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): 252-255 (2020)

症例報告Case Report

膜様部中隔瘤による右室流出路狭窄を呈した心室中隔欠損の一例Right Ventricular Outflow Obstruction by a Membranous Septal Aneurysm in a Ventricular Septal Defect

1近畿大学医学部小児科学教室Department of Pediatrics, Kindai University Faculty of Medicine ◇ Osaka, Japan

2近畿大学医学部心臓血管外科学教室Department of Cardiovascular Surgery, Kindai University Faculty of Medicine ◇ Osaka, Japan

受付日:2019年12月5日Received: December 5, 2019
受理日:2020年3月12日Accepted: March 12, 2020
発行日:2020年10月1日Published: October 1, 2020

巨大な膜様部中隔瘤による右室流出路狭窄を合併した心室中隔欠損の一例を経験した.症例は5歳の女児で,出生時に心雑音を聴取し,心房中隔欠損,膜性部心室中隔欠損,大動脈縮窄症と診断した.生後51日に短絡量が少ない心房中隔欠損と心室中隔欠損は経過観察とし大動脈縮窄の手術を行っている.4歳時の健診で心室中隔から右室流出路に張り出した嚢状の膜様部中隔瘤を認め,心臓カテーテル検査を施行,肺体血流比は1.0であったが右室収縮期圧は79 mmHg,肺動脈収縮期圧26 mmHgと右室流出路狭窄を認めたため,心室中隔欠損に対してパッチ閉鎖術を施行した.膜様部中隔瘤が右室流出路狭窄を来す成人例の報告はあるが,幼少期には稀である.短絡量が少なく経過観察としている心室中隔欠損にもこのような合併症が生じることを考慮し,定期的にフォローアップする必要性がある.

We report the case of a 5-year-old girl with a ventricular septal defect (VSD) complicated by stenosis of the right ventricular outflow tract due to a giant membranous septal aneurysm. The patient had a heart murmur at birth, and was diagnosed with membranous VSD and aortic coarctation. At 3 months old, the VSD showed signs of spontaneous closure and a mild left-to-right (LR) shunt was observed. Therefore, surgery for aortic constriction was performed as a follow-up for the VSD. At 4 years old, echocardiography revealed a giant membranous septal aneurysm protruding into the right ventricular outflow tract, and cardiac catheterization was performed. The Qp/Qs, right ventricular systolic pressure and pulmonary artery systolic pressure were 1.0, 79 mmHg and 26 mmHg, respectively, and right ventricular outflow stenosis was revealed. Although adult cases of membranous septal aneurysms that result in right ventricular outflow stenosis have been reported, pediatric cases are rare. Given such complications, careful observation is necessary for VSDs with a mild LR shunt.

Key words: membranous septal aneurysm; ventricular septal defect; right ventricular obstruction

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