title: Hypoplastic left heart syndrome and pulmonary veno-occlusive disease in an infant.
abstract: This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.
authors.affiliation: Pediatric Cardiology, University of Virginia, 1215 Lee Street, Box 800386, Charlottesville, VA, 22908-0386, USA, mdd3e@virginia.edu.
authors.forename: Marise
authors.initials: M
authors.lastname: D'Souza
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Vergales
authors.forename: K Anitha
authors.initials: KA
authors.lastname: Jayakumar
mesh_terms: D000015:Abnormalities, Multiple; D000792:Angiography; D017809:Fatal Outcome; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis*; D007231:Infant, Newborn; D008297:Male; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis*; D013902:Radiography, Thoracic