title: Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle.
abstract: We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,The Perelman School of Medicine at the University of Pennsylvania,Philadelphia,Pennsylvania,United States of America.
authors.forename: Shaun
authors.initials: S
authors.lastname: Mohan
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,The Perelman School of Medicine at the University of Pennsylvania,Philadelphia,Pennsylvania,United States of America.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Weinberg
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,The Perelman School of Medicine at the University of Pennsylvania,Philadelphia,Pennsylvania,United States of America.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
mesh_terms: D017023:Coronary Angiography; D054326:Coronary Sinus / Q000601:surgery*; D004452:Echocardiography; D005402:Fistula / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging*; D054088:Ventricular Septum / Q000601:surgery*
keywords: Pulmonary atresia; coronary cameral fistulae; intact septum