[Total Repair of Pulmonary Atresia, Ventricular Septal Defect, and Major Aorto-pulmonary Collateral Arteries That were Deemed Unrepairable in an Adolescent and Adults].

Pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries are deemed unrepairable in some patients. Reportedly, 39% to 63% of patients treated by the staged unifocalization pathway, and 25% to 51% of patients treated by the pulmonary artery rehabilitation strategy were deemed unsuitable to undergo total repair. Single-stage complete unifocalization with intracardiac repair can be performed in some older patients with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable. We report four such patients with ages between 12 years and 28 years at surgery. Bosentan hydrate was administered pre-operatively in one female patient for 14 months to ameliorate regional pulmonary hypertension. Seventeen months after repair, she delivered a baby by cesarean section. One patient underwent aortic valve replacement concomitant with repair due to severe aortic regurgitation caused by healed infective endocarditis. All patients were discharged home 9 to 18 days after surgery. After 72 to 100 months of follow-up, three patients are in New York Heart Association (NYHA) class Ⅰ, and the remaining patient are in NYHA class Ⅱ. Single-stage complete unifocalization can allow total repair in adolescents and adults with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable.