Successful preterm cesarean delivery in a woman with right atrial isomerism: a case report

BACKGROUND: Right atrial isomerism is a rare condition marked by congenital splenic aplasia and associated cardiovascular and visceral malformations. The etiology of this condition remains unclear. Owing to the severity of concomitant cardiovascular anomalies, survival into adulthood is uncommon, and successful pregnancy outcomes are exceedingly rare. CASE PRESENTATION: A 30-year-old woman with a history of right atrial isomerism and a single right ventricle, who had undergone total cavopulmonary connection (TCPC) at age 15, was admitted at 32 weeks’ gestation due to decreased fetal movements. Her pregnancy had otherwise been uncomplicated, with preserved cardiac function (NYHA class I–II). A multidisciplinary Pregnancy Heart Team evaluation recommended cesarean delivery due to concerns regarding fetal well-being. A live infant was delivered with Apgar scores of 7 and 8 at 1 and 5 min, respectively. The maternal postoperative course was uneventful, with no cardiac decompensation. CONCLUSIONS: It is exceptionally rare for patients with right atrial isomerism to survive to adulthood and achieve a successful pregnancy outcome. Preconception counseling involving obstetricians, cardiologists, and cardiac surgeons is essential to assess pregnancy feasibility in high-risk women. Patients must be thoroughly informed about the potential increases in maternal and fetal morbidity and mortality. The determination of optimal timing for pregnancy termination must be individualized, incorporating meticulous consideration of maternal risks and fetal viability thresholds.