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Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death

Published online by Cambridge University Press:  19 August 2008

J. Thierrien ,
L. M. Gerlis ,
Philip Kilner  and
Jane Somerville
J. Thierrien
Affiliation:
Jane Somerville Grown Up Congenital Heart Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
L. M. Gerlis*
Affiliation:
Department of Cardiac Morphology, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
Philip Kilner
Affiliation:
Magnetic Resonance Imaging Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
Jane Somerville
Affiliation:
Jane Somerville Grown Up Congenital Heart Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
*
Dr L. M. Gerlis, The National Heart and Lung Institute, Imperial College School of Medicine, Dovehouse Street, London SW3. Tel: 0171 352 8121; Fax: 0171 351 8230.
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Abstract

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

Keywords

Complex pulmonary atresiacoronary arterial fistulapulmonary arterial aneurysm
Type
Clinico-Pathological Correlation
Information
Cardiology in the Young , Volume 9 , Issue 3 , May 1999 , pp. 249 - 256
Copyright
Copyright © Cambridge University Press 1999

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