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The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

The role of extracorporeal membrane oxygenation (ECMO) in the management of severely ill patients with coronavirus disease 2019 (COVID-19) continues to evolve. The purpose of this study is to review a multi-institutional clinical experience in 100 consecutive patients, at 20 hospitals, with confirmed COVID-19 supported with ECMO. This analysis includes our first 100 patients with complete data who had confirmed COVID-19 and were supported with ECMO. The first patient in the cohort was placed on ECMO on March 17, 2020. Differences by the mortality group were assessed using χ2 tests for categorical variables and Kruskal-Wallis rank-sum tests and Welch's analysis of variance for continuous variables. The median time on ECMO was 12.0 days (IQR = 8-22 days). All 100 patients have since been separated from ECMO: 50 patients survived and 50 patients died. The rate of survival with veno-venous ECMO was 49 of 96 patients (51%), whereas that with veno-arterial ECMO was 1 of 4 patients (25%). Of 50 survivors, 49 have been discharged from the hospital and 1 remains hospitalized at the ECMO-providing hospital. Survivors were generally younger, with a lower median age (47 versus 56.5 years, p = 0.014). In the 50 surviving patients, adjunctive therapies while on ECMO included intravenous steroids (26), anti-interleukin-6 receptor blockers (26), convalescent plasma (22), remdesivir (21), hydroxychloroquine (20), and prostaglandin (15). Extracorporeal membrane oxygenation may facilitate salvage and survival of selected critically ill patients with COVID-19. Survivors tend to be younger. Substantial variation exists in the drug treatment of COVID-19, but ECMO offers a reasonable rescue strategy.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10–15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre. Our hypothesis was that patients with pulmonic valve autograft would have better survival than mechanical prosthetic.MethodsThis is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional US-based registry of paediatric cardiac interventions, linked with the National Death Index and United Network for Organ Sharing through 2019. Children (0–20 years old) receiving aortic valve replacement (AVR) from 1982 to 2003 were identified. Kaplan-Meier transplant-free survival was calculated, and Cox proportional hazard models estimated hazard ratios for mechanical AVR (M-AVR) versus pulmonic valve autograft.ResultsAmong 911 children, the median age at AVR was 13.4 years (IQR=8.4–16.5) and 73% were male. There were 10 cardiac transplants and 153 deaths, 5 after transplant. The 25-year transplant-free survival post AVR was 87.1% for autograft vs 76.2% for M-AVR and 72.0% for tissue (bioprosthetic or homograft). After adjustment, M-AVR remained related to increased mortality/transplant versus autograft (HR=1.9, 95% CI=1.1 to 3.4). Surprisingly, survival for patients with M-AVR, but not autograft, was lower for those treated in centres with higher in-hospital mortality.ConclusionPulmonic valve autograft provides the best long-term outcomes for children with aortic valve disease, but AVR results may depend on a centre’s experience or patient selection.

Background The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model adjusts not only for procedure and age group pairings but also for additional patient factors, including the binary presence or absence of a chromosomal abnormality (CA), syndrome (S), and/or a noncardiac congenital anatomic abnormality (NCAA). This analysis refines case-mix adjustment by adding more granular adjustment for individual conditions (CA, S, and NCAA), consistent with a hypothesis that associated risk of mortality differs between individual conditions. Methods CA/S corresponding to the same condition were merged to a single condition code. Odds ratios were estimated for all CA/S. For CA/S associated with at least 10 deaths in neonates and infants and at least 10 deaths in children and adults, odds ratios were estimated for the effect of the CA/S separately in neonates/infants and in children/adults. In addition to these condition/age interactions, condition/age/procedure interactions were explored (eg, effect of Down syndrome was estimated based on age and procedure subgroups, including atrioventricular canal repair and single-ventricle palliation). Bayesian modeling was used to create 5 maximally homogeneous groups of CA/S from 81 candidate CA/S variables. A standard logistic regression model then incorporated indicator variables for the 5 categories of CAs/Ss, 7 unique NCAAs, and all other covariates in the previously published Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Model. Results Analysis included 107,062 operations in 100 centers (2010 to 2015). Operative Mortality was 3,629 (3.4%). In the development sample, the C statistics of the original nonaugmented model and the augmented model were 0.872 and 0.875, respectively. Conclusions The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model has been augmented by addition of covariates representing individual CAs, Ss, and NCAAs.

Background The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Institutes of Health–sponsored United States database, provides a platform to understand this emerging population. Methods Between September 19, 2012, and December 31, 2017, 30 hospitals implanted 508 devices in 423 patients aged younger than 19 years. This past year was one of evolution for the database as its management was transitioned to The Society of Thoracic Surgery; therefore, this report does not include data from institutions not under contract by August 1, 2018. Results Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%), and congenital heart disease (CHD) in 86 (20%), with 52 of these patients having single-ventricle physiology. The two most common support strategies included left VAD in 342 (81%) and biventricular assist device in 64 (15%). Positive outcome (alive on device or bridge to transplantation/recovery) was 80% at 6 months (overall mortality of 20%). The patient cohort for implantable continuous-flow (IC) pumps (n = 197; age at implant, 13.4 ± 3.8 years; 19% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 21% intubated at implant, and 12% with CHD) was significantly different from the paracorporeal continuous-flow (PC) pump cohort (n = 79; age, 3.9 ± 5.2 years; 49% Intermacs profile 1, 86% intubated at implant, and 38% with CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 121; age, 3.3 ± 3.9 years; 41% Intermacs profile 1, 77% intubated at implant, and 21% with CHD). Consistent with their cohort composition, device type positive outcomes at 6 months were PC, 63%; PP, 77%; and IC, 92%. Parametric hazard modeling showed an early hazard for death was associated with Intermacs profile 1, biventricular assist device, percutaneous devices, PC devices, small-volume institutions, low age, and low weight, whereas a constant hazard was associated with intubation and liver dysfunction at time of implant. Conclusions IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs exceed 90% at 6 months, which may represent our field’s maturation in both patient selection and timing of implantation. The PP/PC devices are currently limited to supporting our most challenging patients, those weighing less than 20 kg and those with CHD. The introduction of new devices and our communities’ commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support and will also focus on improving the quality of life of children supported with VADs.

