ニイナミ　ヒロシ   NIINAMI Hiroshi   新浪　博    所属   医学部 医学科（東京女子医科大学病院）    職種   教授・基幹分野長
言語種別	英語
発表タイトル	The Outcome of Surgical Repair for Congenital Unilateral Absence of the Proximal Pulmonary Artery Contralateral to the Aortic Arch
会議名	The 26th Annual Meeting of the Asian Society for Cardiovascular and Thoracic Surgery (ASCVTS 2018 MOSCOW)
主催者	The Asian Society for Cardiovascular and Thoracic Surgery
学会区分	国際学会及び海外の学会
発表形式	口頭
講演区分	一般
発表者・共同発表者	◎OKUGI Satoshi, FURUTA Akihisa, MATSUMURA Goki, NIINAMI Hiroshi
発表年月日	2018/05/25
開催地 （都市, 国名）	Moscow, RUSSIA
概要	OBJECTIVES Congenital unilateral absence of the proximal pulmonary artery contralateral to the aortic arch (UAPPA) is a rare anomaly. The purpose of this retrospective study is to present our institutional surgical experiences for seven patients with UAPPA and to evaluate the affected PA growth in a diameter and the flow distribution of the PA in both lungs METHODS From 1984 to 2017, seven patients with UAPPA presented at our institution. There were three females and four males and their average age at diagnosis was 6.4±12.8 months. The diameter of the PA was measured by using catheter angiography. Paired t test was used to compare the diameter of affected PA between at the time of initial diagnosis and after intracardiac repair. The flow distribution of PA after intracardiac repair was evaluated with lung perfusion scintigraphy. RESULTS Four patients had the palliations to grow the affected PA before the intracardiac repair. The average age at palliation was 16.75±15.1 months (median 15 months). The average age at intracardiac repair was 61.6±94 months (median 10 months). One patient with truncus arteriosus died early after the intracardiac repair due to heart failure. The average diameter of the affected PA was 44.5±20.0 % of normal value at the time of the diagnosis and slightly increased to 57.6±34.2% of normal value after intracardiac repair. However, there was no significant difference. The flow distribution of ipsilateral lung to the affected PA was 21.3±23.3 % of the total lung perfusion after intracardiac repair. CONCLUSION Congenital UAPPA might have a possibility of having affected pulmonary artery developed poorly even after the surgical repair. The average 6 months at initial presentation seems to be somewhat late to develop the closed proximal PA.