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Ann Thorac Surg 2004;77:1087-1088
© 2004 The Society of Thoracic Surgeons

Case report

Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum

Paul Lajos, MDa, Jon Love, MDb, Mubadda A. Salim, MDa, Wenle Wang, MDc, Marcelo G. Cardarelli, MDa*

a Department of Surgery, University of Maryland Medical System, Baltimore, Maryland, USA
b Department of Pediatrics, University of Maryland Medical System, Baltimore, Maryland, USA
c Department of 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: mcard001{at}umaryland.edu

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.




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K. J. Guleserian, L. B. Armsby, R. R. Thiagarajan, P. J. del Nido, and J. E. Mayer Jr
Natural History of Pulmonary Atresia With Intact Ventricular Septum and Right-Ventricle-Dependent Coronary Circulation Managed by the Single-Ventricle Approach
Ann. Thorac. Surg., June 1, 2006; 81(6): 2250 - 2258.
[Abstract] [Full Text] [PDF]


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