Mitral valve anomalies in children are rare but frequently severe, recalcitrant, and not often amenable to primary repair, necessitating mechanical mitral valve replacement (M-MVR). This study examined outcomes of a cohort undergoing a first M-MVR at age younger than 21 years.We queried the Pediatric Cardiac Care Consortium, a multi-institutional United States-based cardiac intervention registry, for patients undergoing first M-MVR for 2-ventricle congenital heart disease. Survival and transplant status through 2014 were obtained from Pediatric Cardiac Care Consortium and linkage with the National Death Index and the Organ Procurement and Transplantation Network.We identified 441 patients (median age, 4.3 years; interquartile range, 1.3 to 10.1 years) meeting study criteria. The commonest disease necessitating M-MVR was atrioventricular canal (44.3%). Early mortality (death90 days after M-MVR) was 11.1%; there was increased risk of early death if age at M-MVR was younger than 2 years (odds ratio, 7.8; 95% confidence interval [CI], 1.1 to 56.6) and with concurrent other mechanical valve placement (odds ratio, 8.5; 95% CI, 2.0 to 35.6). In those surviving more than 90 days after M-MVR, transplant-free survival was 76% at 20 years of follow-up (median follow-up, 16.6 years; interquartile range, 11.9 to 21.3 years). Adjusted analysis in those who survived more than 90 days showed elevated risk of death/transplant for boys (hazard ratio, 1.5; 95% CI, 1.0 to 2.3), age at M-MVR younger than 2 years (10-year survival: hazard ratio, 4.3; 95% CI, 1.2 to 15.1), and nonbileaflet prosthesis placement (hazard ratio, 2.4; 95% CI, 1.3 to 4.3).M-MVR is a viable strategy in children with unrepairable mitral valve disease. Age younger than 2 years at the first M-MVR is associated with significant early risk of death and poorer long-term survival.

Background Aortic valve replacement in young children is associated with technical difficulties and potential morbidity. In contrast to the versatile Ross operation, mechanical prostheses (MP) are uncommonly used. Methods We examined transplant-free survival and cardiac reoperation among 124 young children (aged 1-6 years) who underwent the Ross operation (n = 84) or MP (n = 40) for congenital disease (1982-2003) using the Pediatric Cardiac Care Consortium database. We explored variables influencing outcomes. Results Children who received MP were operated in an earlier era and were more likely to have aortic regurgitation, conotruncal abnormalities, prior aortic valve surgery, and to need Konno annular enlargement. Although no significant differences were found in hospital mortality (1.2% vs 5.0%, P = .24) or 15-year transplant-free survival (94.1% vs 87.5%, P = .16) between Ross and MP recipients, survival diverged with later follow-up (91.3% vs 68.9%, respectively, at 25 years; P = .01). On multivariable regression analysis the association of MP use and transplant-free survival changed over time (hazard ratios, 0.8 [95% confidence interval, 0.1-4.4; P = .78] vs 6.0 [95% confidence interval, 0.6-63.1; P = .13], respectively) before and after 17 years. Cumulative incidence of cardiac reoperation at 10 years was 37.7% and 53.6% after the Ross procedure and MP, respectively (P = .05). The most common reoperation after the Ross procedure was conduit replacement and pacemaker ± automated internal cardiac defibrillator and after MP was pacemaker ± automated internal cardiac defibrillator and redo aortic valve replacement. Conclusions Over the study period there was a trend for increased Ross utilization. Interestingly MP use was associated with comparable operative mortality and survival up to 17 years, albeit with higher need for redo aortic valve replacement. On longer follow-up survival diverged with increased attrition in the MP group, likely because of late valve– and reoperation-related complications.

OBJECTIVE Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA. METHODS A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan. RESULTS CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P

Background The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), originally a National Institutes of Health–sponsored U.S. database, provides a platform to understand the population of children supported with ventricular assist devices (VADs) during this time of increasing numbers, new devices, expanding indications, and improved outcomes. Methods Between September 19, 2012, and December 31, 2019, 44 hospitals implanted 1031 devices in 856 patients under 19 years of age. Results Overall, diagnosis was cardiomyopathy in 497 (58%) patients, congenital heart disease (CHD) in 216 (25%), myocarditis in 85 (10%), and other in 58 (7%). Positive outcome (alive on device or bridge to transplantation and recovery) occurred in 82% at 6 months. The patient cohort for implantable continuous (IC) pumps (n = 365) (age 13.2 ± 3.9 years, 18% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 23% intubated at implantation, 16% with CHD) was significantly different from the paracorporeal continuous (PC) pump cohort (n = 212) (age 3.6 ± 4.9 years, 46% Intermacs profile 1, 81% intubated, 42% CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 230) (age 2.7 ± 3.5 years, 31% Intermacs profile 1, 76% intubated, 26% CHD). Consistent with their cohort composition, positive outcomes at 6 months based on device type were the following: IC, 92%; PC, 68%; and PP, 81%. The incidence of cerebrovascular accidents in the IC, PC, and PP cohorts was 7%, 14%, and 15%, respectively. Conclusions IC VADs, the most common VAD type placed in children, are associated with improved outcomes compared with PP and PC devices, though PP and PC devices are limited to supporting our most challenging patients. Noteworthy, the incidence of cerebrovascular accidents for pediatric VADs has significantly decreased and is now 11% overall. This report demonstrates again that although often attributed to age, size, or device type, much of the burden in mortality and adverse events is correlated to the patient’s overall state at VAD implantation.

