Two Uncomplicated Vaginal Deliveries in a Woman With Scimitar Syndrome: A Case Report

Scimitar syndrome is a rare, complex congenital cardiopulmonary venous anomaly characterized by anomalous drainage of the right pulmonary vein into the inferior vena cava, hypoplasia of the right lung, and dextroposition of the heart. Only a few reports mentioned perinatal outcomes in women with scimitar syndrome. We report two uncomplicated deliveries in a woman with scimitar syndrome, which was diagnosed and surgically palliated at 15 and 19 years of age, respectively. Having had dyspnea on exertion (New York Heart Association (NYHA) Class II), recurrent pneumonia, and hemoptysis, she underwent coil embolization of a systemic artery (renal artery) to the right lung and video-assisted thoracoscopic ligation of the scimitar vein at 19 years of age, which ameliorated her dyspnea on exertion (NYHA Class I). After her cardiac function was confirmed normal without pulmonary hypertension by echocardiography, she was allowed to plan a pregnancy and conceived spontaneously at 23 and 26 years of age. During these pregnancies, she underwent serial echocardiography tests, which showed her cardiac function was stable without pulmonary hypertension. She achieved uncomplicated vaginal deliveries with epidural anesthesia at term. This clinical experience highlights the importance of a multidisciplinary approach for pregnancy and delivery in women with scimitar syndrome. It also demonstrates that favorable maternal and neonatal outcomes are possible in women with surgically palliated scimitar syndrome by ligating the scimitar vein when cardiac function is stable and pulmonary hypertension is denied.