Prenatal Identification, Transitional Hemodynamic Remodeling, and Multidisciplinary Management of Ebstein's Anomaly with Coexistent Urogenital Malformations: An Observational Analytic Case Study

Abstract

Background: Ebstein’s anomaly is an uncommon congenital heart defect with failed tricuspid delamination, right-ventricular atrialization, and severe regurgitation. Prenatal detection is difficult on routine obstetric ultrasonography, and perinatal hemodynamic transition carries substantial mortality. Objective: To document the prenatal identification, transitional neonatal hemodynamic remodeling, and multidisciplinary management of Ebstein’s anomaly with coexistent urogenital malformation. Methods: A 28-year-old multigravida referred at 34–36 weeks with a suspected cardiac anomaly was followed through multidisciplinary re-evaluation, term cesarean delivery, neonatal intensive care, and 3-month follow-up, with serial hemodynamic profiling and risk stratification. Results: Initial referral ultrasound suspected double outlet right ventricle, which was revised to Ebstein’s anomaly (Carpentier Type B) with dilated pulmonary trunk and coexistent bilateral testicular hydrocele and hypospadias upon joint fetomaternal-pediatric cardiology assessment. Delivery at 38–39 weeks yielded a male neonate (3,050 g, Apgar 5/7, SpO2 85%). Postnatal echocardiography confirmed Ebstein’s anomaly with moderate-to-severe tricuspid regurgitation (peak pressure gradient 78 mmHg), a 3–4 mm atrial septal defect with right-to-left shunting, and a 3 mm patent ductus arteriosus with left-to-right shunting. Conservative medical stabilization utilizing non-invasive continuous positive airway pressure, oral captopril, and furosemide averted emergency neonatal cardiac surgery. At 3 months, serial echocardiography revealed spontaneous ductal closure, reduction of tricuspid pressure gradient to 21 mmHg (Δ% = -73.1%), shunt reversal to left-to-right, and complete hydrocele resolution. Conclusion: Late third-trimester diagnostic conversion highlights the need for structured fetal echocardiography beyond four-chamber views. Favorable outcomes in severe Ebstein’s anomaly are achievable through coordinated multidisciplinary care, where falling pulmonary vascular resistance drives non-operative hemodynamic remodeling.