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Cyanosis due to an isolated atrial septal defect: case report and review of the literature

Published online by Cambridge University Press:  24 August 2020

Thomas Krasemann Open the ORCID record for Thomas Krasemann [Opens in a new window] ,
Lennie van Osch-Gevers  and
Pieter van de Woestijne
Thomas Krasemann*
Affiliation:
Department of Paediatric Cardiology, Sophia Children’s Hospital, Rotterdam, Netherlands
Lennie van Osch-Gevers
Affiliation:
Department of Paediatric Cardiology, Sophia Children’s Hospital, Rotterdam, Netherlands
Pieter van de Woestijne
Affiliation:
Department of Cardiothoracic Surgery, Erasmus Medical Centre, Rotterdam, Netherlands
*
Author for correspondence: Thomas Krasemann, Department of Pediatric Cardiology, Sophia Children’s Hospital, Wytemaweg 80, 3015CNRotterdam, Netherlands. Tel: +31-10 70321088. E-mail: t.krasemann@erasmusmc.nl
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Abstract

Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare.

We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities. The baby with clinical features of Marfan’s syndrome had an eventration of the right-sided diaphragm. Surgical closure of the defect resolved the cyanosis, but the child died 10 weeks later of severe valvar dysfunction, related to Marfan’s syndrome.

Mechanisms of cyanosis in patients with atrial septal defects are discussed. Echocardiographic bubble studies both from the lower and upper half of the body may help to clarify the mechanism of an otherwise unexplained cyanosis.

Keywords

Atrial septal defectcyanosisdiaphragmatic eventration
Type
Brief Report
Information
Cardiology in the Young , Volume 30 , Issue 11 , November 2020 , pp. 1741 - 1743
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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