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Non-cardiac cyanosis in left isomerism—report of two cases of multiple pulmonary arteriovenous malformations

Published online by Cambridge University Press:  19 August 2008

Michael Burch ,
Paris Iacovides ,
Parviz Habibi  and
David Celermajer
Michael Burch*
Affiliation:
From the Department of Cardiology, The Hospitalfor Sick Children, Great Ormond Street, London and St. Mary's Hospital, London
Paris Iacovides
Affiliation:
From the Department of Cardiology, The Hospitalfor Sick Children, Great Ormond Street, London and St. Mary's Hospital, London
Parviz Habibi
Affiliation:
From the Department of Cardiology, The Hospitalfor Sick Children, Great Ormond Street, London and St. Mary's Hospital, London
David Celermajer
Affiliation:
From the Department of Cardiology, The Hospitalfor Sick Children, Great Ormond Street, London and St. Mary's Hospital, London
*
Dr. Michael Butch, Department of Cardiology, The Hospital for Sick Children, Great Ormond Street, London, WC IN 3JH, United Kingdom. Tel. 71-405-9200 ext. 5245.
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Summary

Isomerism of the left atrial appendages is often associated with cyanosis due to intracardiac structural abnormalities such as common atrium and/or anomalous pulmonary or systemic venous connections. We report two cases of children with cyanosis and left isomerism but no other lesions within the heart. In these cases, multiple small pulmonary arteriovenous malformations were found bilaterally, resulting in intrapulmonary right-to-left shunting and systemic desaturation.

Keywords

Atrial arrangementisomerism of atrial appendagespolysplenia
Type
Brief Reports
Information
Cardiology in the Young , Volume 3 , Issue 1 , January 1993 , pp. 64 - 66
Copyright
Copyright © Cambridge University Press 1993

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