Successful Staged Fontan Completion for Double-outlet Right Ventricle with Intact Ventricular Septum

Shintaro Okazaki; Koichi Nishida; Satoshi Tamura; Yasunori Ishihara; Satoshi Taniguchi; Masaaki Yamagishi

doi:10.24509/jpccs.170113

Successful Staged Fontan Completion for Double-outlet Right Ventricle with Intact Ventricular Septum

Shintaro Okazaki¹，Koichi Nishida¹，Satoshi Tamura²，Yasunori Ishihara³，Satoshi Taniguchi⁴，Masaaki Yamagishi⁵Shintaro Okazaki¹, Koichi Nishida¹, Satoshi Tamura², Yasunori Ishihara³, Satoshi Taniguchi⁴, Masaaki Yamagishi⁵

¹ Department of Pediatrics, Fukui Cardiovascular Center ◇ Fukui, Japan

² Department of Pediatrics, Municipal Tsuruga Hospital ◇ Fukui, Japan

³ Department of Pediatrics, Fukui Aiiku Hospital ◇ Fukui, Japan

⁴ Department of Cardiovascular Surgery, Fukui Cardiovascular Center ◇ Fukui, Japan

⁵ Department of Pediatric Cardiovascular Surgery, Children’s Medical Center, Kyoto Prefectural University of Medicine ◇ Kyoto, Japan

受付日：2017年2月8日Received: February 8, 2017

受理日：2017年4月11日Accepted: April 11, 2017

発行日：2017年7月1日Published: July 1, 2017

Double-outlet right ventricle with intact ventricular septum is an extremely rare heart malformation. To our knowledge, only 42 cases have been reported, and there is no previous case report achieving Fontan completion. The diagnosis was made by echocardiography and contrast-enhanced cardiac computed tomography. Our patient had additional interesting anomalies such as sinusoidal communications. We describe a successful case of staged Fontan completion for double-outlet right ventricle with intact ventricular septum.

Key words: double-outlet right ventricle; intact ventricular septum; Fontan completion; sinusoidal communication; balloon atrial septostomy

Journal of Pediatric Cardiology and Cardiac Surgery

Case ReportCase Report

Successful Staged Fontan Completion for Double-outlet Right Ventricle with Intact Ventricular Septum