Objectives To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes. Study design Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes. Results Neurodevelopmental testing was performed at 14±1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean psychomotor (PDI) and mental developmental indices (MDI) were 80±18 and 96±14 respectively (normal 100±15, P<0.001 for each). Group-based trajectory analysis provided a two-group model (high” and “low”) for height z-score trajectory and brain type natriuretic peptide (BNP) trajectory. The predicted PDI scores were 15 points higher in the “high” height z-score trajectory compared with the “low” cluster (P<.001). A higher number of serious adverse events during the trial was associated with lower PDI scores (P=.02). The predicted MDI scores were 13–17 points lower in “low height trajectory- high BNP trajectory” group compared with the other three groups (P<.001). MDI scores were also lower in subjects who required extracorporeal membrane oxygenation during the neonatal hospitalization (P=.01) or supplemental oxygen at discharge (P=.01). Conclusions Neurodevelopmental outcome at 14 months of age is impaired in infants with SV physiology. Low height trajectory and high BNP trajectory were associated with worse neurodevelopmental outcomes. Efforts to improve nutritional status alone may not improve neurodevelopmental outcomes.

Objectives The purpose of this analysis was to assess preoperative risk factors prior to the first-stage Norwood surgery in infants with hypoplastic left heart syndrome and related single ventricle lesions, and to evaluate practice patterns in prenatal diagnosis as well as the role of prenatal diagnosis in outcome. Methods Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities and preoperative mortality rates were recorded. Results Of 906 infants, 677 (75%) had prenatal diagnosis, 15% were preterm (<37 weeks), and 16% were low birth weight (<2500 g). Rates of prenatal diagnosis varied by study site (59%-85%, p<0.0001). Major extracardiac congenital abnormalities were less prevalent in those born after prenatal diagnosis (6% vs. 10%, p=0.03). There were 26 (3%) deaths prior to Norwood palliation; preoperative mortality did not differ by prenatal diagnosis status (p=0.49). In multiple logistic regression models, preterm birth (p=0.02), major extracardiac congenital abnormalities (p<0.0001), and obstructed pulmonary venous return (p=0.02) were independently associated with preoperative mortality. Conclusions Prenatal diagnosis occurred in 75%. Preoperative death was independently associated with preterm birth, obstructed pulmonary venous return and major extracardiac congenital abnormalities. Adjusted for gestational age and the presence of obstructed pulmonary venous return, the estimated odds of preoperative mortality were 10 times greater for subjects with a major extracardiac congenital abnormality.

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Congenital extrahepatic portosystemic shunt (CEPS) is associated with polysplenia and heterotaxy and can cause portopulmonary hypertension. We report a 12-month-old girl who acutely died likely due to portopulmonary hypertension secondary to CEPS associated with heterotaxy and polysplenia. A retrospective radiographic review following her autopsy identified an anatomical explanation for the acute death in an infant.