[Surgical Strategy for Pulmonary Atresia with Ventricular Septal Defect with Major Aortopulmonary Collateral Artery]

Although the characteristics and pathophysiology of major aortopulmonary collateral artery (MAPCA) have been better understood, treatment of pulmonary atresia and ventricular septal defect with MAPCA remains challenging. The difficulty in treating this disease is the lack of availability of pulmonary homografts required for pulmonary angioplasty in Japan and the need for manipulation of the posterior mediastinum region, which is not performed in normal cardiac surgery. It is important to build an appropriate surgical strategy by means of preoperative computed tomography( CT) and selective angiography. For treatment, unifocalization or rehabilitation is selected depending on the condition of MAPCA and central pulmonary artery. Because unifocalization is highly invasive, it would be performed at 6 months of age and weigh 5 kg. As a procedure, extensive dissection is performed, and anastomoses between tissue to tissue are basically performed, and reconstruction of the right ventricular outflow tract is performed using a valved conduit that can secure anterograde pulmonary blood flow that is not affected by the condition of the lung. It is effective to confirm postoperative lung perfusion scan and CT, perform a cardiac catheterization test approximately 6 months after surgery, and maintain the pulmonary vessels with catheter intervention if necessary. The ultimate goal of treating this disease is not only to improve cyanosis, but also to maintain pulmonary circulation at lower right ventricular pressure.