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
Pediatric Cardiology and Cardiac Surgery 36(3): 263-268 (2020)

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


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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