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Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle

Published online by Cambridge University Press:  22 January 2016

Shaun Mohan ,
Paul M. Weinberg  and
Andrew C. Glatz
Shaun Mohan
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Paul M. Weinberg
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Andrew C. Glatz*
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Center for Clinical Epidemiology and Biostatistics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: A. C. Glatz, MD, MSCE, Department of Pediatrics, Division of Cardiology, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, 6th Floor, Main Building, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, United States of America. Tel: 215 590 1790; Fax: 215 590 5415; E-mail: glatz@email.chop.edu
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Abstract

We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.

Keywords

Pulmonary atresiaintact septumcoronary cameral fistulae
Type
Brief Reports
Information
Cardiology in the Young , Volume 26 , Issue 5 , June 2016 , pp. 979 - 982
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Copyright
© Cambridge University Press 2016 

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References

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