Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). Results: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. Conclusions: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

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We report a case of hypoplastic left heart syndrome with an anomalous left coronary artery from the pulmonary artery (ALCAPA) identified intraoperatively during the Stage-II palliation. Due to recurring ventricular fibrillation on sternotomy, a hybrid Stage-I palliation was performed. During comprehensive Stage-II, the ALCAPA was reimplanted in the neoaorta and measures, including a nontraditional Damus connection/arch reconstruction and classic bilateral Glenn procedures, were taken to avoid compression of the coronary artery. After a successful Fontan procedure, he continues to do well at 5 years old, becoming the first patient reported in the literature to survive all the three stages of single-ventricle palliation.

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A baby boy with prenatally diagnosed hypoplastic left heart syndrome variant with obstructed veins was born in the operating room (OR) and underwent emergent Norwood operation and repair of obstructed infra-diaphragmatic total anomalous pulmonary venous connection. Post-operatively, esophageal atresia with tracheoesophageal fistula was identified and repaired on day of life 11. The patient is thriving at 22 months of age. ( Level of Difficulty: Advanced. )