Right Ventricular Outflow Tract Obstruction in a Pregnant Woman Caused by Double Chambered Right Ventricle

Background: Congenital heart diseases (CHD) with obstruction of the right ventricular outflow tract can often be difficult to diagnose. Pregnancies present a relevant challenge when deciding on treatment options in this patient population.

Case: We report a case of a 15 weeks pregnant woman aged 34 years who presented with relevant shortness of breath at rest (NYHA IV) and prominent systolic murmur. MR imaging revealed right ventricular outflow tract obstruction with suprasystemic right-sided pressure gradients of 130 mm Hg due to infundibular muscular right ventricular outflow tract obstruction (RVOT) in double-chambered right ventricle. Hypertrophy of the muscular tissue could be documented. After interdisciplinary discussion a surgical approach on cardiopulmonary bypass under close fetal monitoring with NIRS and ultrasound was attempted. Via a median sternotomy the surplus muscular masses and fibrotic structures were resected and a pericardial patch for augmentation of the outflow tract implanted. The patient was discharged home 7 days later with an excellent result and significant reduction of the RVOT gradient to 25 mm Hg. CTG and sonography showed a vivid fetus without signs for intraventricular bleeding.

Discussion: During pregnancy cardiac procedures pose a high risk for both the mother and the unborn child. Even in the first trimester of pregnancy cardiac procedures can be safely performed when taking precautious steps for both the mother and the fetus. This case emphasizes the importance of a detailed perioperative evaluation of adults presenting with manifestation of CHD and the interdisciplinary approach with obstetricians, cardiologists and cardiac surgeons when treating patients during pregnancy.