Congenital heart disease (CHD) is the most commonly reported birth defect in newborns. Neonates with CHD are more likely to be born prematurely, and a higher proportion of preterm neonates have CHD than their term counterparts. The implications of preterm birth on the cardiac and noncardiac organ systems are vast and require special management considerations. The feasibility of surgical interventions in preterm neonates is frequently limited by patient size and delicacy of immature cardiac tissues. Thus, special care must be taken when considering the appropriate timing and type of cardiac intervention. Despite improvements in neonatal cardiac surgical outcomes, preterm and early term gestational ages and low birthweight remain important risk factors for in-hospital mortality. Understanding the risks of early delivery of neonates with prenatally diagnosed CHD may help guide perioperative management in neonates who are born preterm. In this review, we will describe the risks and benefits of early delivery, postnatal cardiac and noncardiac evaluation and management, surgical considerations, overall outcomes, and future directions regarding optimization of perinatal evaluation and management of fetuses and preterm and early term neonates with CHD.

Introduction: Digoxin use in patients with single ventricle physiology after stage I palliation (S1P) has been associated with reduced interstage mortality (IM). However, the S1P hybrid procedure comprised a small fraction of the patients in these evaluations. As it is being performed with increasing frequency, data regarding digoxin effects on IM after this procedure are needed. We sought to determine if digoxin prescription at discharge in infants with the S1P hybrid was associated with improved transplant-free interstage survival. Methods: National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021 were utilized. Infants discharged home after the S1P hybrid hospitalization were included. Patients were excluded if they had 1) supraventricular tachycardia, 2) death during S1P hospitalization, 3) conversion to Norwood during S1P hospitalization, or 4) remained hospitalized until stage II surgery. The primary outcome was transplant-free survival. Chi-square, Fisher’s Exact, Mann-Whitney U tests and multivariable logistic regression were used to compare patients receiving and not receiving interstage digoxin following S1P hybrid. Results: Of the 259 patients from 45 sites with S1P hybrid, 129 (50%) were discharged home on digoxin. Baseline characteristics including birth weight, gestational age at birth, race/ethnicity, cardiac diagnosis, presence of aortic atresia, noncardiac abnormalities or genetic syndromes, and one or more pre-operative risk factors were similar between groups. IM or heart transplant occurred in 30 (23%) patients in the no digoxin group compared to 18 (14%) in the digoxin group (P=0.06). With multivariate logistic regression analysis adjusting for home feeding route, degree of right ventricular dysfunction, atrioventricular valve regurgitation and oxygen saturation at discharge, home digoxin prescription was independently associated with a lower risk of IM or transplant (adjusted OR 0.49; 95% CI 0.25-0.98; P=0.04). Conclusions: In patients with single ventricle physiology who underwent a S1P hybrid procedure, digoxin prescription at hospital discharge was associated with improved transplant-free survival during the interstage period.