2023 年 7 巻 1 号 p. 31-35
Isolated pulmonary artery of ductal origin (IPADO) is a rare congenital cardiovascular disease. We describe two patients with IPADO who were successfully treated with staged repair. The first patient with left IPADO and a closing duct underwent left original Blalock–Taussig shunt at 2 months of age, then, at 11 months old, the left pulmonary artery (PA) was connected to the main PA utilizing the subclavian arterial tissues with anterior augmentation placing an autologous pericardial patch. Right-to-left lung perfusion ratio was 2.05 postoperatively. The second patient with right IPADO and a closed duct underwent right modified Blalock–Taussig shunt (a 3 mm expanded polytetrafluoroethylene tube) at 29 days of age. The right PA was connected to the main PA at 5 months of age using a flap of the main PA wall and an autologous pericardial patch for anterior augmentation. Postoperative right-to-left lung perfusion ratio was 1.14. Staged repair provided balanced distribution of blood flow to the lungs. Reconstruction of the affected PA with the patient’s own vascular tissues, instead of prosthetic materials, was feasible, promising lifetime potential of growth.
