioventricular valve regurgitation, elevated cardiothoracic ratio, or diminished ejection fraction were not associated with mortality.Overall mortality rate for fetuses with AVSD was 48%. The presence of extracardiac anomalies is an independent risk factor for prediction of fetal or neonatal demise. Heart block, isomerism, and noncompaction in fetal AVSD appear to be associated with poor outcomes as well but did not reach statistical significance. This information is useful for counseling parents with fetus AVSD.

  View details for DOI 10.1111/chd.12136

  View details for Web of Science ID 000340525400012

  View details for PubMedID 24102719

• Predictors of bronchopulmonary dysplasia or death in premature infants with a patent ductus arteriosus. Pediatric research Chock, V. Y., Punn, R., Oza, A., Benitz, W. E., Van Meurs, K. P., Whittemore, A. S., Behzadian, F., Silverman, N. H. 2014; 75 (4): 570-575

  Abstract

  Background:Preterm infants with a PDA are at risk for death or development of BPD. However, PDA treatment remains controversial. We investigated if PDA treatment and other clinical or echocardiographic (ECHO) factors were associated with the development of death or BPD.Methods:We retrospectively studied clinical and ECHO characteristics of preterm infants with birth weight <1500 g and ECHO diagnosis of a PDA. Logistic regression and classification and regression tree (CART) analyses were performed to assess variables associated with the combined outcome of death or BPD.Results:Of 187 preterm infants with a PDA, 75% were treated with indomethacin or surgery and 25% were managed conservatively. Death or BPD occurred in 80 (43%). Logistic regression found lower gestational age (OR 0.5), earlier year of birth during the study period (OR 0.9), and larger ductal diameter (OR 4.3) were associated with the decision to treat the PDA, while gestational age was the only variable associated with death or BPD (OR 0.6, 95% CI 0.5-0.8).Conclusion:Only lower gestational age and not PDA treatment or ECHO score was associated with the adverse outcome of death or BPD. Further investigation of PDA management strategies and effects on adverse outcomes of prematurity is needed.Pediatric Research (2013); doi:10.1038/pr.2013.253.

  View details for DOI 10.1038/pr.2013.253

  View details for PubMedID 24378897

• Transcatheter Device Closure of a Congenital Aortic-Left Atrial Tunnel CONGENITAL HEART DISEASE Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-E26

  Abstract

  Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

  View details for DOI 10.1111/chd.12059

  View details for Web of Science ID 000329916300010

  View details for PubMedID 23601836

• Transcatheter device closure of a congenital aortic-left atrial tunnel. Congenital heart disease Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-6

  Abstract

  Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

  View details for DOI 10.1111/chd.12059

  View details for PubMedID 23601836

• The essential echocardiographic features of tetralogy of Fallot 6th World Congress of Pediatric Cardiology and Cardiac Surgery Silverman, N. H. CAMBRIDGE UNIV PRESS. 2013: 871–82

  Abstract

  This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

  View details for DOI 10.1017/S1047951113001704

  View details for Web of Science ID 000330355900014

  View details for PubMedID 24401261

• Echocardiography of transposition of the great arteries CARDIOLOGY IN THE YOUNG Mahle, W. T., Gonzalez, J. H., Kreeger, J., Marx, G., Duldani, G., Silverman, N. H. 2012; 22 (6): 664-670

  Abstract

  The successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.

  View details for DOI 10.1017/S1047951112001503

  View details for Web of Science ID 000313710200007

  View details for PubMedID 23331585

• Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Arunamata, A., Punn, R., Cuneo, B., Bharati, S., Silverman, N. H. 2012; 25 (1): 112-120

  Abstract

  Left ventricular noncompaction (LVNC) has rarely been described in the fetus.The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035).Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.

  View details for DOI 10.1016/j.echo.2011.09.019

  View details for Web of Science ID 000298276500013

  View details for PubMedID 22014428

• Costs of Prenatal Detection of Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Jegatheeswaran, A., Oliveira, C., Batsos, C., Moon-Grady, A. J., Silverman, N. H., Homberger, L. K., Coyte, P., Friedberg, M. K. 2011; 108 (12): 1808–14

  Abstract

  Little information is available about the transportation costs incurred from the missed prenatal diagnosis of congenital heart disease (CHD). The objectives of the present study were to analyze the costs of emergency transportation related to the postnatal diagnosis of major CHD and to perform a cost/benefit analysis of additional training for ultrasound technicians to study the implications of improved prenatal detection rates. The 1-year costs incurred for emergency transportation of pre- and postnatally diagnosed infants with CHD in Northern California and North Western Nevada were calculated and compared. The prenatal detection rate in our cohort (n = 147) was 30.6%. Infants postnatally diagnosed were 16.5 times more likely (p <0.001) to require emergency transport. The associated emergency transportation costs were US$542,143 in total for all patients with CHD. The mean cost per patient was $389.00 versus $5,143.51 for prenatally and postnatally diagnosed infants, respectively (p <0.001). Assuming an improvement in detection rates after 1-day training for ultrasound technicians, the investment in training cost can be recouped in 1 year if the detection rate increased by 2.4% to 33%. Savings of $6,543,476 would occur within 5 years if the detection rate increased to 50%. In conclusion, CHD diagnosed postnatally results in greater costs related to emergency transportation of ill infants. Improving the prenatal detection rates through improved ultrasound technician training could result in considerable cost savings.

  View details for DOI 10.1016/j.amjcard.2011.07.052

  View details for Web of Science ID 000298312400020

  View details for PubMedID 21907953

• Fetal Predictors of Urgent Balloon Atrial Septostomy in Neonates with Complete Transposition JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Silverman, N. H. 2011; 24 (4): 425-430

  Abstract

  In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrial septostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.We reviewed all 26 fetuses from 2001 to 2010 with complete transposition of the great vessels and closely examined the patent foramen ovale and septum primum for hypermobility, restriction, flat appearance, or redundancy. We defined hypermobility as a septum primum flap that oscillates between both atria. We also examined the ductus size and shunting pattern to evaluate whether these features contributed to urgent BAS.In total, 14 of 26 fetuses required urgent BAS with improved cyanosis. Nine fetuses had an urgent BAS and a hypermobile septum, and 12 fetuses had no urgent BAS or hypermobile septum. Eight fetuses had an urgent BAS and a reverse diastolic patent ductus arteriosus, and 11 fetuses had no urgent BAS or reverse diastolic patent ductus arteriosus. A hypermobile septum and reverse diastolic patent ductus arteriosus had a significant association with urgent BAS (P < .01, sensitivity = 0.64 and 0.57, specificity = 1.0 and 0.92, positive predictive value = 1.0 and 0.89, negative predictive value = 0.71 and 0.65). No fetus had a restrictive patent foramen ovale/ductus arteriosus.A hypermobile septum and reverse diastolic patent ductus arteriosus are new prenatal findings to help predict the need for an urgent BAS postnatally in patients with complete transposition of the great vessels.

