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Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood

Part of: Cardiac Morphology

Published online by Cambridge University Press:  29 August 2017

Michael L. O’Byrne ,
Russell R. Cross  and
Gerard R. Martin
Michael L. O’Byrne*
Affiliation:
Division of Cardiology, Center for Pediatric Clinical Effectiveness, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America Department of Pediatrics Perelman School of Medicine, The University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Russell R. Cross
Affiliation:
Department of Pediatrics, Division of Cardiology Children’s National Medical Center, George Washington University School of Health Sciences, Washington, District of Columbia, United States of America
Gerard R. Martin
Affiliation:
Department of Pediatrics, Division of Cardiology Children’s National Medical Center, George Washington University School of Health Sciences, Washington, District of Columbia, United States of America
*
Correspondence to: M. L. O’Byrne, MD, MSCE, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104, United States of America. Tel: +1 215 590 1790; Fax: 1-215-590-4620; E-mail: obyrnem@email.chop.edu
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Abstract

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called “scimitar vein” without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.

Keywords

CHDCTtranscatheter intervention
Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 2 , February 2018 , pp. 329 - 333
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Copyright
© Cambridge University Press 2017 

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