title: Extensive coronary arterial fistula development in patients with univentricular circulation.
abstract: We describe two children, each with a univentricular circulation who had evidence of elevated pulmonary arterial pressure at cardiac catheterization. Both children developed extensive coronary arterial fistulae detected at cardiac catherization. Each child was medically treated with a combination of phosphodiesterase-5 inhibitor and endothelin antagonist pulmonary vasodilators. The presence of chronic hypoxaemia, elevated filling pressures, and the use of pulmonary vasodilators may contribute to coronary endothelial dysfunction, which may result in coronary vasculopathy. This may necessitate early referral for orthotopic cardiac transplantation. © 2013 Wiley Periodicals, Inc.
authors: Department of Paediatric Cardiology, Crumlin, Dublin, 12, Ireland.
authors: Colin J
authors: CJ
authors: McMahon
authors: David
authors: D
authors: Coleman
authors: Paul
authors: P
authors: Oslizlok
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000628:therapy; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods; D003326:Coronary Circulation; D003331:Coronary Vessels / Q000000981:diagnostic imaging*; D004062:DiGeorge Syndrome / Q000175:diagnosis* / Q000628:therapy; D018450:Disease Progression; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000628:therapy; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011859:Radiography; D012494:Sampling Studies; D016157:Vascular Fistula / Q000000981:diagnostic imaging* / Q000601:surgery
keywords: coronary artery; fistula; pulmonary arterial hypertension; univentricular circulation; vasculopathy