Isolated right ventricular apical hypoplasia with atrial fibrillation in a young female at her third trimester: a...

Pregnancy represents a significant life event for most women, inducing extensive physiological adaptations. Consequently, it poses additional risks for women with congenital heart disease (CHD), a leading cause of maternal mortality. Isolated right ventricular apical hypoplasia (IRVAH) is a poorly characterized CHD subset, with pregnancy outcomes remaining unreported. Our case of a primigravida with IRVAH underscores the need for heightened monitoring in this population.

We present a 28-year-old primigravida at 28 gestational weeks referred for structural right heart failure and paroxysmal atrial fibrillation (AF) with no palpitations and dyspnea. Diagnostic evaluation revealed IRVAH on echocardiography, characterized by preserved left ventricular function, severe tricuspid regurgitation, and right ventricular dilation, alongside electrocardiography showing AF; cardiac magnetic resonance imaging (MRI) subsequently confirmed apical right ventricular hypoplasia with no associated structural anomalies. Management was guided by multidisciplinary consensus, initiating stabilization with oral metoprolol (12.5 mg per dose, twice daily) for resting heart rate control (target heart rate <110 bpm). Pregnancy prolongation strategies included weekly fetal growth scans, daily maternal oxygen saturation (SpO₂)/blood pressure (BP) monitoring, and steroid coverage at 30 weeks for fetal lung maturation. Due to maternal AF recurrence despite therapy, fetal growth restriction (<10^th percentile), and the need to avoid hemodynamic stress from labor, an elective cesarean delivery was scheduled at 32 weeks. A preterm infant (1,580 g, Apgar scores 9/10) was delivered via cesarean section under spinal-epidural anesthesia and transferred to the neonatal intensive care unit (NICU) for recovery. Postoperative AF in the mother was successfully controlled with increased oral metoprolol (25 mg per dose, twice daily). At the 1-, 3-, and 6-month follow-ups, maternal cardiac function remained at New York Heart Association (NYHA) class I, and no developmental delays were observed in the infant.

This case demonstrates the critical value of multidisciplinary team collaboration in managing pregnancy complicated by IRVAH, underscoring the need for individualized monitoring and intervention strategies. These findings may serve as a reference for the clinical management of similar cases in the future.