As coronavirus disease 2019 (COVID-19) cases surge worldwide, an urgent need exists to enhance our understanding of the role of extracorporeal membrane oxygenation (ECMO) in the management of severely ill patients with COVID-19 who develop acute respiratory and cardiac compromise refractory to conventional therapy. The purpose of this manuscript is to review our initial clinical experience in 32 patients with confirmed COVID-19 treated with ECMO. A multi-institutional registry and database was created and utilized to assess all patients who were supported with ECMO provided by SpecialtyCare. Data captured included patient characteristics, pre-COVID-19 risk factors and comorbidities, confirmation of COVID-19 diagnosis, features of ECMO support, specific medications utilized to treat COVID-19, and short-term outcomes through hospital discharge. This analysis includes all of our patients with COVID-19 supported with ECMO, with an analytic window starting March 17, 2020, when our first COVID-19 patient was placed on ECMO, and ending April 9, 2020. During the 24 days of this study, 32 consecutive patients with COVID-19 were placed on ECMO at nine different hospitals. As of the time of analysis, 17 remain on ECMO, 10 died before or shortly after decannulation, and five are alive and extubated after removal from ECMO, with one of these five discharged from the hospital. Adjunctive medication in the surviving patients while on ECMO was as follows: four of five survivors received intravenous steroids, three of five survivors received antiviral medications (Remdesivir), two of five survivors were treated with anti-interleukin-6-receptor monoclonal antibodies (Tocilizumab or Sarilumab), and one of five survivors received hydroxychloroquine. Analysis of these 32 COVID-19 patients with severe pulmonary compromise supported with ECMO suggests that ECMO may play a useful role in salvaging select critically ill patients with COVID-19. Additional patient experience and associated clinical and laboratory data must be obtained to further define the optimal role of ECMO in patients with COVID-19 and acute respiratory distress syndrome (ARDS). These initial data may provide useful information to help define the best strategies to care for these challenging patients and may also provide a framework for much-needed future research about the use of ECMO to treat patients with COVID-19.

Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease.This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery.Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita.Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS).Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

ObjectiveSurgical coronary revascularisation in children with congenital heart disease (CHD) is a rare event for which limited information is available. In this study, we review the indications and outcomes of surgical coronary revascularisation from the Pediatric Cardiac Care Consortium, a large US-based multicentre registry of interventions for CHD.MethodsThis is a retrospective cohort study of children (ResultsCoronary revascularisation was accomplished by bypass grafting (n=72, median age 6.8 years, range 3 days–17.4 years) or other operations (n=65, median age 2.6 years, range 5 days–16.7 years) in 137 patients. Most revascularisations were related to the aortic root (61.3%) or coronary anomalies (27.7%), but 10.9% of them were unrelated to either of them. Twenty in-hospital deaths occurred, 70% of them after urgent ‘rescue’ revascularisation in association with another operation. Long-term outcomes were available by external linkage for 54 patients surviving to hospital discharge (median follow-up time 15.0 years, max follow-up 29.8 years) with a 15-year transplant-free survival of 91% (95% CI 83% to 99%).ConclusionsSurgical coronary revascularisation can be performed in children with CHD with acceptable immediate and long-term survival. Outcomes are dependent on indication, with the highest mortality in rescue procedures.

Background Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute–sponsored North American database, provides a platform to understand this emerging population. Methods Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs. FDA approved durable devices as well as temporary support devices were included. The second annual report updates the current Pedimacs data. Patients implanted with temporary devices are included in Pedimacs and this analysis includes this group of paracorporeal continuous flow VADs. Results Over the 4 years, 42 hospitals implanted 432 devices in 364 patients less than 19 yrs of age. Diagnoses included cardiomyopathy in 223 (61%), myocarditis in 41 (11%), and congenital heart disease in 77 (21%), of which 48 had single-ventricle physiology. At implant, 87% were at Intermacs patient profile 1 or 2. The age distribution of children (59% male) supported on VAD included 69 (19%) aged younger than 1 year, 66 (18%) aged 1 to 6 years, 56 (15%) aged 6 to 10 years, and 173 (48%) aged 11 to 19 years. Median follow-up was 2.2 months (range, 1 day to 41.5 months). Median (interquartile) age at implant was 1.7 (0.3–10.0) years for paracorporeal continuous-flow pumps ( n = 60), 1.7 (0.4–5.3) years for paracorporeal pulsatile pumps ( n = 105), and 15.0 (11.3–16.9) years for implantable continuous-flow pumps ( n = 174). Support strategies included LVAD in 293 (80%), biventricular device in 55 (15%), and total artificial heart in 8 (2%). Nearly 50% of patients underwent transplantation within 6 months, with overall mortality of 19%. Adverse event burden continues to be high. Conclusions Pedimacs constitutes the largest longitudinal pediatric VAD registry. Preimplant data across centers will be helpful at creating shared protocols with which to improve outcomes. Adverse events continue to be the major challenge, especially among the young critically ill children with complex congenital disease.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research, and community service.

