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Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage

^a Division of Pediatric Cardiac Surgery, Asan Medical Center, College of Medicine, University of Ulsan, 388-1 Poongnap-dong, Songpa-gu, Seoul, Republic of Korea, 138-736
^b Pediatric Cardiology, Asan Medical Center, College of Medicine, University of Ulsan, Republic of Korea

*Corresponding author. Tel.: +82-2-3010-3589; fax: +82-2-3010-6966.

E-mail address: tjyun{at}amc.seoul.kr (T.-J. Yun).

A twenty-four-day-old girl, who was prematurely born at 36 weeks of gestation, and weighed 2.2 kg, and diagnosed with right atrial isomerism, functionally single ventricle, bilateral superior vena cava (SVC) and obstructive supracardiac total anomalous pulmonary venous drainage (TAPVD) draining to the junction between the right SVC and the right atrium, underwent a hybrid procedure in the operating room, which consisted of pulmonary artery banding, ductus ligation and stenting of the draining vein of TAPVD. Obstruction at the drainage site of TAPVD was initially relieved after stenting, but, one month after the procedure, the distal end of the stent became stenotic and she received bilateral sutureless repair of TAPVD. At postoperative seven months, she underwent bidirectional cavopulmonary shunt uneventfully, and she has been followed-up for two months in a stable state without any problem in the pulmonary venous pathway.

Key Words: TAPVD; Right atrial isomerism; Hybrid procedure