Total Right Ventricular Dependent Coronary Circulation in Pulmonary Atresia With Intact Ventricular Septum

Lajos, Paul MD
Love, Jon MD
Salim, Mubadda A MD
Wang, Wenle MD
Cardarelli, Marcelo G MD

^aDepartment of Surgery, Pediatrics, and Pathology, University of Maryland Medical System, Baltimore, Maryland, USA

Accepted for publication April 9, 2003.

Address reprint requests to Dr Cardarelli, Division of Cardiac Surgery, 22 South Greene St, Rm N4W94, Baltimore, MD, USA 21201-1595; e-mail: [email protected].

The Annals of Thoracic Surgery 77(3):p 1087-1088, March 2004. | DOI: 10.1016/S0003-4975(03)01259-1

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.