Background: Mortality associated with correction of type IV total anomalous pulmonary venous connection (TAPVC) is generally reported in combination with other anatomic types. The objective of this study is to review surgical outcomes associated with the repair of type IV TAPVC by analyzing a multi-institutional cohort specific for this group. We also analyze patient-specific variables that may contribute to poor operative outcomes. Methods: A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) registry identified patients who underwent repair of type IV TAPVC between 1982 and 2007. Variables reviewed included gender, prematurity, age at repair, anatomic pattern, presence of obstruction, associated anomalies, and operative mortality. Subclassifications were defined as type IV A (2+2 pattern), type IV B (3+1 pattern), and type IV C (bizarre). Results: Of the 2,248 patients with the diagnosis of TAPVC, 215 belonged to type IV. For type IV, the overall unadjusted mortality was 26%. There was no difference in mortality based on the particular anatomic drainage pattern. Twenty-eight percent had partial obstruction of the pulmonary venous return, with no patient having complete obstruction. Patients with obstruction had a significantly greater mortality than those without obstruction (39% vs 20%, P = .005). Approximately 16% of patients who present with obstruction of some pulmonary vein(s) underwent an emergency repair. Conclusion: Type IV TAPVC is a rare disease with a diverse anatomic presentation. Even though a small number of the patients with obstruction underwent emergent repair, mortality remained significant. This likely represents the intrinsic lung pathology that must be considered in the postoperative period.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

ObjectivePulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy.MethodsCohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network.ResultsEligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6–22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982–1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3–1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052).ConclusionsTransplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.

Total anomalous pulmonary venous connection (TAPVR) is a rare lesion with the pulmonary veins commonly connecting to a venous confluence that ultimately drains to the desaturated atrial chamber. Long-term medical management results in poor survival, making surgical intervention necessary. Correction during the newborn period is associated with low mortality and excellent mid-term outcomes. Diagnostic advances now provide better visualization of these defects than ever before, allowing accurate and detailed preoperative definition of the anomalous anatomy and physiology. Infants who present with obstruction in the immediate neonatal period require emergent operation and often face a difficult postoperative course, but long-term survival has significantly improved. Surgical repair for infants with less severe cases can be performed electively, but there is evidence to suggest that early repair may result in improved postoperative outcomes. Recurrent pulmonary venous stenosis is a vexing common postoperative morbidity, occurring in up to 25% of TAPVR patients. There are multiple surgical techniques in use to decrease these morbidities, though controversy regarding the optimal method persists. Overall, surgical outcomes for TAPVR have greatly improved, and patients with this condition appear to enjoy excellent long-term functional status today.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry that captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operation groups performed from January 2014 to December 2017. This analysis documents the overall aggregate Operative Mortality for operations performed at North American participants in The Society of Thoracic Surgeons Congenital Heart Surgery Database and the interquartile range (IQR) for participant-specific rates of Operative Mortality for the following procedural groups: off-bypass coarctation repair, 1.5% (IQR, 0.0% to 1.4%); ventricular septal defect repair, 0.5% (IQR, 0.0% to 0.5%); tetralogy of Fallot repair, 1.3% (IQR, 0.0% to 2.0%); complete atrioventricular canal repair, 2.5% (IQR, 0.0% to 4.1%); arterial switch operation, 2.2% (IQR, 0.0% to 3.0%); arterial switch operation and ventricular septal defect repair, 4.6% (IQR, 0.0% to 7.1%); Glenn/hemiFontan, 1.8% (IQR, 0.0% to 2.7%); Fontan operation, 1.0% (IQR, 0.0% to 0.4%); truncus arteriosus repair, 9.5% (IQR, 0.0% to 15.4%); and Norwood (stage 1) operation, 15.0% (IQR, 8.3% to 25.0%).

Background: The balance of hemostasis and anticoagulation is a concern for patients dependent upon ventricular assist devices (VADs). Bleeding is a common complication with both short- and long-term use of these devices. A better understanding of the risk factors and etiologies of bleeding associated with these devices is needed and could improve the overall results. We sought to determine the relationship of mechanical circulatory assist device use with acquired von Willebrand disease (avWD) in children. Methods: Data were analyzed retrospectively via review of the medical record of 19 consecutive patients who were supported with the Berlin EXCOR VAD for greater than 24 hours. Laboratory testing for avWD was performed at the discretion of the clinical team, often in association with clinical bleeding. Results: Of 19 pediatric patients, 10 (52.6%) had laboratory testing consistent with avWD. Median time to detection of avWD was 35 days postimplantation of device (range 0-310 days). Both minor mucosal bleeding and bleeding requiring intervention were highly prevalent in patients in whom avWD was identified (10/10 [100%] and 7/10 [70%]). The mean age of all patients was 3.3 years, but patients found to have avWD tended to be older (mean 5.3 years) and supported with larger volume VADs. Conclusions: This experience demonstrates a high prevalence of avWD following EXCOR implantation. Bleeding, older age, and larger VAD size may be associated with avWD. These results should stimulate critical evaluation of individualized anticoagulation regimens in pediatric VAD patients.

The World Society for Pediatric and Congenital Heart Surgery was founded with the mission to “promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research, and community service.” Early on, the Society’s members realized that a crucial step in meeting this goal was to establish a global database that would collect vital information, allowing cardiac surgical centers worldwide to benchmark their outcomes and improve the quality of congenital heart disease care. With tireless efforts from all corners of the globe and utilizing the vast experience and invaluable input of multiple international experts, such a platform of global information exchange was created: The World Database for Pediatric and Congenital Heart Disease went live on January 1, 2017. This database has been thoughtfully designed to produce meaningful performance and quality analyses of surgical outcomes extending beyond immediate hospital survival, allowing capture of important morbidities and mortalities for up to 1 year postoperatively. In order to advance the societal mission, this quality improvement program is available free of charge to WSPCHS members. In establishing the World Database, the Society has taken an essential step to further the process of global improvement in care for children with congenital heart disease.

