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 31(3): 102-107 (2015)
doi:10.9794/jspccs.31.102

原著Original

左心低形成症候群に対する外科治療3ヶ月Norwood+両方向性Glenn手術と1ヶ月Norwood+右室–肺動脈シャント手術の比較Efficacy of the Norwood Operation with a Right Ventricle-Pulmonary Artery Shunt at 1 Month after Bilateral Pulmonary Artery Banding

1大阪府立母子保健総合医療センター小児医療部門心臓血管外科Department of Cardiovascular Surgery, Chidren's Hospital, Osaka Medical Center and Research Institute for Maternal and Child Health ◇ 〒594-1101 大阪府和泉市室堂町840番地840 Murodo-cho, Izumi-shi, Osaka 594-1101, Japan

2大阪府立母子保健総合医療センター小児医療部門小児循環器科Department of Pediatric Cardiology, Chidren's Hospital, Osaka Medical Center and Research Institute for Maternal and Child Health ◇ 〒594-1101 大阪府和泉市室堂町840番地840 Murodo-cho, Izumi-shi, Osaka 594-1101, Japan

受付日:2014年11月14日Received: November 14, 2014
受理日:2015年3月20日Accepted: March 20, 2015
発行日:2015年5月1日Published: May 1, 2015
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目的:左心低形成症候群(HLHS)に対し,我々は,2004年以降,両側肺動脈絞扼術(BPAB)を先行し,生後3ヶ月でNorwood手術(NW)と両方向性Glenn手術(BDG)を行ってきたが,2012年以降はBPAB後1ヶ月でNWと右室肺動脈シャント手術(RV–PA shunt)を行う方針に変更した.目的は,この治療方針の妥当性を検討すること.

対象と方法:2004年1月から2013年12月までに当院でNWを行ったHLHS患者(類似疾患を含む)28例を対象とし,NW+BDG群(G群)22例とNW+RV–PA shunt群(S群)6例とで,手術成績を比較検討した.

結果:NW時,G群では8例(36.3%)に肺動脈形成を行い,術後急性期の肺動脈バルーン拡張術(PTPA)を20例(91.0%)に要し,病院死亡3例,BDG不成立2例(1例は病院死亡症例と同一症例),術後脳梗塞1例を認めたが,S群ではNW時の肺動脈形成や急性期PTPAを要した症例はなく,生後6~13(中央値7)ヶ月でBDGを行った.BDG時に肺動脈形成を要したのは1例のみで,術後の上大静脈(SVC)圧もG群より有意に低く,急性期PTPAも要さなかった.

結論:BPAB後1ヶ月でのNW+RV–PA shunt術は,PTPAの回避と,BDG後のSVC圧を低く保つことができ,手術成績が向上すると考えられた.

Objective: Between 2004 and 2011 in our institution, we performed the Norwood operation (NW) with bidirectional Glenn shunt (BDG) for patients with hypoplastic left heart syndrome (HLHS) at 3 months old following bilateral pulmonary artery banding (BPAB). Since 2012, we have been performsaing NW with a right ventricular-pulmonary shunt (RV–PA shunt) 1 month after BPAB in HLHS patients. The aim of this study was to evaluate the efficacy of a rapid two-stage NW operation.

Methods: Between January 2004 and December 2013, a total of 28 HLHS patients underwent NW. NW with BDG was performed in 22 patients (Group G) and NW with an RV–PA shunt was performed in 6 patients (Group S). Surgical results were compared between the groups.

Results: In Group G, 8 patients (36.3%) required pulmonary artery plasty and 20 patients (91.0%) underwent catheter intervention for pulmonary artery (PTPA). Postoperative complications comprised 3 in-hospital deaths, 2 BDG take-downs, and 1 cerebrovascular infarction. In Group S, no patients required either pulmonary artery plasty or PTPA, and BDG was performed at a median age of 7 months (range, 6–13 months). Postoperative pressure in the superior vena cava (SVC) was significantly lower in Group S.

Conclusions: NW with an RV–PA shunt 1 month after BPAB enabled avoidance of PTPA and maintenance of a low SVC pressure following BDG, and was thought to improve surgical outcomes.

Key words: hypoplastic left heart syndrome; norwood operation; bilateral pulmonary artery banding; bidirectional glenn shunt; right ventricular–pulmonary artery shunt

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