Claudia Algaze

All Publications

• Intraoperative and Postoperative Hemodynamic Predictors of Acute Kidney Injury in Pediatric Heart Transplant Recipients. Journal of pediatric intensive care Hollander, S. A., Chung, S., Reddy, S., Zook, N., Yang, J., Vella, T., Navaratnam, M., Price, E., Sutherland, S. M., Algaze, C. A. 2024; 13 (1): 37-45

  Abstract

  Acute kidney injury (AKI) is common after pediatric heart transplantation (HT) and is associated with inferior patient outcomes. Hemodynamic risk factors for pediatric heart transplant recipients who experience AKI are not well described. We performed a retrospective review of 99 pediatric heart transplant patients at Lucile Packard Children's Hospital Stanford from January 1, 2015, to December 31, 2019, in which clinical and demographic characteristics, intraoperative perfusion data, and hemodynamic measurements in the first 48 postoperative hours were analyzed as risk factors for severe AKI (Kidney Disease: Improving Global Outcomes [KDIGO] stage ≥ 2). Univariate analysis was conducted using Fisher's exact test, Chi-square test, and the Wilcoxon rank-sum test, as appropriate. Multivariable analysis was conducted using logistic regression. Thirty-five patients (35%) experienced severe AKI which was associated with lower intraoperative cardiac index ( p  = 0.001), higher hematocrit ( p  < 0.001), lower body temperature ( p  < 0.001), lower renal near-infrared spectroscopy ( p  = 0.001), lower postoperative mean arterial blood pressure (MAP: p  = 0.001), and higher central venous pressure (CVP; p  < 0.001). In multivariable analysis, postoperative CVP >12 mm Hg (odds ratio [OR] = 4.27; 95% confidence interval [CI]: 1.48-12.3, p  = 0.007) and MAP <65 mm Hg (OR = 4.9; 95% CI: 1.07-22.5, p  = 0.04) were associated with early severe AKI. Children with severe AKI experienced longer ventilator, intensive care, and posttransplant hospital days and inferior survival ( p  = 0.01). Lower MAP and higher CVP are associated with severe AKI in pediatric HT recipients. Patients, who experienced AKI, experienced increased intensive care unit (ICU) morbidity and inferior survival. These data may guide the development of perioperative renal protective management strategies to reduce AKI incidence and improve patient outcomes.

  View details for DOI 10.1055/s-0041-1736336

  View details for PubMedID 38571984

  View details for PubMedCentralID PMC10987224

• Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining. World journal for pediatric & congenital heart surgery Mac Felmly, L., Mainwaring, R. D., Ho, D. Y., Arunamata, A., Algaze, C., Hanley, F. L. 2024: 21501351231224329

  Abstract

  BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining.METHODS: This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present).RESULTS: Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation.CONCLUSIONS: The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

  View details for DOI 10.1177/21501351231224329

  View details for PubMedID 38321756

• Using clinical decision support systems to decrease intravenous acetaminophen use: implementation and lessons learned. Applied clinical informatics Tse, G., Algaze, C., Pageler, N., Wood, M., Chadwick, W. 2023

  Abstract

  Clinical decision support systems (CDSS) can enhance medical decision-making by providing targeted information to providers. While they have the potential to improve quality of care and reduce costs, they are not universally effective and can lead to unintended harm.To describe the implementation of an unsuccessful interruptive CDSS that aimed to promote appropriate use of intravenous (IV) acetaminophen at an academic pediatric hospital, with an emphasis on lessons learned.Quality improvement methodology was used to study the effect of an interruptive CDSS, which set a mandatory expiry time of 24-hours for all IV acetaminophen orders. This CDSS was implemented on April 5, 2021. The primary outcome measure was number of IV acetaminophen administrations per 1,000 patient days, measured pre- and post-implementation. Process measures were the number of IV acetaminophen orders placed per 1,000 patient days. Balancing measures were collected via survey data and included provider and nursing acceptability and unintended consequences of the CDSS.There was no special cause variation in hospital wide IV acetaminophen administrations and orders after CDSS implementation, nor when the CDSS was removed. A total of 88 participants completed the survey. Nearly half (40/88) of respondents reported negative issues with the CDSS, with the majority stating that this affected patient care (39/40). Respondents cited delays in patient care and reduced efficiency as the most common negative effects.This study underscores the significance of monitoring CDSS implementations and including end-user acceptability as an outcome measure. Teams should be prepared to modify or remove CDSS that do not achieve their intended goal or are associated with low end-user acceptability. CDSS holds promise for improving clinical practice, but careful implementation and ongoing evaluation are crucial for maximizing their benefits and minimizing potential harm.

  View details for DOI 10.1055/a-2216-5775

  View details for PubMedID 37995743

• ECGs Do Not Detect Myocardial Ischemia in Patients with Williams Syndrome and Non-Syndromic Elastin Arteriopathy with Coronary Artery Stenosis. The American journal of cardiology Algaze, C., Chubb, H., Deitch, A. M., Collins, R. T. 2023

  Abstract

  Coronary artery stenosis (CAS) may affect up to 27% of patients with Williams syndrome (WS), which may lead to myocardial ischemia. WS patients face a 25 to 100-fold higher risk of sudden cardiac death, frequently linked to anesthesia. Assessing CAS requires either imaging while under general anesthesia or intraoperative assessment, with the latter considered the gold standard. Our study aimed to identify electrocardiogram (ECG) markers of myocardial ischemia in patients with WS or non-syndromic elastin arteriopathy and documented CAS. We retrospectively reviewed patients with WS/elastin arteriopathy who underwent supravalvar aortic stenosis (SVAS) surgery and CAS assessment from January 1, 2006, to April 30, 2021. A pediatric electrophysiologist, unaware of the patients' CAS status, reviewed their preoperative ECGs for markers of ischemia. We assessed associations of study parameters using Wilcoxon rank-sum and Fisher's exact tests. Out of 34 patients, 62% were male, with a median age of 20 months [IQR: 8, 34]. Coronary artery stenosis was present in 62% (21/34), 76% (16/21) of whom were male. There were no ECG indicators of myocardial ischemia in patients with CAS. In conclusion, CAS was present in over half of children with WS/elastin arteriopathy who underwent repair of SVAS. Coronary artery stenosis in WS/non-syndromic elastin arteriopathy does not appear to exhibit typical ECG-detectable myocardial ischemia. ECGs are not a useful screening tool for CAS in WS/elastin arteriopathy. Given the high anesthesia-related cardiac arrest risk, other noninvasive indicators of CAS are needed.

