Hostname: page-component-78c5997874-g7gxr Total loading time: 0 Render date: 2024-11-06T10:47:19.004Z Has data issue: false hasContentIssue false

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*
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.

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.

Type
Brief Reports
Copyright
Copyright © Cambridge University Press 1993

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Moller, JH, Nakib, A, Anderson, RC, Edwards, JE.Congenital cardiac disease with polysplenia. Circulation 1967; 36: 789799.CrossRefGoogle ScholarPubMed
Rose, V, Izakawa, T, Moe, CAF.Syndromes of asplenia and polysplenia. A review of cardiac and non cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840852.CrossRefGoogle ScholarPubMed
Devine, WA.What if Ivemark had suggested the term “syndrome of visceral sympathy with asplenia “instead of”Asplenia, a teratologic syndrome of visceral heterotaxy”? Cardiol Young 1992; 2: 108113.CrossRefGoogle Scholar
Debich, DE, Devine, WA, Anderson, RH.Polysplenia with normally structured hearts. Am J Cardiol 1990; 65: 12741275.CrossRefGoogle ScholarPubMed
Osler, W.On a family form of recurring epistaxis, associated with multiple telangiectoses of skin and mucous membrane. Bull Hopkins Hosp 1901; 12: 333335.Google Scholar
Stumper, OFW, Sreeram, N, Elzenya, NJ, Sutherland, GR.Diagnosis ofatrial situs by transoesophageal echocardiography. J Am Coll Cardiol 1990; 16: 442446.CrossRefGoogle Scholar