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A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification

Published online by Cambridge University Press:  19 August 2008

L. M. Gerlis  and
J. Somerville
L. M. Gerlis*
Affiliation:
Departments of Paediatric Cardiac Morphology and the Jane Somerville Grown-up Congenital Heart Unit, The Royal Brompton Hospital, Imperial College School of Medicine, London, UK
J. Somerville
Affiliation:
Departments of Paediatric Cardiac Morphology and the Jane Somerville Grown-up Congenital Heart Unit, The Royal Brompton Hospital, Imperial College School of Medicine, London, UK
*
Dr L. M. Gerlis, Imperial College School of Medicine at The National Heart and Lung Institute, Dovehouse Street, London SW3 6LY, UK. Tel: 0171 351 8750; Fax: 0171 351 8230
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Abstract

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.

Keywords

Absent papillary musclescongenital cardiomyopathybifoliate aortic valvecoarctation of aortacongenital cardiac malformationmyocardial calcificationmycordial fascicular disarrayventricular septal defect
Type
Clinco-Pathological Correlation
Information
Cardiology in the Young , Volume 9 , Issue 4 , July 1999 , pp. 348 - 356
Copyright
Copyright © Cambridge University Press 1999

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