Intrauterine fetal demise after prenatal diagnosis of congenital heart disease: assessment of risk

Objectives Elective deliveries in fetal congenital heart disease (CHD) attempt to balance fetal and neonatal risk with the goal of optimizing overall outcome. However, the magnitude of the risk for intrauterine fetal demise (IUFD) is unclear. This study aimed to (1) determine the rate of IUFD and (2) identify fetal risk factors associated with IUFD. Methods Retrospective review of pregnancies complicated by CHD between 1998 and 2010. Data were collected regarding pregnancy outcome, extracardiac anomalies (ECA), genetic and cardiac diagnoses, severity of valve regurgitation, gestational age at birth and birth weight. Fisher's exact test and odds ratios were used to compare outcomes between groups. Results A total of 501 pregnancies analyzed resulted in 445 live births, 22 IUFD, 16 terminations and 18 unknown outcomes. Amongst IUFD, 27% had a genetic diagnosis, 50% had an ECA and 27% had severe valve regurgitation. IUFD odds increased threefold with ECA and sevenfold with severe valve regurgitation. IUFD occurred in 1.2% without risk factors. Conclusions IUFD in fetuses with CHD is associated with ECA, genetic syndromes and severe valve regurgitation. In absence of these fetal characteristics, the occurrence of IUFD is low, although it remains higher than in fetuses without CHD. © 2015 John Wiley & Sons, Ltd. What's Already Known About This Topic? The fetus with congenital heart disease is at increased risk for intrauterine fetal demise. However, the incidence, timing and risk factors that could inform perinatal decision making are not well defined. What Does This Study Add? The presence of a genetic syndrome, extracardiac anomaly or severe valve regurgitation increases the risk for fetal demise, which in this cohort was 1.2%.