  View details for DOI 10.1016/j.echo.2010.12.020

  View details for Web of Science ID 000288781700011

  View details for PubMedID 21324642

• Use of Intravenous Gamma Globulin and Corticosteroids in the Treatment of Maternal Autoantibody-Mediated Cardiomyopathy JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Trucco, S. M., Jaeggi, E., Cuneo, B., Moon-Grady, A. J., Silverman, E., Silverman, N., Hornberger, L. K. 2011; 57 (6): 715–23

  Abstract

  This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy.We have previously shown that 85% of fetuses and infants with maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis suffer demise or need for transplant. In an attempt to improve this outcome, in 1998, we began to empirically treat affected patients with IVIG and corticosteroids.We reviewed the clinical records and echocardiograms of 20 affected patients encountered in our institutions and treated with IVIG and corticosteroids from 1998 to 2009.All 20 were initially referred at a median gestational age of 23 weeks (range 18 to 38 weeks). Nineteen mothers were anti-Ro antibody positive, 8 anti-La antibody positive, and 7 had clinical autoimmune disease. Endocardial fibroelastosis was seen in 16 and was not obvious in 4 others with reduced ventricular function, and 16 (80%) had reduced or borderline ventricular shortening fraction (≤30%) before or after birth. Eighteen had atrioventricular block at referral (16 in 3°). During pregnancy, maternal IVIG was given in 9 and dexamethasone in 17. After birth, 17 infants received IVIG (n = 14) and/or corticosteroids (n = 15). Twelve underwent pacemaker implantation. Four with hydrops at presentation died perinatally, despite initial improvement in function in 3. At a median follow-up of 2.9 years (1.1 to 9.8 years), 16 (80%) patients are currently alive with normal systolic ventricular function and 6 are not paced.Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. Further studies are needed to determine the optimal dose and timing of IVIG administration.

  View details for DOI 10.1016/j.jacc.2010.09.044

  View details for Web of Science ID 000286880100012

  View details for PubMedID 21292131

• Aortic and Mitral Valve Stenosis with Regurgitation: Not Due to Rheumatic Heart Disease ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Talluto, C. J., Silverman, N. H. 2011; 28 (2): E24-E27

  Abstract

  The patient is a 13-year-old Mexican-American immigrant who had no previous medical care. Upon arrival to the United States she was diagnosed with severe mitral valve stenosis and regurgitation. In addition she had severe aortic stenosis with mild to moderate aortic valve regurgitation, which was thought to be rheumatic heart disease. On the basis of the clinical and echocardiographic findings she was taken to the operating room for both mitral and aortic valve replacement. Her operation was complicated by the discovery that her aorta was completely calcified, as were her coronary arteries, mitral valve and aortic valve. She underwent aortic and mitral valve replacement as well as replacement of her aortic arch. Her coronary arteries were patch augmented and reimplanted into the newly created ascending aorta. Based on the pathologic examination of the surgical tissue a diagnosis of Gaucher disease was made.

  View details for DOI 10.1111/j.1540-8175.2010.01253.x

  View details for Web of Science ID 000286669300002

  View details for PubMedID 20718842

• Anomalous coronary arteries from the opposite sinus of Valsalva in asymptomatic siblings treated with pulmonary arterial translocation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Obayashi, D. Y., Yeh, J., Silverman, N. H., Hanley, F. L. 2010; 140 (6): 1430-1432

  View details for DOI 10.1016/j.jtcvs.2010.05.012

  View details for Web of Science ID 000284149200036

  View details for PubMedID 21078428

• Echocardiography of the aortopulmonary window, aorto-ventricular tunnels, and aneurysm of the sinuses of Valsalva CARDIOLOGY IN THE YOUNG Mahle, W. T., Kreeger, J., Silverman, N. H. 2010; 20: 100-106

  Abstract

  There is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.

  View details for DOI 10.1017/S1047951110001149

  View details for Web of Science ID 000285980900012

  View details for PubMedID 21087565

• Echocardiographic imaging of anomalous origin of the coronary arteries CARDIOLOGY IN THE YOUNG Cohen, M. S., Herlong, R. J., Silverman, N. H. 2010; 20: 26-34

  Abstract

  In the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

  View details for DOI 10.1017/S104795111000106X

  View details for Web of Science ID 000285980900004

  View details for PubMedID 21087557

• Surgical management of life threatening events caused by intermittent aortic insufficiency in a native valve: case report JOURNAL OF CARDIOTHORACIC SURGERY Martin, M. H., Perry, S. B., Prochazka, J. V., Hanley, F. L., Silverman, N. H. 2010; 5

  Abstract

  We describe a case of a patient admitted with apparent life threatening events characterized by hypotension and bradycardia. The patient was ultimately found to have intermittent severe aortic insufficiency. Upon surgical exploration, abnormalities were discovered in the aortic valve, which had a small left coronary cusp with absence of the nodulus of Arantius. Following surgical repair of the valve, aimed at preventing the small cusp from becoming stuck in the open position, the patient has remained episode free for over one year.

  View details for DOI 10.1186/1749-8090-5-94

  View details for PubMedID 21034457

• A continuous heparin infusion does not prevent catheter-related thrombosis in infants after cardiac surgery PEDIATRIC CRITICAL CARE MEDICINE Schroeder, A. R., Axelrod, D. M., Silverman, N. H., Rubesova, E., Merkel, E., Roth, S. J. 2010; 11 (4): 489-495

  Abstract

  To determine whether a continuous infusion of heparin reduces the rate of catheter-related thrombosis in neonates and infants post cardiac surgery. Central venous and intracardiac catheters are used routinely in postoperative pediatric cardiac patients. Catheter-related thrombosis occurs in 8% to 45% of pediatric patients with central venous catheters.Single-center, randomized, placebo-controlled, double-blinded trial.Cardiovascular intensive care unit, university-affiliated children's hospital.Children <1 yr of age recovering from cardiac surgery.Patients were randomized to receive either continuous heparin at 10 units/kg/hr or placebo. The primary end point was catheter-related thrombosis as assessed by serial ultrasonography.Study enrollment was discontinued early based on results from an interim futility analysis. Ninety subjects were enrolled and received the study drug (heparin, 53; placebo, 37). The catheter-related thrombosis rate in the heparin group, compared with the placebo group, was 15% vs. 16% (p = .89). Subjects in the heparin group had a higher mean partial thromboplastin time (52 secs vs. 42 secs, p = .001), and this difference was greater for those aged <30 days (64 secs vs. 43 secs, p = .008). Catheters in place > or = 7 days had both a greater risk of thrombus formation (odds ratio, 4.3; p = .02) and catheter malfunction (odds ratio, 11.2; p = .008). We observed no significant differences in other outcome measures or in the frequency of adverse events.A continuous infusion of heparin at 10 units/kg/hr was safe but did not reduce catheter-related thrombus formation. Heparin at this dose caused an increase in partial thromboplastin time values, which, unexpectedly, was more pronounced in neonates.

