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 35(2): 91-98 (2019)
doi:10.9794/jspccs.35.91

ReviewReview

左心低形成症候群に対する右室–肺動脈導管を用いたNorwood手術発想とその後の経緯Modified Norwood Procedure Using a Right Ventricle to Pulmonary Artery Conduit for Hypoplastic Left Heart Syndrome: Innovation and Transition of this New Operative Procedure

大阪母子医療センター心臓血管外科Department of Cardiovascular Surgery, Osaka Women’s and Children’s Hospital ◇ Osaka, Japan

発行日:2019年5月1日Published: May 1, 2019
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左心低形成症候群(HLHS)に対する体外循環下の第一期寛解手術は,1981年にNorwoodらが成功例を報告して以来,体動脈から肺動脈へ人工血管で短絡路(BT shunt)を作成する方法が行われてきたが,本邦における手術成績は不良であった.当時,新生児,乳児期早期に体外循環下に総肺静脈還流異常の修復を行ったright isomerismの症例において,肺動脈閉鎖でBT shuntを行った症例では術後血行動態が安定せず管理に難渋することが多かったのに対し,肺動脈狭窄または肺動脈絞扼術後の症例では術後経過が安定していて救命例が多かった.その理由の1つは,BT shuntでは体血圧の拡張期圧の低下により冠血流量が低下すること,肺血管抵抗の変動によりBT shuntの流量が変化して血行動態が不安定になることにあると考えた.そこで,われわれは右室から肺動脈へ心外導管を用いて肺血流の確保を行うNorwood手術(RV-PA Norwood)を,連続する7例のHLHSに行い全例手術生存し,術後経過が安定したものであったことを報告した.以後RV-PA Norwood法がHLHSに対する第一期寛解手術の1つの選択肢として広く行われるようになり,両方向性Glenn手術の追加を早期に行うことで本症に対する救命率が飛躍的に向上することとなった.

The stage 1 Norwood procedure, using a modified Blalock–Taussig (BT) shunt under cardiopulmonary bypass has been the standard procedure for patients with hypoplastic left heart syndrome (HLHS), with Norwood et al. first reporting successful palliation in 1981. However, the surgical results were unsatisfactory in 1992. Of the patients with right atrial isomerism who underwent repair of total anomalous pulmonary venous connection under cardiopulmonary bypass in the neonatal or early infantile period, many patients with pulmonary atresia, who received a modified BT shunt had a poor clinical outcome due to unstable hemodynamics postoperatively. However, many patients with pulmonary stenosis or post pulmonary artery banding were relatively stable hemodynamically, and survived the procedure. We suggest that the decrease in diastolic blood pressure from blood steal by the BT shunt resulted in a decrease in coronary blood flow. The decrease in pulmonary vascular resistance in the early postoperative period reinforced the increased flow through the BT shunt and ameliorated unstable hemodynamics. We report a modified Norwood procedure using a conduit between the right ventricle and pulmonary artery (RV-PA Norwood procedure). This modification led to satisfactory results and stable postoperative hemodynamics with no operative deaths in seven consecutive patients with HLHS. This modification has now become an option for the stage 1 Norwood procedure. An early bidirectional Glenn procedure following the RV-PA Norwood procedure has markedly improved the prognosis of patients with HLHS.

Key words: hypoplastic left heart syndrome; right ventricle-pulmonary artery conduit; modified Norwood procedure

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This page was last modified on 2019-05-17T19:15:30.000+09:00


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