BACKGROUND The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children. METHODS Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device. RESULTS Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 ± 5.7 years vs 9.8 ± 6.5 years; p < 0.0001) and smaller (0.8 ± 0.5 m2 vs 1.2 ± 0.7 m2; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02). CONCLUSIONS VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with CHD who received implantable continuous-flow VADs, survival rates were higher and comparable to those of children without CHD. Increased experience correlated with better survival in pediatric VADs.

We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery.We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports.There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features.Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

Following several years of planning and design, the World Database for Pediatric and Congenital Heart Surgery (WDPCHS) went live on January 1, 2017. The first operational year of this valuable database has proven very successful. As of February 28, 2018, more than 4,500 patients have been submitted from 39 centers; participation currently spans 19 countries over 6 continents, with 120 more centers in the enrollment pipeline. This update, the first in a series, is intended to provide World Society for Pediatric and Congenital Heart Surgery members and others who care for children with congenital heart disease with a broad overview of current progress and ongoing activities surrounding the WDPCHS.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive registry of clinical outcomes that captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operation groups performed from January 2013 through December 2016. This analysis documents the overall aggregate Operative Mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.4%%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 2.0%); complete atrioventricular canal repair, 2.7% (0.0% to 4.4%); arterial switch operation, 2.2% (0.0% to 2.9%); arterial switch operation and ventricular septal defect repair, 5.1% (0.0% to 8.3%); Glenn/HemiFontan, 2.1% (0.0% to 3.1%); Fontan operation, 1.1% (0.0% to 0.0%); truncus arteriosus repair, 10.1% (0.0% to 15.4%); and Norwood procedure, 15.8% (9.0% to 25.0%).

The goal of this study was to delineate outcomes of children weighing10 kg supported with the Berlin Heart EXCOR Pediatric Ventricular Assist Device (EXCOR Pediatric, Berlin Heart Inc., The Woodlands, Texas) and to identify factors that increased the risk of all-cause mortality in this population.Ventricular assist devices have been shown to be an effective bridge to transplant, with improved outcomes compared with use of extracorporeal membrane oxygenation. Smaller patients may be at greatest risk for poor outcomes, but it remains unclear if mortality is uniform across all smaller candidates.Patients included in the analysis were part of a multicenter prospective cohort study examining the use of the Berlin Heart EXCOR Pediatric Ventricular Assist Device as a bridge to transplant. All children who received the device between May 9, 2007 and December 31, 2010, and who were enrolled in the sponsor's U.S. regulatory database, were identified and analyzed. Multivariable analysis was performed to determine risk factors associated with mortality.A total of 97 children weighing10 kg were included (median age 6.2 months; median weight 6.2 kg; median duration of support 26 days). Successful outcomes were achieved in 56.7% of patients. Independent risk factors for mortality in smaller children included pre-existing congenital heart disease (odds ratio: 4.8 [95% confidence interval: 1.5 to 15.0]; p = 0.007) and an elevated bilirubin level (odds ratio: 5.3 [95% confidence interval: 2.0 to 14.3]; p = 0.001).Overall results for children weighing10 kg were inferior to those of their larger counterparts. This outcome was primarily influenced by congenital heart disease and presence of elevated pre-implant bilirubin levels. These factors should be taken into consideration at decision making because reasonable outcomes can be achieved in a select population of children weighing10 kg.

ObjectiveContemporary outcomes data for complete atrioventricular septal defect (CAVSD) repair are limited. We sought to describe early outcomes of CAVSD repair across a large multicenter cohort, and explore potential associations with patient characteristics, including age, weight, and genetic syndromes.MethodsPatients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having repair of CAVSD (2008-2011) were included. Preoperative, operative, and outcomes data were described. Univariate associations between patient factors and outcomes were described.ResultsOf 2399 patients (101 centers), 78.4% had Down syndrome. Median age at surgery was 4.6 months (interquartile range, 3.5-6.1 months), with 11.8% (n = 284) aged ≤2.5 months. Median weight at surgery was 5.0 kg (interquartile range, 4.3-5.8 kg) with 6.3% (n = 151)

Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD.We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality.There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p0.001). Mean age at repair for group 1 (n = 286) and group 2 (n = 472) was 87.4 days and 21.6 days, respectively (p = 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p = 0.130). Discharge mortality for group 1 and group 2 was similar, at 8.7% and 9.1%, respectively (p = 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%.The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach.

BackgroundThe entity of crossed pulmonary arteries was first described by Jue, Lockman, and Edwards in 1966, in a patient with trisomy 18. Since then, several series have been described, both in terms of the isolated anatomic variant, or its association with other intracardiac or extracardiac anomalies. We describe a rare association that has previously not been reported.Methods and resultsInstitutional Review Board approval for a retrospective chart review was obtained. Over the period 2011 through 2013, we have encountered six patients in whom the crossed origins of the pulmonary arteries from the pulmonary trunk were associated with hypoplasia of the transverse aortic arch, an association that, to the best of our knowledge, has previously not been reported. In all of the patients, the isthmic component of the aortic arch was inserted in an end-to-side manner into the ductal arch, with additional discrete coarctation in half of the patients.ConclusionTo the best of our knowledge, no cases of crossed pulmonary arteries have been described in association with hypoplasia of the transverse aortic arch. We draw comparisons between the cases with exclusively tubular hypoplasia, and those with the added problem of the more typical isthmic variant of aortic coarctation. In all cases, the ability to reconstruct cross-sectional images added significantly to the diagnosis and understanding of these complex lesions. These findings have specific surgical implications, which are discussed.