  View details for DOI 10.1016/j.amjcard.2023.11.020

  View details for PubMedID 37963512

• Racial and Ethnic Disparities in Cardiac Reintervention after Pediatric Cardiac Surgery. The Annals of thoracic surgery Saha, P., Tjoeng, Y. L., Algaze, C., Kameny, R., Pinto, N., Chan, T. 2023

  Abstract

  BACKGROUND: Children undergoing cardiac surgery may require postoperative surgical or catheter-based reintervention prior to discharge. We examined racial/ethnic variations in reintervention and associated in-hospital mortality.METHODS: Children undergoing cardiac surgery from 2004 to 2015 were identified in the Pediatric Health Information Systems (PHIS) database. Regression analysis measured associations between race/ethnicity, in-hospital mortality, and postoperative cardiac surgical or catheter-based reintervention (Surgery/Catheter Reintervention).RESULTS: Of 124,263 patients, 8,265 (6.7%) had a Surgery/Catheter Reintervention. Black patients had fewer reinterventions (5.9% versus 6.7%) and higher in-hospital mortality (3.9% versus 2.7%, p<0.01) than White patients. After adjusting for sociodemographic and illness severity indicators, Black patients remained less likely to receive Surgery/Catheter Reintervention (adjusted Hazard Ratio [aHR] 0.89, 95% confidence interval [CI] 0.82-0.98) despite having similar risk of death after reintervention (adjusted Odds Ratio [aOR] 1.17, 95% CI 0.98-1.41) compared with White patients. Black and Other race/ethnicity patients also had higher risk of death without Surgery/Catheter Reintervention (aHR 1.26, 95% CI 1.08-1.47 and aHR 1.33, 95% CI 1.13-1.57, respectively) than White patients. Similar trends were demonstrated when mechanical circulatory support and cardiac transplantation were included as reinterventions.CONCLUSIONS: Patients of Black and Other race/ethnicity undergoing pediatric cardiac surgery are more likely to die without postoperative cardiac reintervention than White patients. Black patients are also less likely to receive reintervention despite no significant difference in mortality with reintervention. Further studies should evaluate etiologies and methods of addressing these disparities.

  View details for DOI 10.1016/j.athoracsur.2023.10.027

  View details for PubMedID 37923240

• Intensive Care Unit Analgosedation After Cardiac Surgery in Children with Williams Syndrome : a Matched Case-Control Study. Pediatric cardiology Mills, M., Algaze, C., Journel, C., Suarez, G., Lechich, K., Kwiatkowski, M. D., Schmidt, A. R., Collins, R. T. 2023

  Abstract

  OBJECTIVE: Cardiovascular abnormalities are common in patients with Williams syndrome and frequently require surgical intervention necessitating analgesia and sedation in a population with a unique neuropsychiatric profile, potentially increasing the risk of adverse cardiac events during the perioperative period. Despite this risk, the overall postoperative analgosedative requirements in patients with WS in the cardiac intensive care unit have not yet been investigated. Our primary aim was to examine the analgosedative requirement in patients with WS after cardiac surgery compared to a control group. Our secondary aim was to compare the frequency of major ACE and mortality between the two groups.DESIGN: Matched case-control study.SETTING: Pediatric CICU at a Tertiary Children's Hospital.PATIENTS: Patients with WS and age-matched controls who underwent cardiac surgery and were admitted to the CICU after cardiac surgery between July 2014 and January 2021.INTERVENTIONS: None.MEASUREMENTS AND MAIN RESULTS: Postoperative outcomes and total doses of analgosedative medications were collected in the first six days after surgery for the study groups. Median age was 29.8 (12.4-70.8) months for WS and 23.5 (11.2-42.3) months for controls. Across all study intervals (48h and first 6 postoperative days), there were no differences between groups in total doses of morphine equivalents (5.0mg/kg vs 5.6mg/kg, p=0.7 and 8.2mg/kg vs 10.0mg/kg, p=0.7), midazolam equivalents (1.8mg/kg vs 1.5mg/kg, p=0.4 and 3.4mg/kg vs 3.8mg/kg, p=0.4), or dexmedetomidine (20.5 mcg/kg vs 24.4 mcg/kg, p=0.5 and 42.3 mcg/kg vs 39.1 mcg/kg, p=0.3). There was no difference in frequency of major ACE or mortality.CONCLUSIONS: Patients with WS received similar analgosedative medication doses compared with controls. There was no significant difference in the frequency of major ACE (including cardiac arrest, extracorporeal membrane oxygenation, and surgical re-intervention) or mortality between the two groups, though these findings must be interpreted with caution. Further investigation is necessary to elucidate the adequacy of pain/sedation control, factors that might affect analgosedative needs in this unique population, and the impact on clinical outcomes.

  View details for DOI 10.1007/s00246-023-03321-8

  View details for PubMedID 37882809

• Development of an Equity, Diversity, and Inclusion Committee for a collaborative quality improvement network: Pediatric Cardiac Critical Care Consortium (PC4) Equity, Diversity and Inclusion (EDI) Committee: white paper 2023. Cardiology in the young Tjoeng, Y. L., Werho, D. K., Algaze, C., Nawathe, P., Benjamin, S., Schumacher, K. R., Chan, T. 2023: 1-7

  Abstract

  Racial and ethnic disparities are well described in paediatric cardiac critical care outcomes. However, understanding the mechanisms behind these outcomes and implementing interventions to reduce and eliminate disparities remain a gap in the field of paediatric cardiac critical care. The Pediatric Cardiac Critical Care Consortium (PC4) established the Equity, Diversity, and Inclusion (EDI) Committee in 2020 to promote an equity lens to its aim of improving paediatric cardiac critical care quality and outcomes across North America. The PC4 EDI Committee is working to increase research, quality improvement, and programming efforts to work towards health equity. It also aims to promote health equity considerations in PC4 research. In addition to a focus on patient outcomes and research, the committee aims to increase the inclusion of Black, Indigenous, and People of Color (BIPOC) members in the PC4 collaborative. The following manuscript outlines the development, structure, and aims of the PC4 EDI Committee and describes an analysis of social determinants of health in published PC4 research.

  View details for DOI 10.1017/S1047951123002950

  View details for PubMedID 37577942

• The Leadership/Management Concept Scale: differentiating between actions constituting leadership and management LEADERSHIP & ORGANIZATION DEVELOPMENT JOURNAL Collins II, R., Algaze, C., Posner, B. Z. 2023

  View details for DOI 10.1108/LODJ-06-2022-0299

  View details for Web of Science ID 001041747600001

• Arrhythmias in Williams Syndrome. The American journal of cardiology Deitch, A. M., Giacone, H. M., Chubb, H., Algaze, C. A., Lechich, K. M., Collins, R. T. 2023; 195: 91-97

  Abstract

  Williams syndrome (WS) is a congenital, multisystem disorder in which 80% of patients have cardiovascular abnormalities. Sudden cardiac death occurs 25 to 100 times more often in WS than in the general population, and cardiac repolarization is abnormal in WS. We sought to determine the prevalence of primary arrhythmias in patients with WS and whether QTc prolongation impacts arrhythmia risk. We retrospectively reviewed all patients with WS with ambulatory electrocardiogram (ECG) monitoring at our institution between October 2017 and January 2022. The primary outcome was the presence of arrhythmia. Predictors pre-determined for analysis included premature ventricular and atrial complex burden (%), degree of QTc change with varying heart rates, intervals and rhythm on 12-lead ECG, age, gender, symptomatology, and clinical and surgical history. A total of 74 patients (55% female, median age 8years (3, 13) underwent 108 ambulatory monitors. Arrhythmias were present in 9 patients (12%). Within this group of 9 patients, 18/24 serial monitors were abnormal, and 3/9 patients (33%) had >1 arrhythmia type. Older age (p=0.002) and symptoms (syncope, p=0.005) were associated with arrhythmias. Arrhythmia was not associated with the degree of structural heart disease. Atrial tachycardia was the most identified arrhythmia (n=6; 67% of patients with arrhythmias and 8% of the total cohort). The QTc abnormally increased with higher heart rates in all groups. There was a higher number of premature ventricular and atrial complexes per hour in patients with arrhythmias. In conclusion, atrial arrhythmias were the most common arrhythmia in patients with WS and routine ambulatory ECG and intermittent rhythm monitoring are indicated in WS, particularly given the high risk of sudden cardiac death in WS.

