Abstract
BACKGROUND: Fontan surgery outcomes have significantly improved, leading to an increased number of patients surviving into adulthood. However, data on pregnancy outcomes in this population remain limited. METHODS: A systematic search was conducted across four databases, including PubMed, Embase, Web of Science, and Cochrane CENTRAL, from inception to September 25, 2024, without language restrictions. Studies involving pregnancies in women with Fontan circulation that reported maternal or neonatal outcomes were included. Case series and case reports were excluded. RESULTS: A total of 18 studies met the eligibility criteria, including 400 pregnant women with Fontan circulation and 1260 pregnancies. An increase in maternal arrhythmias was observed, with a pooled incidence of 6.59% (95% CI 3.22%-10.83%), all of which were supraventricular tachycardia. The pooled incidence of maternal heart failure was 3.71% (95% CI 0.94%-7.67%), with no report of maternal mortality. Preterm delivery was common, with a pooled incidence of 57.31% (95% CI 40.19%-73.66%). Furthermore, the pooled incidence of neonatal mortality was 4.38% (95% CI 2.08%-7.23%), mainly due to complications from preterm births. CONCLUSION: Pregnancy in women with Fontan circulation is associated with maternal and neonatal adverse outcomes. Preconception counseling and multidisciplinary care are essential for supporting pregnancy planning, ensuring careful monitoring, and improving pregnancy outcomes.