Surgical Reconstruction with Autologous Tissue in a Case of Isolated Unilateral Absence of the Right Pulmonary Artery / 日本心臓血管外科学会雑誌

ABSTRACT

Isolated unilateral absence of the right pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful surgical reconstruction with autologous tissue of this anomaly. The patient was a 1-month-old boy who had been transferred to our center at 3 days of age because of tachypnea and heart murmur. Multi-detector CT and radial angiography imaging revealed isolated unilateral absence of the right pulmonary artery and left patent ductus arteriosus. Conservative treatment did not help his progressive heart failure and pulmonary hypertension due to an acute increase of pulmonary blood flow. Therefore surgical correction was determined to avoid the worsening of those symptoms. Under cardiopulmonary bypass, the right pulmonary artery branching off from the brachiocephalic artery was removed and anastomosed to the main pulmonary artery with an autologous pericardium roll. Symptoms improved postoperatively and he was discharged in good condition on the 21st of postoperative day. Cardiac catheterization, 3 months later, showed excellent results.