  View details for DOI 10.1097/PCC.0b013e3181ce6e29

  View details for PubMedID 20101197

• Response to "Chromosomal Abnormalities and Neuromuscular Disorders Predict Severity and Outcome of Noncompaction in Addition to Cardiac Comorbidities" JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Silverman, N. H. 2010; 23 (3): 338–39

  View details for DOI 10.1016/j.echo.2010.01.016

  View details for Web of Science ID 000275221900016

• Cardiac Segmental Analysis in Left Ventricular Noncompaction: Experience in a Pediatric Population JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Punn, R., Silverman, N. H. 2010; 23 (1): 46-53

  Abstract

  Echocardiography has been used to diagnose and describe left ventricular noncompaction (LVNC). No other study has investigated LVNC using the 16-segment model described by the American Heart Association and the American Society of Echocardiography in children, some of whom have congenital heart disease. Using the ratio of noncompaction to compaction, the authors analyzed the 16 segments and determined if severity was correlated with poor outcomes in a pediatric population.The 16-segment noncompaction/compaction ratio, shortening, and ejection fractions were measured retrospectively in all children with LVNC at a single institution from January 1, 2000, to June 30, 2008.Forty-four patients had LVNC, an incidence of 0.3% of laboratory admissions. Twenty-eight patients (64%) who remained alive were assigned to group 1, and 16 patients (36%) who either died or were transplanted constituted group 2. Group 2 had more patients with significant associated congenital heart disease than group 1 (50% vs 18%, P < .05). We found similar regions of involvement in the 16-segment model with sparing of basal segments and involvement of the midpapillary and apical regions (P < .001); however, patients in group 2 were noted to have more segments involved (6 vs 4, P < .05), lower shortening fractions (16% vs 29%, P < .001), and lower ejection fractions (24% vs 47%, P < .001). The ejection fraction was inversely related to the number of segments (r = -0.63, P < .01), suggesting that more noncompaction portends a worse outcome.In younger patients with noncompaction, poor outcomes such as low ejection fractions, death, and transplantation are related to the number of left ventricular segments involved. There is more associated congenital heart disease in the pediatric population, which carries a poorer prognosis than the disease reported in adult populations.

  View details for DOI 10.1016/j.echo.2009.09.003

  View details for Web of Science ID 000273052600011

  View details for PubMedID 19857942

• A Simple Echocardiographic Screening Tool for Aortic Root Dilatation in Children With Marfan Syndrome Kemna, M., Murphy, D., Silverman, N. LIPPINCOTT WILLIAMS & WILKINS. 2009: S597–S598

  View details for Web of Science ID 000271831501407

• Screening for Aortic Root Dilation in Marfan Syndrome Using the Ratio of the Aortic Root to Descending Aortic Diameters in Children JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Kemna, M. S., Murphy, D. J., Silverman, N. H. 2009; 22 (10): 1109-1113

  Abstract

  The aim of this study was to develop a simpler echocardiographic screening tool for aortic root dilation in pediatric patients with Marfan syndrome. Aortic root dilation represents the most common cause of morbidity and mortality in patients with Marfan syndrome. It is traditionally defined by nomograms, relating aortic root dimensions to height or body surface area. In this study, the descending aorta (DAo) was used as an internal reference, thereby eliminating the need for nomograms when screening for aortic root dilation.The diameters of the aortic root at the sinus of Valsalva and the DAo were measured in 35 patients with Marfan syndrome and 52 normal controls in the parasternal long-axis echocardiographic view. The root/DAo ratio was calculated.A root/DAo ratio > or = 2 provided optimal sensitivity for root dilation (100%), whereas a root/DAo ratio > or = 2.3 provided optimal specificity (100%).The root/DAo ratio provides a simple, rapid, and valuable screening test for aortic root dilation, independent of nomograms and body size.

  View details for DOI 10.1016/j.echo.2009.06.002

  View details for Web of Science ID 000270367900003

  View details for PubMedID 19647408

• Doppler Assessment of the Ratio of the Systolic to Diastolic Duration in Normal Children: Relation to Heart Rate, Age and Body Surface Area JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Sarnari, R., Kamal, R. Y., Friedberg, M. K., Silverman, N. H. 2009; 22 (8): 928-932

  Abstract

  It has been shown that the ratio of systolic duration to diastolic duration (S/D) is a valuable global index of ventricular dysfunction in pediatric dilated and restrictive cardiomyopathy and is also a valuable index of the ventricular function of single systemic right ventricles in children who have undergone palliation of hypoplastic left-heart syndrome. The purposes of this study were to establish normal values for the S/D ratio in children without heart disease and to investigate its variation with heart rate (HR), age, and body surface area.Of 752 children found to have functional murmurs by clinical means, 179 (24% of the total database) had trace holosystolic tricuspid regurgitation. These patients ranged in age from 0.02 months to 19 years. For this study, the diastolic interval was defined as the period of right ventricular filling (ie, the period between 2 tricuspid regurgitant jets) and the systolic interval as the remainder of the cardiac cycle corresponding to the duration of holosystolic tricuspid regurgitation, including the periods of isovolumic contraction and relaxation. The relations between the systolic and diastolic periods and their ratio (S/D ratio) and HR, age, and body surface area were evaluated using univariate and multivariate linear regression analysis.Patient's age ranged from 0.02 months to 19 years (mean, 70.18+/-65.12 months), body surface area from 0.11 to 2.51 m2 (mean, 0.85+/-0.55 m2), and HR from 50 to 156 beats/min (mean, 96.72+/-23.19 beats/min). The systolic period ranged from 208.5 to 467 ms (mean, 314.08+/-52.57 ms) and the diastolic period from 166.5 to 809 ms (mean, 341.34+/-129.61 ms), yielding a S/D ratio ranging from 0.397 to 1.62 (mean, 0.995+/-0.23). Systolic period duration showed a linear negative decrease with increasing HR (y=1.9228x+500.05, r=-0.85). Diastolic duration decreased in an exponential fashion as HR increased (y=130,679x(-1.3232), r=-0.88). The S/D ratio correlated positively with HR (y=-1.656+0.0265 HR-0.000954 HR2, r=76). On multivariate analysis, no significant correlations of S/D ratio with age or body surface area were found.The results of this study provide useful reference values for the S/D ratio across a wide range of HRs in children, adolescents, and young adults.

