title: Coronary sinus orifice atresia with left superior vena cava in patients with univentricular heart.
abstract: Two patients with hypoplastic left heart syndrome had coronary sinus orifice atresia with persistent left superior vena cava. Both patients underwent successfully coronary sinus unroofing. One underwent surgery at the time of the bidirectional Glenn procedure and the other before creation of a total cavopulmonary connection. According to our population-based database, 10.3% of patients with univentricular heart have persistent left superior vena cava, and 2.3% have associated coronary sinus orifice atresia. Our cases highlight the importance of recognizing this anomaly in patients with univentricular heart to avoid high coronary venous pressure, which is potentially lethal.
authors.affiliation: Department of Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. jukka.salminen@hus.fi
authors.forename: Jukka T
authors.initials: JT
authors.lastname: Salminen
authors.forename: Tapio
authors.initials: T
authors.lastname: Hakala
authors.forename: Jaana
authors.initials: J
authors.lastname: Pihkala
authors.forename: Ilkka
authors.initials: I
authors.lastname: Mattila
authors.forename: Juha
authors.initials: J
authors.lastname: Puntila
authors.forename: Heikki
authors.initials: H
authors.lastname: Sairanen
mesh_terms: D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000601:surgery*; D005260:Female; D006325:Heart Atria / Q000601:surgery; D018610:Heart Bypass, Right; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D007223:Infant; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities; D014683:Vena Cava, Superior / Q000002:abnormalities*