PMID- 31266911 OWN - NLM STAT- MEDLINE DCOM- 20190711 LR - 20190711 IS - 0021-5252 (Print) IS - 0021-5252 (Linking) VI - 72 IP - 4 DP - 2019 Apr TI - [Total Repair of Pulmonary Atresia, Ventricular Septal Defect, and Major Aorto-pulmonary Collateral Arteries That were Deemed Unrepairable in an Adolescent and Adults]. PG - 263-268 AB - 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. FAU - Kaneko, Yukihiro AU - Kaneko Y AD - Division of Cardiovascular Surgery, National Medical Center for Children and Mothers, Tokyo, Japan. FAU - Achiwa, Ikuya AU - Achiwa I FAU - Kondo, Ryouichi AU - Kondo R FAU - Takei, Tetsuri AU - Takei T LA - jpn PT - Journal Article PL - Japan TA - Kyobu Geka JT - Kyobu geka. The Japanese journal of thoracic surgery JID - 0413533 SB - IM MH - Adolescent MH - Adult MH - Cesarean Section MH - Child MH - Collateral Circulation MH - Female MH - *Heart Defects, Congenital/surgery MH - *Heart Septal Defects, Ventricular/surgery MH - Humans MH - Pregnancy MH - Pulmonary Artery MH - *Pulmonary Atresia/surgery MH - Young Adult EDAT- 2019/07/04 06:00 MHDA- 2019/07/12 06:00 CRDT- 2019/07/04 06:00 PHST- 2019/07/04 06:00 [entrez] PHST- 2019/07/04 06:00 [pubmed] PHST- 2019/07/12 06:00 [medline] PST - ppublish SO - Kyobu Geka. 2019 Apr;72(4):263-268.