A Case of Ventricular Septal Defect and Atrial Septal Defect in an Infant with Right Lung Aplasia / 日本心臓血管外科学会雑誌

ABSTRACT

This paper describes a very rare case of both ventricular septal defect (VSD) and atrial septal defect (ASD) associated with pulmonary hypertension (PH) successfully repaired via a right thoracotomy in infant with right lung aplasia. A 4-month old infant was admitted to our hospital because of congenital heart disease and right lung abnormalities. Roentogenograms revealed complete opacity of the right hemithorax, with a shift of the mediastinum and the heart to the right. Computed tomography of the chest showed the absence of the right lung and a right bronchus remnant. Therefore, a dignosis of aplasia of the right lung was made at this point. Echocardiogram confirmed VSD and ASD, both of which were 5-mm in diameter, and associated with PH. At the age of 1 year and 7 months, cardiac catheterization was performed, showing pulmonary hypertension with a systolic pulmonary-to-systemic pressure ratio (P_p/P_s) of 0.66. Tolazoline hydrochloride decreased pulmonary vascular resistance (R_p) from 6.92 units·m² to 3.11 units·m². The operation, under cardiopulmonary bypass, was performed via a right thoracotomy approach, because of severe counterclockwise rotation of the heart. VSD and ASD were closed by primary suturing. This approach offered excellent exposure of the intracardiac anatomy in our case. An intraoperative pressure study showed normal pulmonary arterial pressure, the P_p/P_s decreased to 0.33. The postoperative course was uneventful.