  View details for DOI 10.1016/j.echo.2009.05.004

  View details for Web of Science ID 000268503400012

  View details for PubMedID 19553081

• Prenatal Detection of Congenital Heart Disease JOURNAL OF PEDIATRICS Friedberg, M. K., Silverman, N. H., Moon-Grady, A. J., Tong, E., Nourse, J., Sorenson, B., Lee, J., Hornberger, L. K. 2009; 155 (1): 26-31

  Abstract

  To define current frequency of prenatal detection of congenital heart disease (CHD), factors affecting prenatal detection, and its influence on postnatal course.We prospectively identified all fetuses and infants < or =6 months of age with major CHD at 3 referral centers in Northern California over 1 year; we obtained prenatal and demographic data, reviewed prenatal ultrasound (US) and postnatal records, and used logistic regression to analyze maternal, fetal, and prenatal-care provider risk factors for prenatal diagnosis.Ninety-eight of 309 infants with major CHD had prenatal diagnosis (36% accounting for 27 pregnancy terminations); 185 infant-families participated in the postnatal survey, and although 99% had prenatal US, only 28% were prenatally diagnosed. Anomalous pulmonary venous return (0%), transposition of the great arteries (19%), and left obstructive lesions (23%) had the lowest prenatal detection. Heterotaxy (82%), single ventricle (64%), and HLHS (61%) had the highest. Prenatal diagnosis was higher at university versus community practices (P = .001). Sociodemographics were not associated with prenatal diagnosis. Infants diagnosed prenatally were less frequently ventilated (P < .01) or treated with prostaglandin (P < .05).Prenatal detection of major CHD significantly alters postnatal course but remains low despite nearly universal US. CHD type and US practice type are important determinants of prenatal detection.

  View details for DOI 10.1016/j.jpeds.2009.01.050

  View details for Web of Science ID 000267672600010

  View details for PubMedID 19394031

• Anatomy, echocardiography, and surgical approach to double outlet right ventricle 8th International Symposium on Congenital Heart Disease Mahle, W. T., Martinez, R., Silverman, N., Cohen, M. S., Anderson, R. H. CAMBRIDGE UNIV PRESS. 2008: 39–51

  View details for Web of Science ID 000266381000006

  View details for PubMedID 19094378

• Morphological studies of pulmonary arteriovenous shunting in a lamb model of superior cavopulmonary anastomosis PEDIATRIC CARDIOLOGY McMullan, D. M., Reddy, V. M., Gottliebson, W. M., Silverman, N. H., Perry, S. B., Chan, F., Hanley, F. L., Riemer, R. K. 2008; 29 (4): 706-712

  Abstract

  We sought to identify and characterize the abnormal vascular structures responsible for pulmonary arteriovenous shunting following the Glenn cavopulmonary shunt. Superior cavopulmonary shunt is commonly performed as part of the staged pathway to total cavopulmonary shunt to treat univentricular forms of congenital heart disease, however, clinically significant pulmonary arteriovenous malformations develop in some patients after the procedure. The causes of pulmonary arteriovenous malformations and other pulmonary vascular changes that occur after cavopulmonary shunt are not known. Using a juvenile lamb model of superior cavopulmonary anastomosis that reliably produces pulmonary arteriovenous malformations, we performed echocardiography and morphological analyses to determine the anatomic site of shunting and to identify the vascular structures involved. Pulmonary arteriovenous shunting was identified by contrast echocardiography in all surviving animals (n = 40) following superior cavopulmonary anastomosis. Pulmonary vascular corrosion casts revealed abnormal tortuous vessels joining pulmonary arteries and veins in cavopulmonary shunt animals but not control animals. In conclusion, unusual channels that bridged pulmonary arteries and veins were identified. These may represent the vascular structures responsible for arteriovenous shunting following the classic Glenn cavopulmonary shunt. Detailed analysis of these structures may elucidate factors responsible for their development.

  View details for DOI 10.1007/s00246-007-9152-5

  View details for Web of Science ID 000257393800003

  View details for PubMedID 18043856

  View details for PubMedCentralID PMC2970565

• Idiopathic premature closure of the ductus arteriosus: An indication for early delivery ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Soslow, J. H., Friedberg, M. K., Silverman, N. H. 2008; 25 (6): 650-652

  Abstract

  Idiopathic intrauterine closure of the ductus arteriosus, distinct from intrauterine constriction associated with maternal NSAID ingestion or other secondary causes, is an uncommon event that often results in significant morbidity and mortality. We report on a 32-week fetus with meconium pseudocyst that presented with idiopathic intrauterine constriction of the ductus arteriosus, and review the literature. We suggest that early diagnosis and judicious obstetrical management, in conjunction with pediatric cardiology consultation based on serial fetal echocardiography, can lead to timely delivery and reversal of the pathophysiology providing the potential for a favorable outcome.

  View details for DOI 10.1111/j.1540-8175.2008.00667.x

  View details for Web of Science ID 000257653600015

  View details for PubMedID 18422673

• Extension of Doppler-derived echocardiographic measures of pulmonary vascular resistance to patients with moderate or severe pulmonary vascular disease JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Vlahos, A. P., Feinstein, J. A., Schiller, N. B., Silverman, N. H. 2008; 21 (6): 711-714

  Abstract

  Pulmonary vascular resistance (PVR) is a critical parameter in the assessment and treatment of patients with pulmonary hypertension, regardless of origin. Noninvasive estimation of PVR could be helpful.Consecutive patients with known or suggested pulmonary hypertension referred for cardiac catheterization were evaluated prospectively and the PVR was calculated invasively. Subsequently, the tricuspid regurgitation velocity (TRV), the velocity-time integral (mean of 3 measurements) of the right ventricular outflow tract (VTIm), and the right ventricular outflow tract diameter were recorded noninvasively.The TRV/VTIm ratio and the TRV/VTIm corrected for the indexed RVOT diameter correlated well with the PVR at catheterization with R(2) = 0.711 and R(2) = 0.731, respectively, including patients with very high values of PVR. A TRV/VTI(RVOT) value of 38 provided a specificity of 100% for a PVR of 8 Woods units.Noninvasive estimation is feasible over a broad range of PVR values and could be a useful tool to estimate and longitudinally tracked changes in PVR.