  View details for DOI 10.1016/j.amjcard.2023.03.004

  View details for PubMedID 37037070

• Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses. JTCVS open Felmly, L. M., Mainwaring, R. D., Algaze, C., Martin, E., Ma, M., Hanley, F. L. 2023; 13: 344-356

  Abstract

  Acute lung injury is a known complication of pulmonary artery reconstruction for peripheral pulmonary artery stenosis. Severe cases may require support with extracorporeal membrane oxygenation. The purpose of this study was to evaluate the characteristics of patients requiring extracorporeal membrane oxygenation after pulmonary artery reconstruction.This was a retrospective study of 150 patients who underwent surgical repair of peripheral pulmonary artery stenosis at our institution from 2002 to 2022. Underlying diagnoses included Williams syndrome (n = 44), Alagille syndrome (n = 43), elastin arteriopathy (n = 21), tetralogy of Fallot (n = 21), and other (n = 21). Characteristics of patients who required extracorporeal membrane oxygenation were compared with those who did not require extracorporeal membrane oxygenation.Eleven of the 150 patients undergoing pulmonary artery reconstruction (7.3%) required postoperative extracorporeal membrane oxygenation support (10 for acute lung injury and 1 for cardiac insufficiency). Four patients receiving extracorporeal membrane oxygenation had Williams syndrome, 3 patients had Alagille, and 4 patients had tetralogy of Fallot. Patients requiring extracorporeal membrane oxygenation had a higher preoperative right ventricle to aortic peak systolic pressure ratios (mean 1.14 vs 0.95), greater number of pulmonary artery ostial interventions (median, 23 vs 17), and longer duration of cardiopulmonary bypass (median, 597 vs 400 minutes). There were 3 in-hospital deaths (2.0%), 2 of whom required postoperative extracorporeal membrane oxygenation support.The data demonstrate multiple differences between patients who did and did not require extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenosis. These results suggest that the preoperative extent of disease may predispose to the development of acute lung injury requiring extracorporeal membrane oxygenation support.

  View details for DOI 10.1016/j.xjon.2023.01.011

  View details for PubMedID 37063146

  View details for PubMedCentralID PMC10091391

• Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable? Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Ma, M., Martin, E., Algaze, C., Collins, R. T., McElhinney, D., Mainwaring, R., Hanley, F. 2023; 26: 2-8

  Abstract

  Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results.

  View details for DOI 10.1053/j.pcsu.2022.12.003

  View details for PubMedID 36842795

• Team Communication and Expectations Following Pediatric Cardiac Surgery: A Multi-Disciplinary Survey. Pediatric cardiology Bushnell, J., Connelly, C., Algaze, C. A., Bailly, D. K., Koth, A., Mafla, M., Presnell, L., Shin, A. Y., McCammond, A. N. 2022

  Abstract

  Patients and families desire an accurate understanding of the expected recovery following congenital cardiac surgery. Variation in knowledge and expectations within the care team may be under-recognized and impact communication and care delivery. Our objective was to assess knowledge of common postoperative milestones and perceived efficacy of communication with patients and families and within the care team. An 18-question survey measuring knowledge of expected milestones for recovery after four index operations and team communication in the postoperative period was distributed electronically to multidisciplinary care team members at 16 academic pediatric heart centers. Answers were compared to local median data for each respondent's heart center to assess accuracy and stratified by heart center role and years of experience. We obtained 874 responses with broad representation of disciplines. More than half of all respondent predictions (55.3%) did not match their local median data. Percent matching did not vary by care team role but improved with increasing experience (35.8%<2years vs. 46.4%>10years, p=0.2133). Of all respondents, 62.7% expressed confidence discussing the anticipated postoperative course, 78.6% denoted confidence discussing postoperative complications, and 55.3% conveyed that not all members of their care team share a common expectation for typical postoperative recovery. Most respondents (94.6%) stated that increased knowledge of local data would positively impact communication. Confidence in communication exceeded accuracy in predicting the timing of postoperative milestones. Important variation in knowledge and expectations for postoperative recovery in pediatric cardiac surgery exists and may impact communication and clinical effectiveness.

  View details for DOI 10.1007/s00246-022-03059-9

  View details for PubMedID 36436004

• Impact of Abnormal Potassium on Arrhythmia Risk During Pediatric Digoxin Therapy. Pediatric cardiology Mlambo, V. C., Algaze, C. A., Mak, K., Collins, R. T. 2022

  Abstract

  Digoxin is used in children with heart failure and tachyarrhythmia. Its use in patients with single ventricle anatomy has increased following evidence of improved interstage survival after the Norwood procedure. Digoxin has a narrow therapeutic window and may alter serum potassium balance, inducing arrhythmias. We hypothesized digoxin use in the setting of abnormal serum potassium levels is associated with arrhythmias. We reviewed all patients≤18years who received digoxin while admitted at our institution from 2014 to 2021. Admissions<2 nights were excluded. We compared patients with a hemodynamically significant arrhythmia to those without. We performed adjusted mixed-effects logistic regression with arrhythmia as the outcome variable and potassium status as the predictor variable; adjusting for weight, route of digoxin administration, digoxin indication, serum creatinine, and number of interacting drugs prescribed. Abnormal potassium was defined as serum levels<3.5mmol/L or>6.0mmol/L. There were 268 encounters in 171 patients. Potassium levels were abnormal in 75.5% of patients who experienced an arrhythmia during digoxin administration, compared to 42.6% who did not (p<0.001). Odds of arrhythmia was 138% higher in patients with abnormal potassium receiving digoxin (AOR=2.38, 95% CI 1.07-5.29, p=0.03). Receiving intravenous digoxin was also associated with a 7.35 odds of cardiac arrhythmia (AOR 7.35, p=0.006, 95% CI 1.79-30.26). Odds of arrhythmia is increased during digoxin administration when pediatric patients have abnormal potassium levels. Vigilant attention to potassium levels is essential to prevent adverse outcomes during digoxin therapy.

