Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum.
This report describes the case of a full-term gestational female with a prenatal diagnosis of pulmonary atresia with intact ventricular septum. Cardiac ultrasound at birth confirmed the diagnosis with no evidence of coronary artery fistulas. The patient died 6 hours after a central aortic to pulmonary artery shunt had been created with bypass support. Postmortem examination showed a coronary artery fistula rising from the right ventricle and a complete absence of both coronary ostia. This rare finding has been reported sporadically in the English literature.
Lajos
Paul
P
Department of Surgery, University of Maryland Medical System, Baltimore, Maryland, USA.
Love
Jon
J
Salim
Mubadda A
MA
Wang
Wenle
W
Cardarelli
Marcelo G
MG
D003330:Coronary Vessel Anomalies / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D005402:Fistula / Q000151:congenital; D006346:Heart Septum; D006801:Humans; D007231:Infant, Newborn; D018633:Pulmonary Atresia / Q000150:complications*