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
Pediatric Cardiology and Cardiac Surgery 34(4): 207-211 (2018)

症例報告Case Report

心房壁フラップを用いて左心房直接還流型の左上大静脈再建を行った不完全型房室中隔欠損症の一例Reconstruction Using a Right Atrial Wall Flap for a Case of Left Superior Vena Cava Draining into the Left Atrium with an Incomplete Atrioventricular Septal Defect

1福島県立医科大学医学部心臓血管外科Department of Cardiovascular Surgery, Fukushima Medical University ◇ Fukushima, Japan

2福島県立医科大学医学部小児科Department of Pediatrics, Fukushima Medical University ◇ Fukushima, Japan

受付日:2018年4月17日Received: April 17, 2018
受理日:2018年7月10日Accepted: July 10, 2018
発行日:2018年12月20日Published: December 20, 2018

左上大静脈(left superior vena cava: LSVC)が左側心房に直接還流する場合の二心室修復ではLSVCの対処が必要となるが,血行再建の明確な基準はなく確立した再建方法もない.左側心房直接還流型の左上大静脈を有する不完全型房室中隔欠損症に対し,心内修復に加えて右側心房壁フラップを用いたLSVC再建を行った.症例は10か月,体重8.8 kgの女児で,チアノーゼと心不全を来し入院した.診断は両側上大静脈,単心房,不完全型房室中隔欠損症,左側房室弁逆流,動脈管開存症であった.血管造影上はLSVC優位であり,バルーンカテーテルを用いたLSVC閉塞試験では,圧が6 mmHgから38 mmHgへと上昇したため,単純結紮は危険と判断し,再建する方針とした.心内修復後に右側心房前壁をLSVC方向にフラップ状に切開のうえ展開し,LSVC後壁を作成した.前側は新鮮自己心膜を用いて再建した.術後LSVC圧の上昇はなく経過順調だった.右側心房壁フラップを用いたLSVC再建は一つの方法であると考えられた.

Reconstruction of the left superior vena cava (LSVC) is required in biventricular repair when the LSVC drains directly into the left atrium. However, the criteria for reconstruction and a standard method have not been established. We report a case of successful concomitant intra-cardiac repair and LSVC reconstruction using the right atrial wall flap. A 10-month-old girl with a common atrium, an incomplete atrioventricular septal defect, left atrioventricular valve regurgitation, and patent ductus arteriosus presented with severe cyanosis and heart failure. Preoperative angiography showed each superior vena cava (SVC) draining directly into the ipsilateral atrium, with the LSVC larger than the right SVC. With balloon catheter occlusion of the LSVC, the pressure in the LSVC increased from 6 mmHg to 38 mmHg, so simple ligation of the LSVC was considered to be high risk. After intra-cardiac repair, the posterior wall of the LSVC was reconstructed using an atrial wall flap created from the anterior wall of the right side atrium. The anterior wall of the LSVC was enlarged using a fresh autologous pericardial patch. The postoperative recovery was uneventful, with no venous congestion. Reconstruction using an atrial wall flap offers an alternative surgical method for treating patients with a common atrium into which the LSVC drains directly into the left side.

Key words: persistent left superior vena cava; congenital heart disease; atrial wall flap repair; incomplete atrioventricular septal defect

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