Successful Vaginal Delivery in a Young Female With Eisenmenger Syndrome and Double Outlet Right Ventricle: A...

Eisenmenger syndrome (ES), a severe complication of uncorrected congenital heart defects, is characterized by pulmonary hypertension and right-to-left intracardiac shunting resulting in systemic hypoxemia. Double outlet right ventricle (DORV), a rare congenital anomaly where both great arteries arise predominantly from the right ventricle, further exacerbates hemodynamic instability. Pregnancy in women with ES and DORV is associated with extremely high maternal and fetal mortality rates, requiring meticulous, multidisciplinary management.

A 20-year-old Gravida 1 Para 0 woman at 34 weeks gestation presented with progressive dyspnea, cyanosis, and generalized edema. She had a single antenatal visit with no cardiovascular evaluation. Examination revealed finger clubbing, elevated jugular venous pressure, pitting edema, bibasilar lung crackles, and a systolic murmur. Vital signs were notable for heart rate 130 bpm, BP 110/70 mmHg, RR 25/min, and SpO₂ 80% on supplemental oxygen. Laboratory tests showed anemia and elevated CRP without fever. Imaging and echocardiography revealed a large ventricular and atrial septal defect with bidirectional shunt, severe tricuspid regurgitation, and estimated pulmonary artery pressure of 85 mmHg. DORV was suggested with the aorta arising from the right ventricle. A multidisciplinary team opted for vaginal delivery. Labor was induced with misoprostol (Bishop score 5) under continuous maternal monitoring. The patient delivered vaginally after 4 h. The newborn weighed 2.7 kg, with Apgar scores of 5 and 7 at 1 and 5 min, respectively. Postpartum, the mother received diuretics, blood transfusions, and antibiotics for elevated CRP. She stabilized by day seven and was transferred to cardiology for ongoing management.

This case highlights the challenges of managing pregnancy in women with ES and complex congenital heart defects like DORV. The successful vaginal delivery emphasizes the importance of a multidisciplinary approach, individualized treatment plans, and comprehensive monitoring to minimize risks. This case also demonstrates that vaginal delivery can be a viable option in selected patients when meticulously managed.

Careful planning and multidisciplinary care can lead to favorable outcomes in pregnancies complicated by ES and DORV, despite their significant risks.