  View details for DOI 10.1007/s00246-022-03051-3

  View details for PubMedID 36403164

• Criteria for Early Pacemaker Implantation in Patients With Postoperative Heart Block After Congenital Heart Surgery. Circulation. Arrhythmia and electrophysiology Duong, S. Q., Shi, Y., Giacone, H., Navarre, B., Gal, D., Han, B., Sganga, D., Ma, M., Reddy, C. D., Shin, A., Kwiatkowski, D. M., Dubin, A. M., Scheinker, D., Algaze, C. A. 2022: e011145

  Abstract

  Guidelines recommend observation for atrioventricular node recovery until postoperative days (POD) 7 to 10 before permanent pacemaker placement (PPM) in patients with heart block after congenital cardiac surgery. To aid in surgical decision-making for early PPM, we established criteria to identify patients at high risk of requiring PPM.We reviewed all cases of second degree and complete heart block (CHB) on POD 0 from August 2009 through December 2018. A decision tree model was trained to predict the need for PPM amongst patients with persistent CHB and prospectively validated from January 2019 through March 2021. Separate models were developed for all patients on POD 0 and those without recovery by POD 4.Of the 139 patients with postoperative heart block, 68 required PPM. PPM was associated with older age (3.2 versus 1.0 years; P=0.018) and persistent CHB on POD 0 (versus intermittent CHB or second degree heart block; 87% versus 58%; P=0.001). Median days [IQR] to atrioventricular node recovery was 2 [0-5] and PPM was 9 [6-11]. Of the 100 cases of persistent CHB (21 in the validation cohort), 59 (59%) required PPM. A decision tree model identified 4 risk factors for PPM in patients with persistent CHB: (1) aortic valve replacement, subaortic stenosis repair, or Konno procedure; (2) ventricular L-looping; (3) atrioventricular valve replacement; (4) and absence of preoperative antiarrhythmic agent (in POD 0 model only). The POD 4 model specificity was 0.89 [0.67-0.99] and positive predictive value was 0.94 [95% CI 0.81-0.98], which was stable in prospective validation (positive predictive value 1.0).A data-driven analysis led to actionable criteria to identify patients requiring PPM. Patients with left ventricular outflow tract surgery, atrioventricular valve replacement, or ventricular L-Looping could be considered for PPM on POD 4 to reduce risks of temporary pacing and improve care efficiency.

  View details for DOI 10.1161/CIRCEP.122.011145

  View details for PubMedID 36306332

• Importance of Formula-Specific Centile Thresholds for Evaluation of Heart Rate-Corrected QT Prolongation in Williams Syndrome. The American journal of cardiology Algaze, C. A., Deitch, A. M., Chubb, H., Aziz, P. F., Collins, R. T. 2022

  Abstract

  Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia. The ninety-eighth centile for QTc and corrected JT Interval (JTc) of the control population for each formula were used to set the threshold for prolongation. Prevalence comparison was done with Fisher's exact test. Predictors of longer QTc/JTc were assessed using linear regression models adjusting for age, gender, and heart rate. Adjusted odds of QTc/JTc prolongation were evaluated with conditional logistic regression models matched based on age and heart rate. There were 482 electrocardiograms from 188 patients with WS and 1,522 from normal controls. Patients with WS were younger, with higher heart rates and shorter RR and QRS intervals. WS was associated with longer QTc/JTc compared with controls. There were higher odds of prolonged QTc/JTc in patients with WS than controls using both Bazett and Fridericia formulas. In conclusion, this study confirms the higher prevalence of QTc prolongation in WS compared with controls and highlights the importance of setting appropriate formula-specific upper thresholds for QTc prolongation for accurate diagnosis.

  View details for DOI 10.1016/j.amjcard.2022.07.031

  View details for PubMedID 36114024

• Enhanced Recovery After Cleft Palate Repair: A Quality Improvement Project. Paediatric anaesthesia Esfahanian, M., Marcott, S. C., Hopkins, E., Burkart, B., Khosla, R., Lorenz, H. P., Wang, E., De Souza, E., Algaze-Yojay, C., Caruso, T. J. 2022

  Abstract

  BACKGROUND: Children undergoing cleft palate repair present challenges to postoperative management due to several factors that can complicate recovery. Utilization of multimodal analgesic protocols can improve outcomes in this population. We report experience designing and implementing an enhanced recovery after surgery (ERAS) pathway for cleft palate repair to optimize postoperative recovery.AIMS: The primary aim was to implement an ERAS pathway with >70% bundle adherence to achieve a 30% reduction in postoperative opioid consumption within 12 months. Our secondary aims assessed intraoperative opioid consumption, length of stay (LOS), timeliness of oral intake, and respiratory recovery.METHODS: A multidisciplinary team of perioperative providers developed an ERAS pathway for cleft palate patients. Key drivers included patient and provider education, formal pathway creation and implementation, multimodal pain therapy, and target-based care. Interventions included maxillary nerve blockade and enhanced intra- and postoperative medication regimens. Outcomes were displayed as statistical process control charts.RESULTS: Pathway compliance was 77.0%. Patients during the intervention period (n=39) experienced a 49% reduction in postoperative opioid consumption (p<0.0001) relative to our historical cohort (n=63), with a mean difference of -0.33 ±0.11 mg/kg (95% CI -0.55 to -0.12 mg/kg). Intraoperative opioid consumption was reduced by 36% (p=0.002), with a mean difference of -0.27 ±0.09 mg/kg (95% CI -0.45 to -0.09 mg/kg). Additionally, patients in the intervention group had a 45% reduction in time to first oral intake (p=0.02) relative to our historical cohort, with a mean difference of -3.81 ±1.56 hours (95% CI -6.9 to -0.70). There was no difference in PACU or hospital LOS, but there was a significant reduction in variance of all secondary outcomes.CONCLUSION: Opioid reduction and improved timeliness of oral intake is possible with an ERAS protocol for cleft palate repair, but our protocol did not alter PACU or hospital LOS.

  View details for DOI 10.1111/pan.14541

  View details for PubMedID 35929340

• Prevalence and Outcomes of Primary Left Ventricular Dysfunction in Marfan Syndrome. The American journal of cardiology Connor, B. S., Algaze, C. A., Narkeviciute, A., Anguiano, B., Pariani, M., Zarate, Y. A., Collins, R. T. 2022

  Abstract

  Even in the absence of significant valvular disease, patients with Marfan syndrome (MFS) have evidence of impaired left ventricular (LV) performance, suggestive of a primary cardiomyopathy. However, the true prevalence and long-term outcomes of this disease process remain largely unknown. We performed a retrospective analysis of all adult patients with confirmed MFS followed at Stanford Health Care. Those with significant valvular regurgitation, coronary artery disease, or previous cardiac surgery were excluded. LV systolic dysfunction was defined as a LV ejection fraction (LVEF) <55% on transthoracic echocardiography. A total of 753 patients with confirmed MFS were followed up over a median duration of 8 years (interquartile range 4 to 13). Of those, 241 patients (53% women, 71% White) met inclusion criteria and comprised the study cohort. LV systolic dysfunction was present in 30 patients (12%), with a median age of onset of 25 years (interquartile range 19 to 37), median EF of 52% (interquartile range 48 to 54), and evidence of clinical heart failure (New York Heart Association functional class ≥II) in 10% of patients. LV systolic dysfunction was more common in patients with larger aortic root diameters (≥4.0 cm: Odds ratio=4.5, 95% confidence interval=1.2 to 17.1) but was not associated with other cardiovascular manifestations of MFS or traditional atherosclerotic risk factors. In conclusion, apart from significant valvular pathology, LV systolic dysfunction was prevalent in MFS from a young age, suggestive of a primary cardiomyopathy. LV dysfunction was typically mild and subclinical and occurred more commonly in patients with more pronounced aortopathies.

