Unraveling the complexities of double outlet right ventricle (DORV): A rare case of congenital heart disease with bidirectional shunting in a young girl

Double Outlet Right Ventricle (DORV) is a rare congenital heart defect characterized by both the aorta and pulmonary artery originating from the right ventricle. It accounts for 1%-3% of all congenital heart defects and is often associated with additional structural anomalies such as ventricular septal defects (VSDs), which exacerbate hemodynamic complications. We present a case of a 16-year-old female with DORV and 2 VSDs, leading to bidirectional shunting and subsequent pulmonary hypertension. The patient presented with progressive exertional dyspnea, central cyanosis, and clubbing of the fingers, indicative of chronic hypoxia. Echocardiography and Doppler studies confirmed the diagnosis, revealing significant bidirectional shunting and moderate pulmonary hypertension. Two distinct VSDs were identified in the interventricular septum (IVS): the first, a larger perimembranous VSD involving the inlet part with extensions toward the subaortic region, measured 11 mm × 16 mm; the second, a smaller VSD in the mid-part of the trabecular septum, measured 4 mm × 4 mm. Both VSDs permitted bidirectional shunting, predominantly left-to-right, contributing to the patient's cyanosis and pulmonary hypertension, with the larger VSD being routable to the aorta. Treatment involved stabilization with medical management to address pulmonary hypertension and preparation for corrective surgery. Timely surgical correction, including the closure of VSDs and rerouting of the ventriculo-arterial connections, is crucial for improving outcomes. Ongoing follow-up is essential to monitor for complications such as arrhythmias, right heart failure, and progression of pulmonary hypertension. The case underscores the complexity of DORV and the need for a multidisciplinary approach in its management.