title: Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum: a case report.
abstract: Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion and accounts for 1-3% of critically ill infants with congenital heart disease. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate is still high in most series. The optimal surgical procedure depends on the size and morphology of the tricuspid valve and right ventricle and the presence or absence of right ventricle-dependent coronary circulation. Therefore, it is pivotal to define the precise morphologic and hemodynamic characteristics, especially coronary artery anatomy. In this report, we describe a full-term female neonate with cyanosis soon after birth. Two-dimensional and color Doppler echocardiography corroborated the diagnosis of PAIVS and showed a small right ventricle. Cardiac catheterization indicated PAIVS and further revealed right ventricle-dependent coronary circulation. A systemic-to-pulmonary artery shunt was constructed with a positive immediate result.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
authors.forename: Meng-Hsun
authors.initials: MH
authors.lastname: Lee
authors.forename: Jiunn-Ren
authors.initials: JR
authors.lastname: Wu
authors.forename: Zeng-Kong
authors.initials: ZK
authors.lastname: Dai
authors.forename: Jong-Hau
authors.initials: JH
authors.lastname: Hsu
authors.forename: Chau-Chi
authors.initials: CC
authors.lastname: Chou
authors.forename: Huai-Min
authors.initials: HM
authors.lastname: Chen
mesh_terms: D006328:Cardiac Catheterization; D003326:Coronary Circulation; D004562:Electrocardiography; D005260:Female; D006337:Heart Murmurs / Q000209:etiology; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D007363:Intensive Care Units, Neonatal; D018633:Pulmonary Atresia / Q000150:complications* / Q000601:surgery; D016896:Treatment Outcome