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 36(3): 263-268 (2020)
doi:10.9794/jspccs.36.263

症例報告Case Report

混合型総肺静脈還流異常症修復術後に遺残垂直静脈を介する症候性門脈体循環シャントを来した1例Symptomatic Portosystemic Shunt via the Unligated Vertical Vein Following Repair of a Mixed-Type Total Anomalous Pulmonary Venous Connection: A Case Report

1兵庫県立こども病院循環器内科Departments of Cardiology, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

2兵庫県立こども病院小児集中治療科Pediatric Intensive Care Medicine, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

3兵庫県立こども病院心臓血管外科Cardiovascular Surgery, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

受付日:2019年10月21日Received: October 21, 2019
受理日:2020年3月26日Accepted: March 26, 2020
発行日:2020年10月1日Published: October 1, 2020
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機能的単心室を有する無脾症児の総肺静脈還流異常症(TAPVC)に対する修復術では,肺静脈還流の形態により術後肺静脈閉塞のリスクを考慮して,垂直静脈を処理せずに修復する術式を選択する場合がある.今回,混合型TAPVC修復術後の遺残垂直静脈を介して症候性門脈体循環シャント(PSS)を来した稀な症例を経験したので報告する.症例は混合型TAPVCの無脾症男児で,日齢18にTAPVC修復術を施行した.左上大静脈に還流する右肺静脈と,門脈に還流する左肺静脈を別々に心房に吻合し,左右の垂直静脈はいずれも放置した.術後に肝逸脱酵素上昇,凝固能異常,低血糖,高アンモニア血症を認め,遺残垂直静脈をシャント血管とする症候性PSSと診断した.症状は保存的治療で改善したが,将来,Fontan循環に向かう血行動態への影響を考慮し,遺残垂直静脈絞扼術を施行した.門脈への異常還流を伴う混合型TAPVCでは,単心室血行動態において遺残垂直静脈を介したPSSを来しうるため,これを念頭においた術前精査や術式検討,周術期管理が必要である.

Mixed-type total anomalous pulmonary venous connection (TAPVC) with univentricular circulation has a wide variation of pulmonary venous anatomy and drainage. Surgical intervention for mixed-type TAPVC, including management of the vertical vein, remains controversial. Herein, we report a rare case of symptomatic portosystemic shunt via the unligated vertical vein following repair of the mixed-type TAPVC. An 18-day-old male neonate with a mixed-type TAPVC and asplenia underwent surgical repair of the TAPVC. The right vertical vein draining to the superior vena cava, and the left vertical vein draining to the portal vein were left patent. Elevated liver enzymes, coagulation abnormalities, hyperammonemia, and hypoglycemia were detected following surgery. Echocardiography revealed a portosystemic shunt draining from the portal vein to the left-sided atrium via the left unligated vertical vein. Elevated atrial pressure was detected on cardiac catheterization, and as a result, we performed banding of the left unligated vertical vein. This case highlights the need to discuss the portosystemic shunt as a postoperative complications in mixed-type TAPVC with univentricular circulation when considering both the surgical procedure and postoperative management.

Key words: total anomalous pulmonary venous connection; unligated vertical vein; portosystemic shunt; asplenia

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This page was last modified on 2020-10-19T13:56:25.000+09:00


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