Importance Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse. Objective To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics. Design, Setting, and Participants Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018. Exposures We examined patient-associated and surgery-associated risk factors affecting survival. Main Outcomes and Measures We analyzed the transplant-free survival during early ( Results Of the 3283 patients who survived repair for simple TOF and met the study’s inclusion criteria, 56.4% were male and 43.6% were female. Twenty-five–year survival following TOF repair was 94.5%. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49) and non–valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19). Presence of a genetic abnormality was associated with increased risk of death both in the early (HR, 3.64; 95% CI, 2.05-6.47) and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44). Conclusions and Relevance Long-term survival after simple TOF repair is excellent. Staged repair and non–valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.

Background Treatment of congenital heart disease may include placement of a right ventricle to pulmonary artery conduit that requires future surgical replacement. We sought to identify surgeon-modifiable factors associated with durability (defined as freedom from surgical replacement or explantation) of the initial conduit in children less than 2 years of age at initial insertion. Methods Since 2002, 429 infants were discharged from 24 Congenital Heart Surgeons' Society member institutions after initial conduit insertion. Parametric hazard analysis identified factors associated with conduit durability while adjusting for patient characteristics, the institution where the conduit was inserted, and time-dependent interval procedures performed after conduit insertion but before replacement/explantation. Results In all, 138 conduit replacements (32%) and 3 explantations (1%) were performed. Conduit durability at a median follow-up of 6.0 years (range, 0.1 to 11.7) was 63%. After adjusting for interval procedures and institution, placement of a conduit with smaller z-score was associated with earlier replacement/explantation ( p = 0.002). Moreover, conduit durability was substantially reduced with aortic allografts ( p = 0.002) and pulmonary allografts ( p = 0.03) compared with bovine jugular venous valved conduits (JVVC). The JVVC were 12 mm to 22 mm in diameter at insertion (compared with 6 mm to 20 mm for allografts); therefore, a parametric propensity-adjusted analysis of patients with aortic or pulmonary allografts versus JVVC with diameter of 12 mm or greater was performed, which verified the superior durability of JVVC. Conclusions Pulmonary conduit type and z-score are associated with late conduit durability independent of the effects of institution and subsequent interval procedures. Surgeons can improve long-term conduit durability by judiciously oversizing, and by selecting a JVVC.

Background The long‐term outcomes of patients undergoing interventions for congenital heart disease ( CHD ) remain largely unknown. We linked the Pediatric Cardiac Care Consortium ( PCCC ) with the National Death Index ( NDI ) and the United Network for Organ Sharing Dataset ( UNOS ) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant. Methods and Results We used direct identifiers from 59 324 subjects registered in the PCCC between 1982 and 2003 to test for completeness of case ascertainment of subjects with known vital and heart transplant status by linkage with the NDI and UNOS registries. Of the 4612 in‐hospital deaths, 3873 were identified by the NDI as “true” matches for a sensitivity of 84.0% (95% CI, 82.9–85.0). There was no difference in sensitivity across 25 congenital cardiovascular conditions after adjustment for age, sex, race, presence of first name, death year, and residence at death. Of 455 known heart transplants in the PCCC , there were 408 matches in the UNOS registry, for a sensitivity of 89.7% (95% CI , 86.9–92.3). An additional 4851 deaths and 363 transplants that occurred outside the PCCC were identified through 2014. Conclusions The linkage of the PCCC with the NDI and UNOS national registries is feasible with a satisfactory sensitivity. This linkage provides a conservative estimate of the long‐term death and heart transplant events in this cohort.

Background Outcomes for repair of total anomalous pulmonary venous connection (TAPVC) from individual institutions suggest a significant improvement in mortality over the past several decades. The purpose of this study is to review the outcomes after repair of TAPVC from a large multiinstitutional registry. Methods A retrospective review of the multiinstitutional database, the Pediatric Cardiac Care Consortium (PCCC), was used to identify patients with the diagnosis of TAPVC who underwent complete correction between 1982 and 2007. Data reviewed included age, decade of primary operation, anatomic type, presentation, and in-hospital mortality. Results Of the 118,084 surgical procedures submitted to the PCCC, 2,191 (1.9%) consisted of primary surgical correction of TAPVC. Sixty-one percent of the cohort was male, with 6.8% reported as premature. Overall in-hospital surgical mortality for simple TAPVC was 13%. Mortality was 20% from 1982 to 1989, 16% from 1990 to 1999, and 8% from 2000 to 2007. Obstruction to the anomalous pulmonary venous connection occurred in 29%, with a mortality of 26%. Conclusions Surgical outcomes from repair of congenital cardiac anomalies have significantly improved over the past several decades. Multiinstitutional large databases are needed to confirm results published from single-institution experiences. Although improvements in surgical repair of TAPVC have occurred over the past three decades, specific subtypes still experience significant mortality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2011 to December 2014 and documenting overall discharge mortality (interquartile range among programs with more than 9 cases): off-bypass coarctation, 1.0% (0.0% to 0.9%); ventricular septal defect repair, 0.7% (0.0% to 1.1%); tetralogy of Fallot repair, 1.0% (0.0% to 1.7%); complete atrioventricular canal repair, 3.2% (0.0% to 6.5%); arterial switch operation, 2.7% (0.0% to 5.6%); arterial switch operation plus ventricular septal defect, 5.3% (0.0% to 6.7%); Glenn/hemiFontan, 2.1% (0.0% to 3.8%); Fontan operation, 1.4% (0.0% to 2.4%); truncus arteriosus repair, 9.6% (0.0 % to 11.8%); and Norwood procedure, 15.6% (10.0% to 21.4%).

