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Introduction: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes., Methods: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves., Results: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64., Conclusion: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.

Background: Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation., Methods: A retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children's hospital was performed., Results: A total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995-2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05)., Conclusion: We report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

Background: Risk assessment in heart transplantation is critical for candidate selection, but current models inadequately assess individual risk of postoperative mortality. We sought to identify risk factors and develop a scoring system to predict mortality after heart transplantation in children., Methods: The records of patients undergoing heart transplantation at our institution from 2010 through 2016 were reviewed. Clinical characteristics were recorded and compared between survivors and nonsurvivors. We used Cox proportional hazard modeling of factors associated with postoperative mortality to develop a risk factor score., Results: There were 74 patients who underwent heart transplantation at a mean age of 8.8 ± 6.6 years. Congenital heart disease was the most common indication, comprising 48.6% of the cohort. Overall mortality was 18.9%, with 10 of 14 dying within 30 days of the operation or during the initial postoperative admission (early mortality). Preoperative factors associated with overall mortality were single-ventricle congenital heart disease (hazard ratio [HR], 3.2; p = 0.042), biventricular assist device (HR, 4.8; p = 0.043), history of four or more sternotomies (HR, 3.9; p = 0.023), panel reactive antibody exceeding 10% (HR, 4.4; p = 0.013), any previous operation at another institution (HR, 3.2; p = 0.038), and pulmonary vein disease (HR, 4.7; p = 0.045). Each risk factor was assigned a point value, based on similar magnitude of the HRs. A score of 4 or higher predicted mortality with 57% sensitivity and 90% specificity., Conclusions: In this single-center pediatric cohort, postheart transplantation mortality could be predicted using patient-specific risk factors. The cumulative effect of these risk factors predicted mortality with high specificity., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Infants with Congenital Heart Disease (CHD) often exhibit growth failure. This can affect anthropometric and neurodevelopmental outcomes well into childhood. To determine the resting energy expenditure (REE), body composition, and growth in infants with CHD at 3 months of age, with the secondary aim to identify predictors of REE as compared with healthy infants., Design and Methods: This descriptive study is a subanalysis of a prospective study investigating predictors of growth in postoperative infants with CHD compared with healthy infants. Growth measurements, REE, and body composition were obtained in all infants. Analysis included chi-square for association between categorical variables, t-tests, ANOVA and ANCOVA. Outcome measures included the REE as determined by indirect calorimetry, anthropometric z-scores and body composition at 3 months of age., Setting: Participants were recruited from the Cardiac Intensive Care Unit of a large, urban, pediatric cardiac center and pediatric primary care practices., Results: The analysis included 93 infants, 44 (47%) with CHD. Of the infants with CHD, 39% had single ventricle (SV) physiology. There was no difference in REE related to cardiac physiology between infants with CHD and healthy infants or between infants with SV and biventricular (BV) physiology. Anthropometric z-scores for weight (-1.1 ± 1.1, P < 0.001), length (-0.7 ± 1.1, P < 0.05), and head circumference (-0.6 ± 1.2, P < 0.001) were lower in infants with CHD at 3 months of age. The percentage of body fat (%FAT) in postoperative infants with SV (24% ± 6, P = 0.02) and BV (23% ± 5, P < 0.001) physiology were lower than in healthy infants (27% ± 5), with no difference in REE., Conclusion: At 3 months of age, there was no difference in REE between postsurgical infants with CHD and healthy infants. Infants with CHD had lower growth z-scores and %FAT. These data demonstrate decreased %FAT contributed to growth failure in the infants with CHD., (© 2013 Wiley Periodicals, Inc.)

