日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Tracheomalacia is a complex anomaly associated with congenital heart disease. Surgery is required for patients with severe symptomatic tracheomalacia. Reported here is a case of hypoplastic left heart syndrome (HLHS) complicated by tracheomalacia with symptoms that improved after the patient underwent a modified Norwood operation involving a novel pulmonary artery trunk-saving procedure. A 5-month-old girl with HLHS underwent bilateral pulmonary artery banding at 12 days of age. Bronchoscopy was performed owing to respiratory distress, and tracheomalacia was diagnosed at 4 months. At 5 months of age, a Norwood and bidirectional Glenn procedure was scheduled for her cardiac malformation. The procedure consisted of dividing both pulmonary arteries at the base of the main pulmonary artery trunk, closing the branch defect directly, and end-to-end anastomosis of both pulmonary arteries to utilize the main pulmonary artery trunk tissue for aortic reconstruction longitudinally and to make circumstances small for the room of the left pulmonary hilum. Postoperatively, her respiratory distress was resolved and bronchoscopy demonstrated an improved tracheal shape.