第21巻 2025年8月　第3号

■症例報告

肺動脈弁下膜様狭窄を伴った成人両大血管右室起始の1例
平山　愛子¹, 佐々木　孝¹, 網谷　亮輔¹, 上田　仁美¹, 宮城　直人¹, 山下　裕正¹, 丸山　雄二¹, 宮城　泰雄¹, 功刀　しのぶ², 石井　庸介^1
1日本医科大学付属病院心臓血管外科
²日本医科大学付属病院病理診断科

An Adult Case of Double Outlet Right Ventricle and Right Ventricular Outflow Tract Stenosis Caused by Subpulmonary Membrane
Aiko Hirayama¹, Takashi Sasaki¹, Ryosuke Amitani¹, Hitomi Ueda¹, Naoto Miyagi¹, Hiromasa Yamashita¹, Yuji Maruyama¹, Yasuo Miyagi¹, Shinobu Kunugi² and Yosuke Ishii^1
1)Department of Cardiovascular Surgery, Nippon Medical School Hospital
²⁾Department of Analytic Human Pathology, Nippon Medical School Hospital

We report a rare case of surgery for double-outlet right ventricle (DORV) and right ventricular outflow tract (RVOT) stenosis in adulthood; we also present a review of the literature. A 65-year-old man who had been diagnosed with a ventricular septal defect when he was a child and had undergone catheter ablation for paroxysmal atrial fibrillation at 57 years of age presented to our hospital with exertional fatigue. Echocardiography revealed DORV, a ventricular septal defect (VSD), and RVOT stenosis caused by subpulmonary membrane. Cardiac catheterization revealed a pulmonary to systemic flow ratio (Qp/Qs) of 2.7, a mean pulmonary artery pressure of 42 mmHg, a pulmonary vascular resistance (Rp) of 5.25 unit・m², and a pressure gradient between RVOT and the right ventricular inlet of 30 mmHg. Right ventricular pressure was 80% of left ventricular pressure, and pulmonary hypertension with increased pulmonary blood flow was observed. During surgery, the patient underwent VSD closure and baffling of the left ventricle to the aorta, resection of the subpulmonary membrane in the right ventricular outflow tract, and closure of the left atrial appendage. The postoperative course was uneventful. The patient was extubated on the first postoperative day, transferred to the general ward on the sixth day, and discharged on the seventeenth day. Postoperative echocardiography indicated that the RVOT stenosis had improved, and that right ventricular pressure had decreased to less than 50% of the left ventricular pressure.

日医大医会誌 2025; 21(3), 258-264

Key words
double outlet right ventricle, right ventricular outflow tract stenosis, adult congenital heart disease, subpulmonary membrane

Correspondence to
Takashi Sasaki, Department of Cardiovascular Surgery, Nippon Medical School Hospital, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
E-mail：t-sasaki0@nms.ac.jp

受付：2025年2月17日　受理：2025年4月11日