Objective: To determine the incidence, risk factors, and outcomes after early, unplanned intervention on systemic-to-pulmonary artery shunts in neonates., Methods: We retrospectively studied all neonates undergoing systemic-to-pulmonary artery shunt placement at The Children's Hospital of Philadelphia between September 1, 2002, and May 1, 2005. Patients requiring transcatheter or surgical systemic-to-pulmonary artery shunt intervention before discharge were compared with those not undergoing shunt intervention., Results: A total of 206 patients underwent shunt placement. Diagnoses included hypoplastic left heart syndrome (62.1%), pulmonary atresia (15%), tricuspid atresia (4.9%), tetralogy of Fallot (2.4%), and other lesions with obstruction to systemic (10.7%) or pulmonary blood flow (4.9%). Twenty-one interventions occurred in 20 patients (9.7%). Risk factors for intervention included heterotaxy syndrome (P = .04), congenital abnormality (P = .04), and a trend toward lower birthweight. In patients with a modified Blalock-Taussig shunt, similar risk factors were identified and the incidence of intervention decreased with increasing shunt size. In-hospital mortality was 30% (6/20) for the cases and 8.1% (15/186) for the nonintervention group (P = .02). Long-term survival was significantly lower in patients requiring intervention (P = .002). This group also had a higher incidence of infections (P < .001) and extracorporeal membrane oxygenation (P < .001), and longer hospital stay (P = .001)., Conclusions: In neonates undergoing systemic-to-pulmonary artery shunt placement, approximately 10% underwent shunt intervention before discharge. Some factors, such as low birthweight, shunt size, noncardiac congenital abnormalities, and heterotaxy syndrome, may help identify patients at risk. Patients undergoing intervention experienced increased morbidity and mortality., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: To determine the effect of reintervention for coarctation after stage 1 reconstruction for hypoplastic left heart syndrome and variants on survival, suitability for Fontan, and morbidity at Fontan., Methods: A retrospective review of echocardiograms, catheterizations, hospital records of patients who underwent stage 1 reconstruction from January 2002 to May 2005, with a cross-sectional analysis of hospital survivors, was performed. Kaplan-Meier curves were derived for patients alive more than 30 days after stage 1 reconstruction., Results: A total of 176 patients underwent stage 1 reconstruction. Forty-three patients (23%) underwent balloon angioplasty (n = 43) or surgical intervention (n = 4) for re-coarctation. Median time to intervention was 123 (1-316) days. Seven of 43 patients (16%) underwent more than 1 balloon angioplasty. Thirty-nine patients underwent intervention before stage 2 reconstruction, and 4 patients had intervention between stage 2 reconstruction and Fontan. Kaplan-Meier curves showed no difference in freedom from death or transplant between patients who did and did not undergo intervention for re-coarctation. Fontan completion was performed in 107 patients. By echocardiogram, the prevalence of moderate to severe ventricular dysfunction between groups was similar at Fontan; however, significant atrioventricular valve regurgitation was more common in patients who required intervention (28/33 vs 40/65, P = .02). Overall Fontan mortality was 2% and not different between groups. Length of stay was not different between patients with and without re-coarctation., Conclusions: Reintervention for coarctation after stage 1 reconstruction is common. Hemodynamic differences between groups did not affect Fontan completion, mortality, or hospital length of stay. Follow-up is necessary to determine the impact of re-coarctation on longer-term mortality and morbidity., (2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: We previously reported no difference in morbidity or mortality in a cohort of infants undergoing stage 1 and 2 reconstructions for hypoplastic left heart syndrome with either a modified Blalock-Taussig shunt or a right ventricular to pulmonary artery conduit. This article compares the hemodynamics and perioperative course at the time of the Fontan completion and reports longer-term survival for this cohort., Methods: We retrospectively reviewed the hospital records of all patients who underwent stage 1 reconstruction between January 2002 and May 2005 and subsequent surgical procedures, as well as cross-sectional analysis of hospital survivors., Results: A total of 176 patients with hypoplastic left heart syndrome or a variant underwent stage 1 reconstruction with either modified Blalock-Taussig shunt (n = 114) or right ventricular to pulmonary artery conduit (n = 62). Shunt selection was at the discretion of the surgeon. The median duration of follow-up was 58 months (range 1-87 months). By Kaplan-Meier analysis, shunt type did not influence survival or freedom from transplant at 5 years (right ventricular to pulmonary artery conduit 61%; 95% confidence limit, 47-72 vs modified Blalock-Taussig shunt 70%; 95% confidence limit, 60-77; P = .55). A total of 107 patients underwent Fontan (69 modified Blalock-Taussig shunts and 38 right ventricular to pulmonary artery conduits) with 98% (105/107) early survival. Patients with a right ventricular to pulmonary artery conduit shunt pre-Fontan had higher pulmonary artery (13 +/- 8 mm Hg vs 11 +/- 3 mm Hg, P = .026) and common atrial (8 +/- 2.3 mm Hg vs 6.8 +/- 2.7 mm Hg, P = .039) pressures. By echocardiography evaluation, there was more qualitative moderate to severe ventricular dysfunction (right ventricular to pulmonary artery conduit 31% [12/36] vs modified Blalock-Taussig shunt 17% [11/67], P = .05) and moderate to severe atrioventricular valve regurgitation (right ventricular to pulmonary artery conduit 40% [14/35] vs modified Blalock-Taussig shunt 16% [11/67], P = .01) in the right ventricular to pulmonary artery conduit group. Use of diuretic therapy, angiotensin-converting enzyme inhibition, reflux medications, and tube feedings were not different between groups. Overall, 5 patients underwent heart transplantation (right ventricular to pulmonary artery conduit 4 vs modified Blalock-Taussig shunt 1, P = .1) before Fontan. There was no difference in age or weight at Fontan, bypass time, intensive care unit or hospital length of stay, postoperative pleural effusions, or need for reoperation between groups., Conclusions: Interim analyses continue to suggest there is no survival advantage of one shunt type compared with the other. Longer-term follow-up of a randomized patient population remains of utmost importance., (2010. Published by Mosby, Inc.)

Objective: To define the modes of presentation, incidence of major organ dysfunction, predictors of hospital mortality, and adverse outcomes in neonates with critical heart disease admitted to a tertiary care center., Design: Retrospective chart review., Setting: A tertiary care pediatric cardiac intensive care unit and neonatal intensive care unit., Patients: The medical records for all neonates (< or = 30 days of age) with heart disease admitted to the cardiac intensive care unit or neonatal intensive care unit between October 1, 2002, and September 30, 2003, were reviewed., Interventions: None., Measurements and Main Results: A total of 190 neonates met inclusion criteria during this 1-yr period, of which 146 (77%) had at least one surgical procedure. Single ventricle heart disease was present in 42%. The most common mode of presentation was following a prenatal diagnosis (53%), followed by diagnosis in the newborn nursery (38%) and diagnosis after newborn hospital discharge (8%). The most common presenting findings in the newborn nursery were isolated murmur (38%) or cyanosis (32%), while circulatory collapse (38%) was the most common presentation after discharge. For the entire study cohort, 13% had a known genetic syndrome, 23% had a major noncardiac congenital anomaly, and 16% weighed < 2.5 kg. The hospital mortality for the entire cohort was 7.4%. Risk factors associated with an increased risk of hospital mortality included younger age at admission, higher number of cardiopulmonary bypass runs, and need for postoperative cardiopulmonary resuscitation. Total hospital length of stay was > 1 month in 17% of neonates., Conclusions: In patients with complex congenital heart disease, including nearly half with single ventricle heart disease, neonatal hospital mortality was 7%. These patients have a high frequency of multiple congenital anomalies, genetic syndromes, low birth weight, and prolonged length of stay.