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

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 34(1): 46-51 (2018)
doi:10.9794/jspccs.34.46

症例報告Case Report

二期的に根治した右肺動脈上行大動脈起始症の2例Two Cases of Staged Repair of Anomalous Origin of Right Pulmonary Artery from the Ascending Aorta

1九州大学病院心臓血管外科Department of Cardiovascular Surgery, Kyushu University Hospital ◇ Fukuoka, Japan

2九州大学病院小児科Department of Pediatrics, Kyushu University Hospital ◇ Fukuoka, Japan

受付日:2017年10月10日Received: October 10, 2017
受理日:2018年1月12日Accepted: January 12, 2018
発行日:2018年1月1日Published: January 1, 2018
HTMLPDFEPUB3

右肺動脈上行大動脈起始症(anomalous origin of the right pulmonary artery from ascending aorta: AORPA)は生後すぐより心不全,呼吸不全を呈し,診断後早期に根治術を行うことが一般的である.今回我々は二期的に根治術を施行した2例を経験した.症例1は日齢25の女児で,RSウイルス肺炎,呼吸不全と診断され前医より紹介された.入院時の心臓超音波検査,造影CT検査でAORPAと診断した.RSウイルス感染症であり人工心肺の使用は危険性が高いため,日齢36に右肺動脈絞扼術を行った.RSウイルス肺炎の改善を待ち生後3か月時に根治術を施行した.術後経過は良好であり,術後58日目に退院となった.症例2は1か月の女児で,1か月検診で心雑音を指摘され,精査でAORPAと診断された.術前の心臓カテーテル検査にて肺血管抵抗>10 units·m2と高度肺高血圧(pulmonary hypertension: PH)を認めており,まず右肺動脈絞扼術を行い,その13日後に根治術を施行した.術後経過は良好であり,術後25日目に退院となった.一期的根治がハイリスクな症例には,二期的手術も治療選択として有用なオプションであった.

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation. Clinical manifestations usually appear in infants or, more rarely, in newborns and include respiratory distress or congestive heart failure due to increased pulmonary resistance. Primary total correction of AORPA reportedly can result in excellent survival with a low incidence of reintervention. We report here two cases of staged repair of AORPA. Case 1 involved a 25-day-old girl who had a complicating respiratory syncytial (RS) virus infection. RS virus infection in children with congenital heart disease is associated with high mortality and morbidity, and cardiac surgery performed with cardiopulmonary bypass during symptomatic RS virus infection is associated with a high risk of postoperative complications, especially postoperative pulmonary hypertension. Therefore, we decided that the initial palliation should consist of right pulmonary artery banding, and total correction was achieved 2 months later. Case 2 involved an almost 2-month-old girl who initially underwent right pulmonary artery banding due to severe pulmonary hypertension, and total correction was achieved 13 days later. Both patients were discharged in good condition without any clinical symptoms. Thus, right pulmonary artery banding appears to be a good surgical option for patients with AORPA and complicated condition.

Key words: respiratory syncytial virus infection; anomalous origin of right pulmonary artery from the ascending aorta; right pulmonary artery banding

This page was created on 2018-02-13T10:05:57.989+09:00
This page was last modified on 2018-03-06T11:34:58.673+09:00


このサイトは(株)国際文献社によって運用されています。