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Common pulmonary vein atresia: report of three cases and review of the literature

Published online by Cambridge University Press:  29 October 2015

Michael Perez ,
T. K. Susheel Kumar ,
Mario Briceno-Medina ,
Mohammed Alsheikh-Ali ,
Shyam Sathanandam  and
Christopher J. Knott-Craig
Michael Perez*
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
T. K. Susheel Kumar
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
Mario Briceno-Medina
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
Mohammed Alsheikh-Ali
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
Shyam Sathanandam
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
Christopher J. Knott-Craig
Affiliation:
Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, Tennessee, United States of America
*
Correspondence to: M. Perez, The Heart Institute, Le Bonheur Children’s Hospital, 848 Adams Street, Memphis, TN 38103, United States of America. Tel: 214 734 0327; Fax: 901-287-5970; E-mail: mperez2@uthsc.edu
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Abstract

Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.

Keywords

Common pulmonary vein atresiaCHDcyanosispulmonary vein atresia
Type
Review Articles
Information
Cardiology in the Young , Volume 26 , Issue 4 , April 2016 , pp. 629 - 635
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Copyright
© Cambridge University Press 2015 

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