  View details for DOI 10.1016/j.amjcard.2022.04.012

  View details for PubMedID 35550817

• Surgical Repair of Supravalvar Aortic Stenosis in Association With Transverse and Proximal Descending Aortic Abnormalities. World journal for pediatric & congenital heart surgery Mainwaring, R. D., Collins, R. T., Ma, M., Martin, E., Arunamata, A., Algaze-Yojay, C., Hanley, F. L. 2022; 13 (3): 353-360

  Abstract

  BACKGROUND: Supravalvar aortic stenosis (SVAS) may be an isolated defect of the proximal ascending aorta. However, more severe cases have extension of the arteriopathy into the transverse and proximal descending aorta. The purpose of this study was to review our experience with SVAS with and without aortic arch arteriopathy.METHODS: This was a retrospective review of 58 patients who underwent surgical repair of SVAS. The median age at repair was 18 months. A total of 37 patients had Williams syndrome. A total of 31 (53%) patients had associated peripheral pulmonary artery stenosis and 23 (39%) had coronary artery ostial stenosis (CAOS).RESULTS: A total of 37 of 58 (64%) patients had surgical repair of SVAS without the need for arch intervention while 21 (36%) patients had repair of the distal aortic arch. There were 3 (5.2%) operative deaths, 2 of whom had aortic arch involvement and one without arch involvement. There were 2 deaths after discharge from the hospital. Patients who needed arch surgery were more likely to have severe arch gradients compared to those without arch involvement (71% vs 30%, P < .05), were more likely to undergo concomitant procedures for peripheral pulmonary artery stenosis or CAOS (90% vs 62%, P < .05), and to have Williams syndrome (86% vs 51%, P < .05).CONCLUSIONS: More than one-third of patients who had SVAS repair at our institution had procedures directed at the transverse or proximal descending aorta. Patients with arch involvement had more severe arch obstruction, required more concomitant procedures, and were more likely to have Williams syndrome.

  View details for DOI 10.1177/21501351221085975

  View details for PubMedID 35446223

• A clinical scoring system for early onset (neonatal) Marfan syndrome. Genetics in medicine : official journal of the American College of Medical Genetics Zarate, Y. A., Morris, S. A., Blackshare, A., Algaze, C. A., Connor, B. S., Kim, A. J., Yutzey, K. E., Miller, E. M., Weaver, K. N., Collins, R. T. 2022

  Abstract

  PURPOSE: This study aimed to develop objective diagnostic criteria for early onset Marfan syndrome (eoMFS) to facilitate early diagnosis and timely interventions.METHODS: On the basis of an extensive literature review and the responses from a survey distributed among providers with expertise in the diagnosis and management of eoMFS, we developed an age-based, diagnostic scoring system encompassing 10 features common to eoMFS (9 clinical+ 1 laboratory) and divided them into cardiac, systemic, and FBN1 (on the basis of the location of the pathogenic FBN1 variant) scores.RESULTS: In total, 77 individuals with eoMFS (13 newly reported) and 49 individuals diagnosed with classical Marfan syndrome during early childhood were used to validate the criteria. Median cardiac (8 vs 0, P < .001), systemic (11 vs 3, P < .001), FBN1 (5 vs 0, P < .001), and total (23 vs 4, P < .001) scores were significantly higher in individuals with eoMFS than in those without. A proposed clinical score (cardiac+ systemic) cutoff of ≥14 points showed excellent sensitivity (100%), specificity (92%), and reliability (correctly classified= 94%).CONCLUSION: Distinct from classical Marfan syndrome in phenotype and morbidity, eoMFS can be diagnosed clinically using an objective scoring system encompassing the typical physical features and cardiac disease manifestations. Although genetic testing can be suggestive of eoMFS, genetic testing alone is insufficient for diagnosis.

  View details for DOI 10.1016/j.gim.2022.03.016

  View details for PubMedID 35420547

• A clinical scoring system for early onset (neonatal) Marfan syndrome Zarate, Y., Morris, S., Blackshare, A., Algaze, C., Connor, B., Kim, A., Yutzey, K., Miller, E., Weaver, K., Collins, T. ELSEVIER SCIENCE INC. 2022: S353

  View details for DOI 10.1016/j.gim.2022.01.573

  View details for Web of Science ID 000796586200313

• Impact of a clinical pathway on acute kidney injury in patients undergoing heart transplant. Pediatric transplantation Algaze, C. A., Margetson, T. D., Sutherland, S. M., Kwiatkowski, D. M., Maeda, K., Navaratnam, M., Samreth, S. P., Price, E. P., Zook, N. B., Yang, J. K., Hollander, S. A. 2021: e14166

  Abstract

  BACKGROUND: To evaluate the impact of a clinical pathway on the incidence and severity of acute kidney injury in patients undergoing heart transplant.METHODS: This was a 2.5-year retrospective evaluation using 3years of historical controls within a cardiac intensive care unit in an academic children's hospital. Patients undergoing heart transplant between May 27, 2014, and April 5, 2017 (pre-pathway) and May 1, 2017, and November 30, 2019 (pathway) were included. The clinical pathway focused on supporting renal perfusion through hemodynamic management, avoiding or delaying nephrotoxic medications, and providing pharmacoprophylaxis against AKI.RESULTS: There were 57 consecutive patients included. There was an unadjusted 20% reduction in incidence of any acute kidney injury (p=.05) and a 17% reduction in Stage 2/3 acute kidney injury (p=.09). In multivariable adjusted analysis, avoidance of Stage 2/3 acute kidney injury was independently associated with the clinical pathway era (AOR -1.3 [95% CI -2.5 to -0.2]; p=.03), achieving a central venous pressure of or less than 12mmHg (AOR -1.3 [95% CI -2.4 to -0.2]; p=.03) and mean arterial pressure above 60mmHg (AOR -1.6 [95% CI -3.1 to -0.01]; p=.05) in the first 48h post-transplant, and older age at transplant (AOR - 0.2 [95% CI -0.2 to -0.06]; p=.002).CONCLUSIONS: This report describes a renal protection clinical pathway associated with a reduction in perioperative acute kidney injury in patients undergoing heart transplant and highlights the importance of normalizing perioperative central venous pressure and mean arterial blood pressure to support optimal renal perfusion.

