心室中隔欠損を伴う右肺動脈上行大動脈起始症の低出生体重児に対する段階的外科修復 —根治術後肺動脈吻合部狭窄の解除に工夫を要した1例— Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis

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<p>症例は在胎35週1,561 gで出生した男児.生直後の心エコーで右肺動脈上行大動脈起始症(AORPA)および膜性部心室中隔欠損症(VSD)と診断された.急速に心不全が進行したが,低体重のため日齢14に右肺動脈絞扼術を先行し,体重が増加した5カ月後に根治術を行った.右肺動脈の再建には主肺動脈右側壁をflap状に用いたが,術後に上行大動脈による圧排と瘢痕形成のため高度の右肺動脈狭窄をきたした.再手術では上行大動脈をいったん離断して右肺動脈狭窄部をパッチ形成した後,圧迫を回避するために上行大動脈後壁をパッチで延長して大動脈背側の空間を拡大した.上行大動脈前壁の一部は成長を期待して自己組織同士で吻合した.VSDを伴うAORPAの低出生体重児に対する外科修復の報告は稀であり,右肺動脈吻合部狭窄解除術の工夫を中心に報告した.右肺動脈形態や左肺高血圧の推移について,長期にわたる慎重な経過観察を要する.</p>

<p>A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.</p>

収録刊行物

  • 日本心臓血管外科学会雑誌

    日本心臓血管外科学会雑誌 47(5), 207-210, 2018

    特定非営利活動法人 日本心臓血管外科学会

各種コード

  • NII論文ID(NAID)
    130007496075
  • 本文言語コード
    JPN
  • ISSN
    0285-1474
  • データ提供元
    J-STAGE 
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