Pulmonary Atresia and Intact Ventricular Septum With Ventriculo-Coronary Connection: Intraoperative Implications of Second- Stage Univentricular Palliation Guided by Transesophageal Echocardiography

PULMONARY ATRESIA with intact ventricular septum (PA-IVS) is a complex cyanotic congenital heart condition accounting for 3% of all congenital heart disease. 1 Anderson RH Anderson C Zuberbuhler JR Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum. Cardiol Young. 1991; 1: 105-113 Crossref Scopus (34) Google Scholar This malformation is characterized by lack of egress from the right ventricle (RV) into the pulmonary trunk, along with varying degrees of tricuspid valve and RV hypoplasia. Coronary circulation abnormalities, such as ventriculo-coronary connections, coronary stenosis, or atresia, also can be associated with this complex cardiac anomaly. 2 Zuberbuhler JR Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J. 1979; 41: 281-288 Crossref PubMed Scopus (98) Google Scholar ,3 Giglia TM Mandell VS Connor AR et al. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation. 1992; 86: 1516-1528 Crossref PubMed Scopus (139) Google Scholar This case report describes intraoperative implications of ventriculo-coronary connection detected by transesophageal echocardiography (TEE) in a child with PA-IVS with functionally univentricular physiology undergoing stage- 2 palliation.