  View details for DOI 10.1111/petr.14166

  View details for PubMedID 34727417

• Intraoperative and Postoperative Hemodynamic Predictors of Acute Kidney Injury in Pediatric Heart Transplant Recipients JOURNAL OF PEDIATRIC INTENSIVE CARE Hollander, S. A., Chung, S., Reddy, S., Zook, N., Yang, J., Vella, T., Navaratnam, M., Price, E., Sutherland, S. M., Algaze, C. A. 2021

  View details for DOI 10.1055/s-0041-1736336

  View details for Web of Science ID 000704825900002

• A Fetal Risk Stratification Pathway for Neonatal Aortic Coarctation Reduces Medical Exposure. The Journal of pediatrics Maskatia, S. A., Kwiatkowski, D., Bhombal, S., Davis, A. S., McElhinney, D. B., Tacy, T. A., Algaze, C., Blumenfeld, Y., Quirin, A., Punn, R. 2021

  Abstract

  To test the hypothesis that a fetal stratification pathway will effectively discriminate between infants at different levels of risk for surgical coarctation and reduce unnecessary medicalization.We performed a pre-post non-randomized study in which we prospectively assigned fetuses with prenatal concern for coarctation to one of three risk categories and implemented a clinical pathway for postnatal management. Postnatal clinical outcomes were compared with a historical control group that were not triaged based on the pathway.The study cohort included 109 fetuses, 57 treated along the fetal coarctation pathway, and 52 historical controls. Among mild-risk fetuses, 3% underwent surgical coarctation repair (0% of those without additional heart defects), compared with 27% of moderate-risk and 63% of high-risk. Combined fetal aortic, mitral and isthmus z-score best discriminated which patients underwent surgery, AUC=0.78(0.66,0.91). Patients triaged according to the fetal coarctation pathway had reduced delivery location changes (76% vs 55%, p=0.025), and umbilical venous catheter placement (74% vs 51%, p=0.046) compared with historical controls. Trends towards shorter intensive care unit stay, hospital stay and time to enteral feeding did not reach statistical significance.A stratified risk-assignment pathway effectively identifies a group of fetuses with low rate of surgical coarctation, and reduces unnecessary medicalization in infants who do not undergo aortic surgery. Incorporation of novel measurements or imaging techniques may improve specificity of high-risk criteria.

  View details for DOI 10.1016/j.jpeds.2021.06.047

  View details for PubMedID 34181988

• Target Based Care: An Intervention to Reduce Variation in Postoperative Length of Stay. The Journal of pediatrics Shin, A. Y., Rao, I. J., Bassett, H. K., Chadwick, W., Kim, J., Kipps, A. K., Komra, K., Loh, L., Maeda, K., Mafla, M., Presnell, L., Sharek, P. J., Steffen, K. M., Scheinker, D., Algaze, C. A. 2020

  Abstract

  OBJECTIVES: To derive care targets and evaluate the impact of displaying them at the point of care on postoperative length of stay (LOS).STUDY DESIGN: A prospective cohort study using 2 years of historical controls within a freestanding, academic children's hospital. Patients undergoing benchmark cardiac surgery between May 4, 2014 and August 15, 2016 (preintervention) and September 6, 2016 to September 30, 2018 (postintervention) were included. The intervention consisted of displaying at the point of care targets for the timing of extubation, transfer from the intensive care unit (ICU), and hospital discharge. Family satisfaction, reintubation, and readmission rates were tracked.RESULTS: The postintervention cohort consisted of 219 consecutive patients. There was a reduction in variation for ICU (difference in SD -2.56, p < 0.01), and total LOS (difference in SD -2.84, P < .001). Patients stayed on average 0.97 fewer days (p<0.001) in the ICU (median -1.01 [IQR -2.15,-0.39], 0.7 fewer days (p<0.001) on mechanical ventilation (median -0.54 [IQR -0.77,-0.50], and 1.18 fewer days (p<0.001) for the total LOS (median -2.25 [IQR -3.69,-0.15]. Log transformed multivariable linear regression demonstrated the intervention to be associated with shorter ICU LOS (beta coefficient -0.19, SE 0.059, p<0.001), total postoperative LOS (beta coefficient -0.12, SE 0.052, p=0.02), and ventilator duration (beta coefficient -0.21, SE 0.048, p<0.001). Balancing metrics did not differ after the intervention.CONCLUSIONS: Target based care is a simple, novel intervention associated with reduced variation in LOS and absolute LOS across a diverse spectrum of complex cardiac surgeries.

  View details for DOI 10.1016/j.jpeds.2020.09.017

  View details for PubMedID 32920104

• 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

• Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot. Journal of the American Heart Association Adamson, G. T., McElhinney, D. B., Zhang, Y. n., Feinstein, J. A., Peng, L. F., Ma, M. n., Algaze, C. A., Hanley, F. L., Perry, S. B. 2020: e017981

  Abstract

  Background Due in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs. Methods and Results All infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross-sectional area were less likely to undergo complete repair. Conclusions MAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross-sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.

  View details for DOI 10.1161/JAHA.120.017981

  View details for PubMedID 33283588

• Younger age remains a risk factor for prolonged length of stay after bidirectional cavopulmonary anastomosis CARDIOLOGY IN THE YOUNG Koth, A. M., Algaze, C. A., Sakarovitch, C., Long, J., Kamra, K., Wright, G. E., Alexander-Banys, B., Maeda, K., Shin, A. Y. 2019; 29 (3): 369–74

  View details for DOI 10.1017/S1047951118002470

  View details for Web of Science ID 000463109300017

• Younger age remains a risk factor for prolonged length of stay after bidirectional cavopulmonary anastomosis. Cardiology in the young Koth, A. M., Algaze, C. A., Sakarovitch, C., Long, J., Kamra, K., Wright, G. E., Alexander-Banys, B., Maeda, K., Shin, A. Y. 2019: 1–6

  Abstract

  OBJECTIVE: This study sets out to determine the influence of age at the time of surgery as a risk factor for post-operative length of stay after bidirectional cavopulmonary anastomosis.METHODS: All patients undergoing a Glenn procedure between January 2010 and July 2015 were included in this retrospective cohort study. Demographic data were examined. Standard descriptive statistics was used. A univariable analysis was conducted using the appropriate test based on data distribution. A propensity score for balancing the group difference was included in the multi-variable analysis, which was then completed using predictors from the univariable analysis that achieved significance of p<0.1.RESULTS: Over the study period, 50 patients met the inclusion criteria. Patients were separated into two cohorts of ⩾4 months (28 patients) and <4 months (22 patients). Other than height and weight, the two cohorts were indistinguishable in their pre-operative saturation, medications, catheterisation haemodynamics, atrioventricular valve regurgitation, and ventricular function. After adjusting group differences, younger age was associated with longer post-operative length of hospitalisation - adjusted mean 15 (±2.53) versus 8 (±2.15) days (p=0.03). In a multi-variable regression analysis, in addition to ventricular dysfunction (beta coefficient=8.8, p=0.05), Glenn procedures performed before 4 months were independently associated with longer length of stay (beta coefficient=-6.9, p=0.03).CONCLUSION: We found that Glenn procedures performed after 4 months of age had shorter post-operative length of stay when compared to a younger cohort. These findings suggest that balancing timing of surgery to decrease the inter-stage period should take into consideration differences in post-operative recovery with earlier operations.

