Single papillary muscle形態を呈した完全型房室中隔欠損症の1例  [in Japanese] Surgical Repair of Complete AVSD with Single Papillary Muscle  [in Japanese]

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Abstract

症例はDown症の3カ月, 男児. 胎児心エコー検査にて完全型房室中隔欠損症(c-AVSD)と診断後, 在胎38週2日2,387 gにて出生した. 生後2カ月時の術前心臓カテーテルおよび心エコー検査では一側心室低形成や共通房室弁狭窄所見は認められなかったが, 左室内の両乳頭筋サイズに著明な不均衡所見を認め, single papillary muscleが疑われた. その後房室弁逆流, 心不全所見の増強を認め, 生後3カ月, 体重3.73 kgにて心内修復術施行となった. 手術診断はRastelli A型のc-AVSD, 術中所見では左側房室弁の腱索は共通前尖後尖ともに全て単一の乳頭筋に付着しており, 通常のcleft閉鎖では心内修復術後parachute型僧帽弁(PMV)の血行動態が懸念された. このため手術ではtwo-patch methodにて欠損孔閉鎖のうえ, 左側房室弁の共通前尖後尖間にapposition zoneを形成するように留意して体表面積の正常弁口径90%にてcleft基部のみの修復を行った. 術後は一時的にNO使用と両側の胸水貯留を認めたが, 心機能および左側房室弁機能は良好に経過した. 単一乳頭筋によるparachute型左側房室弁形態を伴う完全型房室中隔欠損症では, 心内修復にあたり術後の弁狭窄を回避し良好な房室弁機能を維持するために修復方法を慎重に検討することが重要と考えられた.

We report a three-month-old male case of Down syndrome and complete atrioventricular septal defect (c-AVSD) with a single papillary muscle. After fetal diagnosis of c-AVSD, the patient was delivered normally in the 38<SUP>th</SUP> week. Cardiac catheterization and echocardiography showed Rastelli type A c-AVSD with well-balanced ventricles and trivial atrioventricular valve regurgitation. On the other hand, disproportional papillary muscles were seen in the left ventricle (LV) suggesting a functionally single papillary muscle. At the age of three months, intra-cardiac repair using the two-patch method was performed. There was only one papillary muscle in the anterolateral position of the LV, and all cords of both bridging leaflets attached to that muscle. To prevent postoperative stenosis of the left atrioventricular valve (AVV), only the base of the cleft was repaired by making exact apposition zones of both leaflets. Although treatment for pulmonary hypertension and pleural effusion was needed, the patient recovered to a stable condition after the surgery. Finally, the patient was discharged from the hospital with good LV contraction and left AVV function under normal pulmonary arterial pressure. So far, several reports mention a single papillary muscle in c-AVSD morphologically but surgical strategies concerning the left AVV cleft and papillary muscle have not been established yet. Therefore, it is important to develop an adequate procedure to prevent postoperative stenosis and regurgitation in repairing the left AVV portion.

Journal

  • Pediatric Cardiology and Cardiac Surgery

    Pediatric Cardiology and Cardiac Surgery 28(6), 315-319, 2012

    Japanese Society of Pediatric Cardiology and Cardiac Surgery

Codes

  • NII Article ID (NAID)
    130002591245
  • Text Lang
    JPN
  • ISSN
    0911-1794
  • Data Source
    J-STAGE 
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