Advances in palliation of congenital heart disease have resulted in improved survival to adulthood. Many of these patients ultimately develop end-stage heart failure requiring left ventricular assist device implantation (LVAD). However, morphologic differences in the systemic ventricle of these patients require careful attention to cannula placement. We report on the evolution of our surgical technique for implanting LVADs in 3 patients with transposition of the great arteries and congenitally corrected transposition of the great arteries. Applying standard LV cannulation techniques to the systemic ventricle led us too anteriorly in our first patient, creating obstruction by the moderator band. Subsequent use of epicardial and transesophageal echocardiography allowed for intraoperative localization of the intracardiac muscular structures to identify the optimal cannulation site. The acute angle of the inflow cannula on the DeBakey LVAD (MicroMed Technology, Houston, TX) required flipping the device 180°. The HeartMate II device (Thoratec, Pleasanton, CA) could be shifted towards the midline. One patient underwent successful transplant and 2 are home waiting for a donor organ. We conclude from our experience that LVAD surgery can be safely performed in patients with congenital heart disease when implanted under echocardiographic guidance.

Background: The surgical mortality associated with repair of coarctation of the aorta (CoA) over a 25-year period was examined. Risk factors for discharge mortality were evaluated as well as the surgical techniques and its evolution over the period studied. Methods: Utilizing the pediatric cardiac care consortium, we conducted a retrospective review of patients less than 18 years of age submitted between 1982 and 2007. Variables reviewed included weight at birth, age and weight at the operation, type of coarctation repair, associated cardiac anomalies, year of repair, center-specific volume, postoperative length of stay, and in-hospital mortality. Results: There were 7,860 patients submitted with the procedural code for repair of CoA. Forty-five percent underwent repair within 30 days of life (n = 3,549), including 1,444 patients who were operated upon within the first 7 days of life (18% of all patients). Seventy percent (n = 5,528) of patients had an isolated CoA (iCoA). The overall mortality for the entire group was 4.2% (n = 331), decreasing to 2.0% (n = 114) for iCoA ( P < .0001). A hypoplastic aortic arch occurred in 4.6%, with a mortality of 10.6%. Coarctectomy with an end-to-end reconstruction was the most common procedure performed. Multivariable modeling for discharge mortality was significant for diagnosis of ventricular septal defect, operative weight, operative year, and diagnosis of aortic arch hypoplasia. Conclusions: Operative repair of CoA is accomplished with a low mortality, although certain subgroups have persistently inferior outcomes. The techniques utilized for aortic reconstruction have evolved, with coarctectomy and an end-to-end anastomosis becoming the dominant surgical procedures.

There are numerous congenital defects that may present with human hearts, and many typically require surgical intervention. The primary goal of this chapter is to briefly define such abnormalities and introduce the reader to the various classification schemes that have been used to describe their relative anatomical and functional features. The chapter will also highlight the more common surgical procedures utilized to treat congenital cardiac lesions.

Acute heart failure can occur in children as a result of hemodynamic insults imposed on the heart by structural congenital defects or in anatomically normal hearts in which the myocardium is damaged by an inflammatory or infectious process (myocarditis, metabolic diseases leading to cardiomyopathy). Postcardiotomy heart failure following surgical repair of congenital heart defects can also lead to the need for postoperative support. Mechanical circulatory support devices have been used successfully as a bridge to recovery in children, especially in the management of acute myocarditis or postcardiotomy heart failure. The use of these devices as a bridge to transplantation has also been shown to decrease waiting list mortalities and improve the efficiency of donor organ utilizations in children. However, currently available mechanical circulatory support options for infants and children are still quite limited, especially with regard to size options for smaller patients and the long-term duration of support often required. Future devices are currently in development for clinical use on a broad scale and will greatly facilitate the successful support of children with heart failure as a bridge to myocardial recovery or heart transplantation.

The Pediatric Cardiac Care Consortium (PCCC) was developed to provide individual institutions feedback about outcomes of common surgical and catheter based procedures and allow comparison of these outcomes on an international scale. The PCCC successfully accomplished this mission for over 30 years. The data was accurate, and provided to the individual centers on an annual basis. Recently, the database has elected to stop collecting information from the member institutions. As before, leaders within this organization have had to evolve the mission and find applications for the rich data that currently exists within the database. This chapter describes the history, current status, and future of the PCCC.