  View details for DOI 10.1016/j.echo.2007.10.004

  View details for Web of Science ID 000256283900008

  View details for PubMedID 18187297

• Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches 85th Annual Meeting of the American-Association-for-Thoracic-Surgery Gulati, R., Reddy, V. M., Culbertson, C., Helton, G., Suleman, S., Reinhartz, O., Silverman, N., Hanley, F. L. MOSBY-ELSEVIER. 2007: 1171–U12

  Abstract

  Patients with a coronary artery arising from the wrong sinus are susceptible to ischemia and sudden death. Risk is higher when the artery courses interarterially--between the pulmonary artery and aorta--has an intramural course, or has an abnormal orifice. In single coronary ostium without intramural course, unroofing and coronary reimplantation are inappropriate, and coronary artery bypass grafting is suboptimal. For this variant, we have devised pulmonary artery translocation.A retrospective review of 18 patients undergoing repair between January 1999 and March 2005 was performed. Mean age was 8.1 years (range 6 weeks-16 years). All anomalous arteries coursed interarterially. Ten patients had a right coronary artery from the left coronary sinus; 8 had a left coronary artery from the right sinus. Eleven had an intramural course, and 4 had a single coronary ostium without an intramural course. Ten (56%) patients had symptoms: chest pain (9/10), syncope (3/10), or dyspnea (2/10). Repair was implemented by unroofing (n = 11), reimplantation (n = 3), or pulmonary artery translocation (1 lateral, 3 anterior). All patients were followed up clinically and echocardiographically.At a mean of 2.2 years (2 weeks-5 years), there was no mortality. Symptoms improved and function remained normal in all but 1 patient. He had sustained multiple infarcts in the anomalous artery's distribution and required transplantation despite repair.Repair is indicated in all patients with coronary insufficiency and in asymptomatic patients with high-risk morphologic abnormalities. We recommend unroofing when an intramural component (or slit-like orifice) is present, reimplantation for separate ostia without an intramural course, and pulmonary artery translocation for single ostium without an intramural course. Coronary artery bypass grafting is thus avoided.

  View details for DOI 10.1016/j.jtcvs.2007.02.051

  View details for Web of Science ID 000250576200011

  View details for PubMedID 17976445

• Heparin and catheter-related thrombosis in infants following cardiac surgery: Interim results from a randomized trial 80th Annual Scientific Session of the American-Heart-Association (AHA) Schroeder, A. R., Silverman, N., Axelrod, D. M., Merkel, E., Roth, S. J., Packard, L. LIPPINCOTT WILLIAMS & WILKINS. 2007: 515–15

  View details for Web of Science ID 000250394302357

• Right ventricular mechanical dyssynchrony in children with hypoplastic left heart syndrome JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Friedberg, M. K., Silverman, N. H., Dubin, A. M., Rosenthal, D. N. 2007; 20 (9): 1073-1079

  Abstract

  Mechanical dyssynchrony predicts response to cardiac resynchronization therapy in adults with heart failure. Children with hypoplastic left heart syndrome (HLHS) are susceptible to right ventricular (RV) failure; however, mechanical dyssynchrony has not been studied in this population with newly available methodologies. We investigated RV mechanical dyssynchrony in children with HLHS using vector velocity imaging.We used vector velocity imaging to quantify the SD of time to peak velocity, strain, and strain rate among 6 RV segments to define intraventricular RV synchrony in 16 children with HLHS and RV and left ventricular (LV) synchrony in 16 healthy age-matched control subjects. We further investigated relations between QRS duration and mechanical dyssynchrony and between mechanical dyssynchrony and systolic function.Children with HLHS had significant RV mechanical dyssynchrony versus LV and RV control subjects (strain 37 +/- 35 vs 8 +/- 8 milliseconds, P = .003 [LV], 9 +/- 11 milliseconds, P = .005 [RV]; strain rate 31 +/- 37 vs 10 +/- 13 milliseconds, P = .04 [LV], 14 +/- 15 milliseconds, P = .09 [RV]). There was no significant relationship between QRS duration and mechanical dyssynchrony and no obvious relation between the degree of mechanical dyssynchrony and the RV fractional area of change.Children with HLHS have RV mechanical dyssynchrony unrelated to surface electrocardiographic QRS duration. This may contribute to RV dysfunction and may indicate the usefulness of cardiac resynchronization therapy in this population.

  View details for DOI 10.1016/j.echo.2007.02.015

  View details for Web of Science ID 000249454900007

  View details for PubMedID 17566698

• The systolic to diastolic duration ratio in children with hypoplastic left heart syndrome: A novel doppler index of right ventricular function JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Friedberg, M. K., Silverman, N. H. 2007; 20 (6): 749-755

  Abstract

  Right ventricular (RV) function is an important determinant of clinical status in children with hypoplastic left heart syndrome (HLHS). However, assessment of RV function remains challenging because of its complex morphology. We investigated the S/D duration ratio in children with HLHS as a novel index of global RV function.We measured systolic (S) and diastolic (D) duration using tricuspid regurgitation duration from Doppler flow, to calculate the S/D ratio in 33 children with HLHS and 33 control subjects matched for age and sex. We compared the S/D ratio between patients with HLHS and control subjects, between patients with HLHS and normal and abnormal RV function, and between patients with HLHS at different stages of palliation. We further correlated the S/D ratio with catheterization data.Patients and control subjects were well matched for age (3.12 +/- 4.5 vs 3 +/- 4.5 years, not significant) sex, and heart rate (cycle length 524 +/- 179 vs 575 +/- 162 milliseconds, not significant). Patients with HLHS had a significantly higher S/D ratio than control subjects (1.65 +/- 0.85 vs 0.85 +/- 0.2, P < .0001). The S/D ratio became increasingly elevated in HLHS at higher heart rates, but not in control subjects. The S/D ratio was significantly higher in patients with HLHS and decreased RV function as compared with patients with HLHS and normal RV function (2.2 +/- 0.7 vs 1.5 +/- 0.47, P = .006), and significantly increased in patients with Norwood stage 1 versus patients with Norwood stages 2 and 3 HLHS (2.16 vs 1.4 and 1.32, respectively, P < .01 and P < .001, respectively). The S/D ratio did not correlate with catheterization-derived RV end-D pressure or cardiac index.Patients with HLHS have an increased S/D ratio as a result of a shortened D and prolonged S. Measurement of the S/D duration ratio using Doppler flow is a novel method to augment assessment of global RV function in HLHS.