  View details for PubMedID 30698131

• LEVERAGING AGGREGATE DATA AT THE POINT OF CARE REDUCES VARIATION FOR PEDIATRIC NEUROSURGERY PATIENTS Steffen, K., Su, F., Algaze, C., Duethman, L., Jacobs, K., Casazza, M., Chantra, J., Loh, L., Shin, A., Grant, G. LIPPINCOTT WILLIAMS & WILKINS. 2019

  View details for Web of Science ID 000498593401642

• Applying Lessons from an Inaugural Clinical Pathway to Establish a Clinical Effectiveness Program. Pediatric quality & safety Algaze, C. A., Shin, A. Y., Nather, C., Elgin, K. H., Ramamoorthy, C., Kamra, K., Kipps, A. K., Yarlagadda, V. V., Mafla, M. M., Vashist, T., Krawczeski, C. D., Sharek, P. J. 2018; 3 (6): e115

  Abstract

  Introduction: Clinical effectiveness (CE) programs promote standardization to reduce unnecessary variation and improve healthcare value. Best practices for successful and sustainable CE programs remain in question. We developed and implemented our inaugural clinical pathway with the aim of incorporating lessons learned in the build of a CE program at our academic children's hospital.Methods: The Lucile Packard Children's Hospital Stanford Heart Center and Center for Quality and Clinical Effectiveness partnered to develop and implement an inaugural clinical pathway. Project phases included team assembly, pathway development, implementation, monitoring and evaluation, and improvement. We ascertained Critical CE program elements by focus group discussion among a multidisciplinary panel of experts and key affected groups. Pre and postintervention compared outcomes included mechanical ventilation duration, cardiovascular intensive care unit, and total postoperative length of stay.Results: Twenty-seven of the 30 enrolled patients (90%) completed the pathway. There was a reduction in ventilator days (mean 1.0+0.5 versus 1.9+1.3 days; P < 0.001), cardiovascular intensive care unit (mean 2.3+1.1 versus 4.6+2.1 days; P < 0.001) and postoperative length of stay (mean 5.9+1.6 versus 7.9+2.7 days; P < 0.001) compared with the preintervention period. Elements deemed critical included (1) project prioritization for maximal return on investment; (2) multidisciplinary involvement; (3) pathway focus on best practices, critical outcomes, and rate-limiting steps; (4) active and flexible implementation; and (5) continuous data-driven and transparent pathway iteration.Conclusions: We identified multiple elements of successful pathway implementation, that we believe to be critical foundational elements of our CE program.

  View details for DOI 10.1097/pq9.0000000000000115

  View details for PubMedID 31334447

• Diagnostic errors in paediatric cardiac intensive care CARDIOLOGY IN THE YOUNG Bhat, P. N., Costello, J. M., Aiyagari, R., Sharek, P. J., Algaze, C. A., Mazwi, M. L., Roth, S. J., Shin, A. Y. 2018; 28 (5): 675–82

  Abstract

  IntroductionDiagnostic errors cause significant patient harm and increase costs. Data characterising such errors in the paediatric cardiac intensive care population are limited. We sought to understand the perceived frequency and types of diagnostic errors in the paediatric cardiac ICU.Paediatric cardiac ICU practitioners including attending and trainee physicians, nurse practitioners, physician assistants, and registered nurses at three North American tertiary cardiac centres were surveyed between October 2014 and January 2015.The response rate was 46% (N=200). Most respondents (81%) perceived that diagnostic errors harm patients more than five times per year. More than half (65%) reported that errors permanently harm patients, and up to 18% perceived that diagnostic errors contributed to death or severe permanent harm more than five times per year. Medication side effects and psychiatric conditions were thought to be most commonly misdiagnosed. Physician groups also ranked pulmonary overcirculation and viral illness to be commonly misdiagnosed as bacterial illness. Inadequate care coordination, data assessment, and high clinician workload were cited as contributory factors. Delayed diagnostic studies and interventions related to the severity of the patient's condition were thought to be the most commonly reported process breakdowns. All surveyed groups ranked improving teamwork and feedback pathways as strategies to explore for preventing future diagnostic errors.Paediatric cardiac intensive care practitioners perceive that diagnostic errors causing permanent harm are common and associated more with systematic and process breakdowns than with cognitive limitations.

  View details for PubMedID 29409553

• Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bauser-Heaton, H., Borquez, A., Asija, R., Wise-Faberowski, L., Zhang, Y., Downey, L., Perry, S. B., Koth, A., Peng, L. F., Algaze, C. A., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1696–1707

  Abstract

  Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.

  View details for PubMedID 29352588

• Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients CIRCULATION-CARDIOVASCULAR INTERVENTIONS Bauser-Heaton, H., Borquez, A., Han, B., Ladd, M., Asija, R., Downey, L., Koth, A., Algaze, C. A., Wise-Faberowski, L., Perry, S. B., Shin, A., Peng, L. F., Hanley, F. L., McElhinney, D. B. 2017; 10 (4)

  Abstract

  Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

  View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

  View details for PubMedID 28356265

• INSURANCE TYPE IS ASSOCIATED WITH LONGER HOSPITAL DURATION BUT SIMILAR MORTALITY AMONG CHILDREN UNDERGOING CONGENITAL HEART SURGERY Ryan, K., Algaze, C., Sakarovitch, C., Do, T., Yarlagadda, V., Fernandes, S., McElhinney, D., Roth, S., Krawczeski, C., Shin, A. ELSEVIER SCIENCE INC. 2017: 569

  View details for Web of Science ID 000397342301091

• Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass PEDIATRIC CRITICAL CARE MEDICINE Algaze, C. A., Koth, A. M., Faberowski, L. W., Hanley, F. L., Krawczeski, C. D., Axelrod, D. M. 2017; 18 (1): 34-43

