Journal of Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2433-1783 Print ISSN: 2433-2720
Japanese Society of Pediatric Cardiology and Cardiac Surgery
Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Journal of Pediatric Cardiology and Cardiac Surgery 3(1): 28-34 (2019)

Case ReportCase Report

Cor Triatriatum Dexter in a Infant with a Hypoplastic Right Ventricle

1Department of Pediatrics, Yamagata University Faculty of Medicine ◇ Yamagata, Japan

2Second Department of Surgery, Yamagata University Faculty of Medicine ◇ Yamagata, Japan

受付日:2018年7月31日Received: July 31, 2018
受理日:2018年11月6日Accepted: November 6, 2018
発行日:2019年1月31日Published: January 31, 2019

Cor triatriatum dexter (CTD) is a very rare cardiac anomaly in which a membrane divides the right atrium (RA) into two chambers. Clinical presentation of CTD is slightly variable. Most patients are asymptomatic, and a lesion is discovered during echocardiography or surgery for other cardiac lesions. When accompanied with interatrial defects, patients with CTD exhibit cyanosis because of the right-to-left shunt via interatrial communication. Moreover, CTD and cyanotic congenital heart diseases require differentiation. To our knowledge, there are no reports of infants with a hypoplastic RV who were diagnosed with CTD, and there is no consistent opinion about the best treatment strategy for CTD. Here, we describe a male neonate with CTD and a hypoplastic RV having symptoms of central cyanosis soon after birth. A large membranous structure was identified in the RA and flow from the inferior vena cava was partially redirected into the left atrium (LA) via the patent foramen ovale (FO). We surgically resected a large membranous structure from the RA and partially closed the FO when the patient was 4 months old. We performed cardiac catheterization 12 months after surgery and confirmed that the RV was well-developed and had good function. We speculate that for CTD with hypoplastic RV and inter-atrial communication, resection of the Eustachian valve and partial closure of the FO may be effective treatment strategies as long as the size of the RV is considered. Further studies on RV size and intervention for the FO in such cases of CTD are needed.

Key words: Cor triatriatum dexter; embryonic right sinus venous valve persistence; cyanosis; neonate; hypoplastic right ventricle

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