  View details for DOI 10.1016/j.echo.2006.11.014

  View details for Web of Science ID 000247090900011

  View details for PubMedID 17543747

• Mechanical dyssynchrony in children with systolic dysfunction secondary to cardiomyopathy: A Doppler tissue and vector velocity imaging study JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Friedberg, M. K., Silverman, N. H., Dubin, A. M., Rosenthal, D. N. 2007; 20 (6): 756-763

  Abstract

  Mechanical dyssynchrony is common in adults with heart failure and its presence predicts response to cardiac resynchronization therapy. However, mechanical dyssynchrony and its quantification by echocardiography have not been extensively studied in children with cardiomyopathy. We investigated mechanical dyssynchrony in children with systolic dysfunction secondary to cardiomyopathy using Doppler tissue imaging (DTI) and vector velocity imaging (VVI).We used DTI and VVI to quantify mechanical dyssynchrony in 22 children with systolic dysfunction secondary to cardiomyopathy and in 25 healthy control subjects. We analyzed DTI results corrected for cardiac dimensions and evaluated correlation between electrical and mechanical dyssynchrony and between mechanical dyssynchrony and systolic function.DTI and VVI revealed significant mechanical dyssynchrony among children with cardiomyopathy. Intraventricular and interventricular delays as defined by DTI, and the SD of time to peak velocity, strain, and strain rate as defined by VVI were 2 to 3 times higher in patients with cardiomyopathy as compared with control subjects. There was no significant relationship between electrical and mechanical dyssynchrony.Children with systolic dysfunction secondary to cardiomyopathy have mechanical dyssynchrony, unrelated to electrical dyssynchrony, which can be measured by recent echocardiographic techniques including DTI and VVI. Children with cardiomyopathy and mechanical dyssynchrony are potential candidates for cardiac resynchronization therapy.

  View details for DOI 10.1016/j.echo.2006.11.007

  View details for Web of Science ID 000247090900012

  View details for PubMedID 17543748

• Diagnosis of patent ductus arteriosus by a neonatologist with a compact, portable ultrasound machine JOURNAL OF PERINATOLOGY Lee, H. C., Silverman, N., Hintz, S. R. 2007; 27 (5): 291-296

  Abstract

  To conduct a pilot study assessing a neonatologist's accuracy in diagnosing patent ductus arteriosus (PDA) using compact, portable ultrasound after limited training.Prospective study of premature infants scheduled for echocardiography for suspected PDA. A neonatologist with limited training performed study exams before scheduled exams. Sensitivity and specificity were calculated, compared to the scheduled echocardiogram interpreted by a cardiologist.There were 24 exams. Compared to the scheduled exam, the neonatologist's exam had sensitivity 69% (95% confidence interval (CI), 41 to 89%) and specificity 88% (95% CI, 47 to 99%). When a cardiologist interpreted the study exams, the sensitivity was 87% (95% CI, 60 to 98%) and specificity 71% (95% CI, 29 to 96%).A neonatologist with limited training was able to detect PDA with moderate success. A more rigorous training process or real-time transmission with cardiologist interpretation may substantially improve accuracy. Institutions with experienced technicians and on-site pediatric cardiologists may not gain from intensive training of neonatologists, but hospitals where diagnosis and treatment of PDA would be delayed may benefit from such processes.

  View details for DOI 10.1038/sj.jp.7211693

  View details for Web of Science ID 000246105400008

  View details for PubMedID 17363908

• Right ventricular mechanical dyssynchrony in children with hypoplastic left heart syndrome: A target for cardiac resynchronization therapy? Friedberg, M. K., Silverman, N. H., Dubin, A. M., Rosenthal, D. N. ELSEVIER SCIENCE INC. 2007: 257A

  View details for Web of Science ID 000244651801499

• Echocardiographic diagnosis of persistent fifth aortic arch ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Bernheimer, J., Friedberg, M., Chan, F., Silverman, N. 2007; 24 (3): 258-262

  Abstract

  We describe echocardiography and computerized tomographic imaging findings in the two subtypes of fifth aortic arch in infants. In the first form, the fifth aortic arch creates a systemic to systemic connection extending from the ascending to the descending aorta. When this form appears in isolation, it does not cause hemodynamic disturbance, although associated cardiac lesions, such as aortic coarctation or interruption and patent ductus arteriosus, may influence the clinical presentation. In the second form the fifth aortic arch connects the systemic and pulmonary circulations from persistent connections between the embryological fifth and sixth arches, which may cause significant hemodynamic disturbance from left to right shunting. Although this form has been associated with a wide array of congenital cardiac lesions, this is the first description of 5th aortic arch in association with atrioventricular septal defect and double outlet right ventricle.

  View details for DOI 10.1111/j.1540-8175.2007.00383.x

  View details for Web of Science ID 000244343900007

  View details for PubMedID 17313637

• The systolic to diastolic duration ratio in children with heart failure secondary to restrictive cardiomyopathy JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Friedberg, M. K., Silverman, N. H. 2006; 19 (11): 1326-1331

  Abstract

  Systole (S) and diastole (D) are fundamental to cardiac function, yet their durations are not routinely evaluated. We defined the S/D duration ratio in 11 children with restrictive cardiomyopathy (CM) and 31 control subjects, using tricuspid regurgitant flow duration. We correlated S/D ratio, S duration, and D duration with heart rate and compared groups. Heart rates of control subjects and patients with restrictive CM were similar (P = .995). The S/D ratio was higher in patients with restrictive CM than in control subjects (1.6 +/- 0.81 vs 0.8 +/- 0.19, P < .001) as a result of shortened D (0.43 +/- 0.13 vs 0.56 +/- 0.06, P < .01) and prolonged S (0.58 +/- 0.12 vs 0.44 +/- 0.06, P < .01). The S/D ratio was highly correlated to heart rate in restrictive CM (r = 0.95, P < .0001). The high S/D ratio found in restrictive CM, found also in dilated CM, suggests that this index is generic to heart failure rather than to a specific disease. The S/D ratio is easily measured using Doppler flow, enhancing echocardiographic assessment of D function in children.

  View details for DOI 10.1016/j.echo.2006.05.024

  View details for Web of Science ID 000242280700005

  View details for PubMedID 17098134

• Segmental wall-motion abnormalities after an arterial switch operation indicate ischemia ANESTHESIA AND ANALGESIA Rouine-Rapp, K., Rouillard, K. P., Miller-Hance, W., Silverman, N. H., Collins, K. K., Cahalan, M. K., Bostrom, A., Russell, I. A. 2006; 103 (5): 1139–46

  Abstract

  We prospectively studied 29 consecutive neonates undergoing an arterial switch operation to determine if segmental wall motion abnormalities (SWMA) represented myocardial ischemia. Intraoperative transesophageal echocardiogram was recorded at baseline and twice after cardiopulmonary bypass. Cardiac troponin I (cTnI) levels were measured before sternal incision and 3, 6, 12, 24, 48, and 72 h after removal of the aortic cross-clamp. Immediate postoperative Holter and 15-lead electrocardiograms (ECG) were evaluated for ischemia. Transthoracic echocardiograms were obtained before hospital discharge. At bypass termination, immediately after protamine administration, segmental wall motion was normal in nine neonates and abnormal in 20. SWMA were transient in five and present at the time of chest closure in 15 neonates. Neonates in whom SWMA were present at chest closure had more segments involved than those in whom SWMA were transient (P > 0.001). Neonates with SWMA at chest closure had higher cTnI levels postoperatively versus neonates with normal wall motion (P = 0.02). Postoperative ECG data were available in 26 neonates. There was ECG evidence of myocardial ischemia in two of eight neonates with normal wall motion, one of five with transient SWMA, and nine of 13 with SWMA at chest closure. CTnI levels at 12, 24, and 48 h and intraoperative SWMA were predictive of postoperative SWMA. We believe these data indicate that SWMA, which persist at the completion of an arterial switch operation, and which are present in multiple myocardial segments, correlate with myocardial ischemia. Further follow-up of these patients is needed to determine if increased intraoperative myocardial ischemia correlates with long-term outcomes.

