Surgical Treatment of Double Outlet Right Ventricle with Absent Pulmonary Valve and Bronchiarctia

The patient is a female of 14‐year‐old with body weight 44 kg, who was referred to our hospital for cardiac murmur and history of exercise intolerance and recurrent respiratory tract infections. She presented cyanotic. Electrocardiogram showed right ventricle hypertrophy and right bundle branch block. The chest X‐ray showed right pulmonary artery dilation, pulmonary blood vessels decreased, heart shape enlargement and cardio/thoracic ratio at 0.52. Echocardiography and cardiac computerized tomography (CT) scan supported the diagnosis. The CT scan also showed severe left bronchial stenosis [Figure 1a]. The diagnosis was confirmed in the operation. The operation of total correction was performed under cardiopulmonary bypass with hypothermia. Operative findings included right ventricle enlargement, the aorta overriding the ventricle septal defect (VSD) more than 90%, aorta/main pulmonary artery ratio at 1:1 in diameter, right ventricular outflow tract, and the pulmonary valve annulus stenosis. The absence of pulmonary valve is presented in Figure 1b. After clamping the aorta, the cardioplegia solution was given through the aortic root. Oblique incision was made in the right ventricle outlet tract, abnormal muscles were resected, and the VSD was repaired with autologous pericardial. The right ventricular outflow tract and the main pulmonary artery were widened transannulusly using autologous pericardial with three leaflets. Inotropic drugs were used to maintain cardiac function postoperatively. The patient recovered well during 1‐year follow‐up.