BACKGROUND: In previous work from a single center, Technical Performance Score (TPS), a tool that assesses technical adequacy of repair, has been shown to be strongly associated with outcomes in congenital cardiac surgery. We sought to validate the efficacy of TPS in a multicenter environment. METHODS: All patients (1 day to 62 years) who were discharged from January 1 to December 31, 2011; and who underwent 9 congenital cardiac procedures (Arterial switch operation [84], Bidirectional Glenn [75], Atrioventricular canal repair [135], Fontan [97], Arch repair on pump [58], Stage I Procedure [85], Pulmonary valve replacement [116], Tetralogy of Fallot repair [112], and Ventricular septal defect repair [163]); from 5 centers were included. Based on echocardiograms (echo) prior to discharge or death, and unplanned reinterventions at surgical site; TPS was assigned using previously established criteria. Case complexity was determined by RACHS-1 category. Outcomes included (a) major postoperative adverse events (AE) excluding unplanned reinterventions, (b) length of ventilation, and (c) postoperative hospital stay. Adjusted analysis used logistic/linear regression to determine odds ratio (OR) and regression coefficient (b) for each outcome. RESULTS: There were 925 hospital discharges: 418 (45%) were RACHS-1 category 2, 295 (32%) category 3, 85 (9%) category 4, 86 (9%) category 6 and the cohort included 41 (4%) adults. TPS were as follows: 491 (53%) class 1-optimal, 263 (28%) class 2-adequate, 131 (14%) class 3-inadequate and 40 (4%) had no TPS assigned because of a lack of or incomplete echos (NA). There were 26 (2.8%) deaths (81% of deaths were in class 3) and 105 (11%) adverse events. Occurrence of major adverse events, ventilation time and hospital length of stay were all significantly higher in class 3 (Figure). On multivariable analysis adjusting for age, RACHS-1, prematurity, and presence of non-cardiac anomalies; Class 3 TPS was associated with a higher odds of AE (OR 7.4, CI 4.1-13.2, p CONCLUSION: TPS predicts outcomes after congenital heart surgery in a multicenter cohort, and can serve as quality assessment tool. Outcomes may be favorably influenced by focusing on technical excellence.

Mechanical ventricular assistance has become a reliable tool for the support of children and infants with heart failure. The devices have shown efficacy both as a bridge to transplantation and as a bridge to recovery. The potential complications that may occur with long-term support have not been fully described. This article reports the occurrence of a large pseudoaneurysm associated with the ascending aorta following explantation of the EXCOR Pediatric ventricular assist device. A management strategy for this potentially lethal complication is described.

Background . Transmyocardial laser revascularization (TMR) has been demonstrated effective for relieving angina, although prior studies have yielded inconsistent results regarding postoperative myocardial perfusion and function. This study evaluated long-term changes in myocardial perfusion and contractile reserve after TMR in a model of hibernating myocardium. Methods . Miniswine had subtotal left circumflex coronary artery occlusion to reduce resting blood flow to 10% of baseline. After 2 weeks in the low-flow state, positron emission tomography and dobutamine stress echocardiography were performed to document ischemic, viable (hibernating) myocardium in the left circumflex distribution. Animals then had sham redo thoracotomy ( n = 4) or TMR ( n = 6). Six months later the positron emission tomography and dobutamine stress echocardiography studies were repeated. Results . Myocardial blood flow in the left circumflex distribution as measured by positron emission tomography was significantly reduced in all animals after 2 weeks in the low-flow state. In animals that had TMR, there was significant improvement in myocardial blood flow to the lased regions 6 months postoperatively. No significant change in myocardial blood flow was seen in sham animals at 6 months. Dobutamine stress echocardiography after 2 weeks of low-flow demonstrated severe hypocontractility at rest in the left circumflex region of all animals, with a biphasic response to dobutamine consistent with hibernating myocardium. In animals that had TMR, there was a trend toward improved resting function and significantly improved regional stress function in the lased segments 6 months postoperatively, consistent with a reduction in ischemia. Global left ventricular wall motion at peak stress improved significantly as well. There was no change in wall motion 6 months postoperatively in sham-operated animals. Conclusions . This study found improvements in myocardial perfusion and regional and global contractile reserve 6 months after TMR in a porcine model of hibernating myocardium. This improved perfusion and function likely accounts for the clinical benefits of the procedure.

The diagnosis and management of follicular carcinoma of the thyroid gland remains a controversial topic. Fine needle aspiration, although very sensitive with other types of thyroid cancer, has limited accuracy with follicular lesions. The role of suppression combined with observation has yet to gain widespread acceptance. The extent of surgical excision of follicular carcinoma also raises several competing views. The goal of this review is to address these issues and present an algorithm for the management of follicular neoplasms of the thyroid.

A 2.6 kg female with the diagnosis of type "B" interruption of the "right" aortic arch and mitral atresia underwent stage I palliation with translocation and anastomosis of the distal right carotid artery to the descending aorta. The distal main pulmonary artery was anastomosed to the under surface of the neoaortic arch. A modified Blalock-Taussig shunt was constructed between the left side innominate artery and the left pulmonary artery. The child awaits stage II palliation.

Long chain free fatty acids (FFA) are the preferred metabolic substrates of myocardium under aerobic conditions. However, under ischemic conditions long chain FFA have been shown to be harmful both clinically and experimentally. Serum levels of free fatty acids frequently are elevated in patients with myocardial ischemia. The proposed mechanisms of the detrimental effects of free fatty acids include: (1) accumulation of toxic intermediates of fatty acid metabolism, such as long chain acyl-CoA thioesters and long chain acylcarnitines, (2) inhibition of glucose utilization, particularly glycolysis, during ischemia and/or reperfusion, and (3) uncoupling of oxidative metabolism from electron transfer. The relative importance of these mechanisms remains controversial. The primary site of FFA-induced injury appears to be the sarcolemmal and intracellular membranes and their associated enzymes. Inhibitors of free fatty acid metabolism have been shown experimentally to decrease the size of myocardial infarction and lessen postischemic cardiac dysfunction in animal models of regional and global ischemia. The mechanism by which FFA inhibitors improve cardiac function in the postischemic heart is controversial. Whether the effects are dependent on decreased levels of long chain intermediates and/or enhancement of glucose utilization is under investigation. Manipulation of myocardial fatty acid metabolism may prove beneficial in the treatment of myocardial ischemia, particularly during situations of controlled ischemia and reperfusion, such as percutaneous transluminal coronary angioplasty and coronary artery bypass grafting.