  View details for DOI 10.1213/01.ane.0000240874.26646.d0

  View details for Web of Science ID 000241570600014

  View details for PubMedID 17056946

• Morphology and function of the bicuspid aortic valve with and without coarctation of the aorta in the young AMERICAN JOURNAL OF CARDIOLOGY Ciotti, G. R., Vlahos, A. P., Silverman, N. H. 2006; 98 (8): 1096–1102

  Abstract

  This study sought to determine the morphology and function of bicuspid aortic valves (BAVs) with and without coarctation of the aorta (CoA) in a young population. The transthoracic echocardiograms of 117 patients with BAVs and 62 patients with CoA were retrospectively reviewed and compared with normal transthoracic echocardiographic results. In each patient, the area subtended by the aortic cusps and valve and the diameters of the aortic root at different levels were measured, and additionally in each BAV, the type of cusp fusion and the presence and degree of aortic stenosis and/or regurgitation were evaluated. The median age of patients with BAVs was 4 years (range 0 days to 34 years), and the median age of patients with CoA was 1.9 years (range 0 days to 16.5 years). BAVs with right and left coronary cusp fusion were significantly associated with CoA (p <0.0001) and cardiac anomalies (p <0.0001), whereas BAVs with noncoronary and right coronary cusp fusion were affected by valvar dysfunction (p <0.001). Compared with normal tricuspid aortic valves, BAVs had aortic root dilation, even in patients with no hemodynamic disturbance, particularly at the level of the ascending aorta (p <0.0001); the difference was still significant comparing BAVs and CoA with tricuspid aortic valves and CoA (p <0.0001). In conclusion, different morphologies of BAVs are associated with different cardiac abnormalities, valvar function, and aortic root dilation. Although detectable early in life, valvar dysfunction and aortic root dilation progress with age.

  View details for DOI 10.1016/j.amjcard.2006.05.035

  View details for Web of Science ID 000241427400022

  View details for PubMedID 17027579

• Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: Two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies EUROPEAN JOURNAL OF ECHOCARDIOGRAPHY Greenway, S. C., Bradley, T. J., Caldarone, C. A., Silverman, N. H., Hanley, F. L., Smallhorn, J. F. 2006; 7 (5): 379-382

  Abstract

  This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.

  View details for DOI 10.1016/j.euje.2005.10.010

  View details for Web of Science ID 000242966600007

  View details for PubMedID 16356774

• Diagnosis and prognosis of fetuses with double outlet right ventricle PRENATAL DIAGNOSIS Kim, N., Friedberg, M. K., Silverman, N. H. 2006; 26 (8): 740-745

  Abstract

  To examine the echocardiographic features, associated anomalies, and outcomes of fetuses diagnosed with double outlet right ventricle (DORV).Fetuses with DORV were identified from 987 fetal echocardiograms performed from August 1, 2002 to August 1, 2004. The medical records of 19 fetuses with DORV were reviewed.Nineteen fetuses were diagnosed with DORV. The pregnancy was terminated in 15.8% (3/19) of cases. Postnatal assessment was not obtained in 26.3% (5/19) cases. Accurate prenatal diagnosis of the relationship of the ventricular septal defect (VSD) with the great arteries was achieved in 14 of 14 cases (100%). Chromosomal abnormalities (21.4%), abnormalities of body situs (35.7%), and other extracardiac abnormalities were diagnosed in association. Only 4 of 13 (30.8%) infants with confirmed DORV survived beyond 28 days.DORV is found in fetuses with a large spectrum of associated cardiac and extracardiac lesions. The overall prognosis for fetuses with DORV is poor throughout the spectrum of these lesions, not only related to the primary lesion but also depending on the associated intra- and extracardiac abnormalities. While the complexity of the heart disease in fetuses with DORV makes detailed fetal diagnosis challenging, it can be accomplished with a high degree of accuracy.

  View details for DOI 10.1002/pd.1500

  View details for Web of Science ID 000239931300018

  View details for PubMedID 16807954

• Images in cardiovascular medicine. Near sudden death from cardiac lipoma in an adolescent. Circulation Friedberg, M. K., Chang, I. L., Silverman, N. H., Ramamoorthy, C., Chan, F. P. 2006; 113 (21): e778-9

  View details for PubMedID 16735681

• Near sudden death from cardiac lipoma in an adolescent CIRCULATION Friedberg, M. K., Chang, I. L., Silverman, N. H., Ramamoorthy, C., Chan, F. P. 2006; 113 (21): E778–E779

  View details for DOI 10.1161/CIRCULATIONAHA.105.589630

  View details for Web of Science ID 000237964600018

• Echo-morphological correlates concerning the functionally univentricular heart in the setting of isomeric atrial appendages 5th Annual International Symposium on Congenital Heart Disease Mahle, W. T., Silverman, N. H., Marx, G. R., Anderson, R. H. CAMBRIDGE UNIV PRESS. 2006: 35–42

  View details for DOI 10.1017/S1047951105002308

  View details for Web of Science ID 000241738500006

  View details for PubMedID 16401361

• Cardiac ventricular diastolic and systolic duration in children with heart failure secondary to idiopathic dilated cardiomyopathy AMERICAN JOURNAL OF CARDIOLOGY Friedberg, M. K., Silverman, N. H. 2006; 97 (1): 101-105

  Abstract

  Systole and diastole are the fundamental periods of the cardiac cycle, yet little emphasis has been placed on their relative duration when evaluating heart failure. Cardiac intervals are used to assess ventricular function, but the relative duration of systole and diastole for defining function have not been evaluated. We hypothesized that in heart failure, systole is prolonged and diastole shortened. We defined systole and diastole in 16 children with idiopathic dilated cardiomyopathy and in 16 normal controls, matched for age and gender, using the mitral regurgitant (MR) and tricuspid regurgitant (TR) flow duration. The systole and diastole durations (expressed as a fraction of the cardiac cycle) were correlated with heart rate and age and compared between groups. The subjects were compared with gender- and age-matched controls (9.98 +/- 6.1 vs 9.88 +/- 6.08 years, p = NS). The 2 groups had similar heart rates (104 +/- 31 vs 92 +/- 34 beats/min, p = NS). The systole duration was not significantly different when measured by MR versus TR duration (0.60 +/- 0.10 vs 0.57 +/- 0.11 of the cardiac cycle, respectively, p = NS). Systole was prolonged in subjects compared with controls (0.60 +/- 0.1 vs 0.42 +/- 0.08, respectively, using MR duration, p < 0.0001 and 0.57 +/- 0.11 vs 0.41 +/- 0.07, respectively, using TR duration, p = 0.0008). The systolic/diastolic ratio was 0.77 +/- 0.24 in the controls versus 1.57 +/- 0.98 in the patients with idiopathic dilated cardiomyopathy using the TR duration (p < 0.005) and 1.67 +/- 0.68 using the MR duration (p < 0.0001). The systole duration correlated with heart rate in subjects (r = 0.79, p = 0.0003) and controls (r = 0.69, p = 0.003). In conclusion, systole is significantly prolonged and diastole correspondingly shortened in heart failure. Reversal of the normal systolic/diastolic ratio may compromise cardiac filling and function. The systole and diastole duration are easily measured using routine Doppler flow, enhancing assessment of cardiac function in children.

