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 32(3): 208-212 (2016)

症例報告Case Report

肺動脈幹温存法Norwood変法を行い改善した,左心低形成症候群に合併する気管支軟化症の一例Modified Norwood Procedure (Pulmonary Artery Trunk-Saving Method) for Tracheomalacia in a Patient with Hypoplastic Left Heart Syndrome

1三重大学大学院医学系研究科胸部心臓血管外科Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine ◇ Mie, Japan

2三重大学大学院医学系研究科小児科Department of Pediatrics, Mie University Graduate School of Medicine ◇ Mie, Japan

受付日:2015年12月20日Received: December 20, 2015
受理日:2016年2月16日Accepted: February 16, 2016
発行日:2016年5月1日Published: May 1, 2016

気管支軟化症は先天性心疾患に合併することが稀にあり,症状に応じて大動脈胸骨固定術や外ステント術等治療を必要とする疾患である.今回われわれは左心低形成症候群に合併した気管支軟化症に対して新しい手技として肺動脈幹を温存するNorwood変法手術を導入し,気管支軟化症の改善に有効であった症例を経験したので報告する.5ヶ月女児,HLHSの診断で日齢12日に両側肺動脈絞扼術を行っている.4ヶ月時に呼吸器症状から気管支鏡検査を施行し,左気管支軟化症と診断された.手術はNorwood,BDG手術を施行し,大動脈再建には肺動脈幹温存法(PA trunk saving法:PATS)を用いた.主肺動脈から左右肺動脈を起始部で離断,端々吻合し,離断部を直接閉鎖することで大動脈再建に利用できる主肺動脈長軸距離を確保し,短軸径の縮小で左肺門部狭窄を回避した.術後に呼吸器症状は消失し,気管支鏡検査でも気管枝内腔形状の改善を認めた.

Tracheomalacia is a complex anomaly associated with congenital heart disease. Surgery is required for patients with severe symptomatic tracheomalacia. Reported here is a case of hypoplastic left heart syndrome (HLHS) complicated by tracheomalacia with symptoms that improved after the patient underwent a modified Norwood operation involving a novel pulmonary artery trunk-saving procedure. A 5-month-old girl with HLHS underwent bilateral pulmonary artery banding at 12 days of age. Bronchoscopy was performed owing to respiratory distress, and tracheomalacia was diagnosed at 4 months. At 5 months of age, a Norwood and bidirectional Glenn procedure was scheduled for her cardiac malformation. The procedure consisted of dividing both pulmonary arteries at the base of the main pulmonary artery trunk, closing the branch defect directly, and end-to-end anastomosis of both pulmonary arteries to utilize the main pulmonary artery trunk tissue for aortic reconstruction longitudinally and to make circumstances small for the room of the left pulmonary hilum. Postoperatively, her respiratory distress was resolved and bronchoscopy demonstrated an improved tracheal shape.

Key words: tracheomalacia; HLHS; modified Norwood; PA trunk saving method

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