  Abstract

  To describe the prevalence and risk factors for acute kidney injury in patients undergoing the extracardiac Fontan operation with and without cardiopulmonary bypass, and to determine whether acute kidney injury is associated with duration of mechanical ventilation, cardiovascular ICU and hospital postoperative length of stay, and early mortality.Single-center retrospective cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Patients with a preoperative creatinine before undergoing first-time extracardiac Fontan between January 1, 2004, and April 30, 2012.None.Acute kidney injury occurred in 55 of 138 patients (39.9%), including 41 (29.7%) with stage 1, six (4.4%) with stage 2, and eight (5.8%) with stage 3 acute kidney injury. Cardiopulmonary bypass was strongly associated with a higher risk of any acute kidney injury (adjusted odds ratio, 4.8 [95% CI, 1.4-16.0]; p = 0.01) but not stage 2/3 acute kidney injury. Lower renal perfusion pressure on the day of surgery (postoperative day, 0) was associated with a higher risk of stage 2/3 acute kidney injury (adjusted odds ratio, 1.2 [95% CI, 1.0-1.5]; p = 0.03). Higher vasoactive-inotropic score on postoperative day 0 was associated with a higher risk for stage 2/3 acute kidney injury (adjusted odds ratio, 1.9 [95% CI, 1.0-3.4]; p = 0.04). Stage 2/3 acute kidney injury was associated with longer cardiovascular ICU length of stay (mean, 7.3 greater d [95% CI, 3.4-11.3]; p < 0.001) and hospital postoperative length of stay (mean, 6.4 greater d [95% CI, 0.06-12.5]; p = 0.04).Postoperative acute kidney injury in patients undergoing the extracardiac Fontan operation is common and is associated with lower postoperative renal perfusion pressure and higher vasoactive-inotropic score. Cardiopulmonary bypass was strongly associated with any acute kidney injury, although not stage 2/3 acute kidney injury. Stage 2/3 acute kidney injury is a compelling risk factor for longer cardiovascular ICU and hospital postoperative length of stay. Increased attention to and management of renal perfusion pressure may reduce postoperative acute kidney injury and improve outcomes.

  View details for DOI 10.1097/PCC.0000000000000984

  View details for Web of Science ID 000392249300005

• Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass. Pediatric critical care medicine Algaze, C. A., Koth, A. M., Faberowski, L. W., Hanley, F. L., Krawczeski, C. D., Axelrod, D. M. 2016: -?

  Abstract

  To describe the prevalence and risk factors for acute kidney injury in patients undergoing the extracardiac Fontan operation with and without cardiopulmonary bypass, and to determine whether acute kidney injury is associated with duration of mechanical ventilation, cardiovascular ICU and hospital postoperative length of stay, and early mortality.Single-center retrospective cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Patients with a preoperative creatinine before undergoing first-time extracardiac Fontan between January 1, 2004, and April 30, 2012.None.Acute kidney injury occurred in 55 of 138 patients (39.9%), including 41 (29.7%) with stage 1, six (4.4%) with stage 2, and eight (5.8%) with stage 3 acute kidney injury. Cardiopulmonary bypass was strongly associated with a higher risk of any acute kidney injury (adjusted odds ratio, 4.8 [95% CI, 1.4-16.0]; p = 0.01) but not stage 2/3 acute kidney injury. Lower renal perfusion pressure on the day of surgery (postoperative day, 0) was associated with a higher risk of stage 2/3 acute kidney injury (adjusted odds ratio, 1.2 [95% CI, 1.0-1.5]; p = 0.03). Higher vasoactive-inotropic score on postoperative day 0 was associated with a higher risk for stage 2/3 acute kidney injury (adjusted odds ratio, 1.9 [95% CI, 1.0-3.4]; p = 0.04). Stage 2/3 acute kidney injury was associated with longer cardiovascular ICU length of stay (mean, 7.3 greater d [95% CI, 3.4-11.3]; p < 0.001) and hospital postoperative length of stay (mean, 6.4 greater d [95% CI, 0.06-12.5]; p = 0.04).Postoperative acute kidney injury in patients undergoing the extracardiac Fontan operation is common and is associated with lower postoperative renal perfusion pressure and higher vasoactive-inotropic score. Cardiopulmonary bypass was strongly associated with any acute kidney injury, although not stage 2/3 acute kidney injury. Stage 2/3 acute kidney injury is a compelling risk factor for longer cardiovascular ICU and hospital postoperative length of stay. Increased attention to and management of renal perfusion pressure may reduce postoperative acute kidney injury and improve outcomes.

  View details for PubMedID 27792123

• Use of a Checklist and Clinical Decision Support Tool Reduces Laboratory Use and Improves Cost. Pediatrics Algaze, C. A., Wood, M., Pageler, N. M., Sharek, P. J., Longhurst, C. A., Shin, A. Y. 2016; 137 (1): 1-8

  Abstract

  We hypothesized that a daily rounding checklist and a computerized order entry (CPOE) rule that limited the scheduling of complete blood cell counts and chemistry and coagulation panels to a 24-hour interval would reduce laboratory utilization and associated costs.We performed a retrospective analysis of these initiatives in a pediatric cardiovascular ICU (CVICU) that included all patients with congenital or acquired heart disease admitted to the cardiovascular ICU from September 1, 2008, until April 1, 2011. Our primary outcomes were the number of laboratory orders and cost of laboratory orders. Our secondary outcomes were mortality and CVICU and hospital length of stay.We found a reduction in laboratory utilization frequency in the checklist intervention period and additional reduction in the CPOE intervention period [complete blood count: 31% and 44% (P < .0001); comprehensive chemistry panel: 48% and 72% (P < .0001); coagulation panel: 26% and 55% (P < .0001); point of care blood gas: 43% and 44% (P < .0001)] compared with the preintervention period. Projected yearly cost reduction was $717, 538.8. There was no change in adjusted mortality rate (odds ratio 1.1, 95% confidence interval 0.7-1.9, P = .65). CVICU and total length of stay (days) was similar in the pre- and postintervention periods.Use of a daily checklist and CPOE rule reduced laboratory resource utilization and cost without adversely affecting adjusted mortality or length of stay. CPOE has the potential to hardwire resource management interventions to augment and sustain the daily checklist.

  View details for DOI 10.1542/peds.2014-3019

  View details for PubMedID 26681782

• Expanding the Phenotype of Cardiovascular Malformations in Adams-Oliver Syndrome AMERICAN JOURNAL OF MEDICAL GENETICS PART A Algaze, C., Esplin, E. D., Lowenthal, A., Hudgins, L., Tacy, T. A., Tierney, E. S. 2013; 161A (6): 1386-1389

  Abstract

  We describe a newborn with a phenotype consistent with Adams-Oliver syndrome and truncus arteriosus. Although cardiovascular malformations associated with this syndrome have been previously published in the literature, this is the first description of truncus arteriosus in a patient with Adams-Oliver syndrome. We review other reports of Adams-Oliver syndrome previously described with cardiovascular malformations, consider possible genetic and embryologic mechanisms, and emphasize the need for cardiology consultation when a diagnosis of Adams-Oliver syndrome is suspected in the differential diagnosis. © 2013 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ajmg.a.35864

  View details for Web of Science ID 000320649700021

• EXTRACARDIAC CONDUIT FONTAN PROCEDURE WITHOUT THE USE OF CARDIOPULMONARY BYPASS: EARLY OUTCOMES 62nd Annual Scientific Session of the American-College-of-Cardiology Algaze, C. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E503–E503

  View details for Web of Science ID 000316555200503

• MAJOR AORTOPULMONARY COLLATERAL ARTERY NOMENCLATURE 62nd Annual Scientific Session of the American-College-of-Cardiology Algaze, C. A., Peng, L. F., Feinstein, J. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E543–E543

  View details for Web of Science ID 000316555200543