  View details for Web of Science ID 000234587200021

  View details for PubMedID 16377292

• Isomerism of the left atrial appendage associated with ventricular noncompaction AMERICAN JOURNAL OF CARDIOLOGY Friedberg, M. K., Ursell, P. C., Silverman, N. H. 2005; 96 (7): 985-990

  Abstract

  This study investigated the rare association of ventricular noncompaction with isomerism of the left atrial appendage and heart block, demonstrating a dire prognosis for this condition. Prenatal recognition is possible by ultrasound, facilitating appropriate prenatal counseling.

  View details for DOI 10.1016/j.amjcard.2005.05.063

  View details for Web of Science ID 000232626400021

  View details for PubMedID 16188529

• Myofibroblastic tumor of the heart: A rare intracardiac tumor JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gandy, K. L., Burtelow, M. A., Reddy, V. M., Silverman, N. H. 2005; 130 (3): 888-889

  View details for DOI 10.1016/j.jtcvs.2005.02.018

  View details for Web of Science ID 000231915400046

  View details for PubMedID 16153948

• Right ventricular outflow tract obstruction in an infant. Heart Friedberg, M., Silverman, N. H. 2005; 91 (6): 748-?

  View details for PubMedID 15894767

  View details for PubMedCentralID PMC1768927

• Mitral valve leaflet excursion velocity in the pediatric population: A comparable diastolic index to the color m-mode propagation velocity JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Friedberg, M. K., Silverman, N. H. 2005; 18 (2): 142-147

  Abstract

  Color M-mode (CMM) Doppler flow propagation velocity (Vp) is a reliable diastolic index, but may be difficult to define. We hypothesized that because the valve is opened by flow, the anterior mitral leaflet excursion velocity (MEVApex ), interrogated from the cardiac apex, may be similar to the CMM-Vp. We investigated the MEVApex by apical M-mode echocardiography in 49 children and adolescents with normal echocardiograms, and compared it with the CMM-Vp and other indices. The mean CMM-Vp and MEVApex were similar (41.5 +/- 6 vs 40 +/- 5.7, respectively, mean +/- SD; P = .5) and correlated positively ( r = 0.71, P < .0001, Bland-Altman analysis). The peak velocity of mitral inflow (E wave) divided by the Vp (E/Vp) and E/MEVApex correlated well (2.54 +/- 0.58 vs 2.52 +/- 0.58, respectively, mean +/- SD; r = 0.857, P < .001, Bland-Altman analysis). The MEVApex is comparable with the CMM-Vp and the E/MEVApex with E/Vp. The Vp may be assessed either by CMM or conventional M-mode from the apex. In our experience, the latter is easier to do. Study in other populations is warranted.

  View details for DOI 10.1016/j.echo.2004.08.044

  View details for Web of Science ID 000226972000007

  View details for PubMedID 15682051

• Measurement of the Tei index: A comparison of m-mode and pulse Doppler methods JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Tham, E. B., Silverman, N. H. 2004; 17 (12): 1259-1265

  Abstract

  The Tei index is a useful echocardiographic measure of ventricular function in adults and children. Most studies have used pulse or tissue Doppler imaging to calculate this index. M-mode, with its higher frame rate and sharp deflections, may be an accurate method of calculating the Tei index.M-mode and pulse Doppler (PD) measurement of the Tei index were performed in 68 children. The mitral closure to opening time ("a") and ejection time ("b") intervals for calculating the Tei index were measured by M-mode and PD methods.Both M-mode and PD were found to be reproducible methods of calculating the Tei index. There was a close correlation between measurements of the "a" and "b" intervals by M-mode and PD, with less variability with M-mode than with PD.M-mode is a simple, reproducible echocardiographic alternative method for calculating the Tei index in children and has a lower variance than PD.

  View details for DOI 10.1016/j.echo.2004.07.009

  View details for Web of Science ID 000225590900008

  View details for PubMedID 15562264

• Changing indications for fetal echocardiography in a University Center population PRENATAL DIAGNOSIS Friedberg, M. K., Silverman, N. H. 2004; 24 (10): 781-786

  Abstract

  We hypothesized that increased facility with fetal echocardiographic diagnosis by obstetricians is associated with changes in its indications and yields.We reviewed 300 fetal echocardiograms (December 2002-August 2003) and compared our findings with previous studies.Mean maternal age was 31 +/- 6 (range 16-44) years. Gestational age was 24 +/- 5 weeks (mean +/- SD; median 22, range 15-38). Indications for fetal echocardiography included family history of congenital heart disease (CHD) (23%), maternal diabetes (18%), obstetrical scan suspicious for CHD (13%), arrhythmia (12%) maternal rheumatologic disease (7%), extracardiac congenital anomalies (6%), chromosomal anomaly (6%) and exposure to a potential fetal teratogen (5%). High yield indications included chromosomal anomaly (47%) and a suspicious obstetrical scan (42%). Low yield indications included family history of CHD (4%) and teratogen exposure (0%). 1/7 of the patients with increased nuchal translucency had pulmonary atresia/intact ventricular septum. No anomalies were associated with the single umbilical artery.Indications and yields of fetal echocardiography have changed over the last decade. The frequency of an obstetrical scan suspicious for CHD has increased 2.5 to 3 times over a decade and continues to have high yield. Thus, increasing prenatal detection of CHD depends, to a large extent, on increasing the skills of obstetricians.

  View details for DOI 10.1002/pd.981

  View details for Web of Science ID 000224872800005

  View details for PubMedID 15503290

• Changing indications for Fetal Echocardiography in a university center population Friedberg, M. K., Silverman, N. H. ELSEVIER SCIENCE INC. 2004: 380A

  View details for DOI 10.1016/S0735-1097(04)91605-1

  View details for Web of Science ID 000189388501607