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Clinicopathological correlation—pulmonary atresia with imperforate tricuspid valve and aneurysmal dilatation of ascending aorta

Published online by Cambridge University Press:  19 August 2008

Rafael Hirsch ,
Leon Gerlis  and
Jane Somerville
Rafael Hirsch*
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
Leon Gerlis
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
Jane Somerville
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
*
Dr. Rafael Hirsch, Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, Sidney Street, London, United Kingdom.
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Abstract

A patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.

Keywords

Pulmonary atresia with intact ventricular septumimperforate tricuspid valveMarfan-like diseasesingle coronary arteryhypoplastic pulmonary arteries
Type
Clinico-Pathological Correlation
Information
Cardiology in the Young , Volume 3 , Issue 3 , July 1993 , pp. 220 - 224
Copyright
Copyright © Cambridge University Press 1993

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References

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