Prenatally diagnosed coronary artery abnormalities in hypoplastic left heart syndrome are associated with a higher probability of heart transplantation.

OBJECTIVE: Coronary artery abnormalities (CA) occur in patients with hypoplastic left heart syndrome (HLHS) and may be associated with higher mortality and heart transplantation (HT). We aimed to determine whether fetuses with HLHS and prenatal CA have a higher risk of death or HT. METHODS: We performed a retrospective review of fetal echocardiograms with HLHS from 2011 to 2018. We excluded fetuses with ventricular septal defects, elective termination, death in utero, planned postnatal non-intervention, or absent follow-up data. Presence or absence of CA was determined by review of serial fetal echocardiograms. Survival analysis was used to evaluate the relationship between prenatal CA and death or HT. RESULTS: Of 86 patients with fetal HLHS, 11 had prenatal diagnosis of CA. Of these, six required HT and five died (one after undergoing HT); only one remains alive without HT. Of those without prenatal CA (n = 75), 25 died and 7 underwent HT. Patients with prenatal diagnosis of HLHS and CA had a significantly increased likelihood of death or HT (p-value <0.05). CONCLUSION: Prenatal diagnosis of CA in our cohort of patients with HLHS was associated with increased risk of death or HT. These data have significance for prenatal counseling and postnatal management.

Incidence and effect of early postoperative ventricular arrhythmias after congenital heart surgery.

BACKGROUND: Postoperative arrhythmias after pediatric congenital heart disease (CHD) surgery are a known cause of morbidity and are associated with mortality. A comprehensive evaluation of early postoperative ventricular arrhythmias (VAs) after CHD surgery has not been reported. OBJECTIVES: We sought to determine the incidence of in-hospital VAs after CHD surgery and assess the clinical relevance of this arrhythmia during the postoperative hospital course. METHODS: Patients undergoing CHD surgery at our center from September 2007 through December 2016 were prospectively enrolled. Univariate and multivariate analysis was used to assess the association between postoperative VAs and in-hospital mortality, adjusting for postoperative extracorporeal membrane oxygenation and stage 1 single ventricle palliation operations. RESULTS: A total of 2503 postoperative courses in 1835 patients were included. In all, 464 (18.5%) had VAs, of whom 135 (29.1%) received treatment. Monomorphic ventricular tachycardia was the most frequently treated ventricular arrhythmia (TVA; n=91 [62.3%]). TVAs were associated with increased postoperative extracorporeal membrane oxygenation (13.3% vs 5.5%; P < .001) and in-hospital mortality (14.9% vs 4.0%; P < .001). In multivariate analysis, TVA was an independent risk factor for in-hospital mortality (adjusted odds ratio 2.44; 95% confidence interval 1.21-4.92). CONCLUSION: Early postoperative VAs after CHD surgery are more common than previously reported. Postoperative VAs are associated with increased in-hospital mortality, and the subgroup of TVAs is an independent risk factor for in-hospital mortality.