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A complex variant of tricuspid atresia: survival to 60 years without surgery

Published online by Cambridge University Press:  19 August 2008

L M Gerlis ,
J Mayet  and
J Somerville
L M Gerlis*
Affiliation:
From the Grown Up Congenital Heart Unit and the Section of Cardiac Morphology, Imperial College School of Medicine at National Heart and Lung Institute, London, UK
J Mayet
Affiliation:
From the Grown Up Congenital Heart Unit and the Section of Cardiac Morphology, Imperial College School of Medicine at National Heart and Lung Institute, London, UK
J Somerville
Affiliation:
From the Grown Up Congenital Heart Unit and the Section of Cardiac Morphology, Imperial College School of Medicine at National Heart and Lung Institute, London, UK
*
L M Gerlis Paediatrics, Imperial College School of Medicine at National Heart & Lung Institute, Dovehouse Street, London SW3 6LY. Tel: 0171 351 8751, Fax: 0171 351 8230
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Summary

We report a case of tricuspid atresia in which the symptoms underwent spontaneous improvement during childhood, and did not return until the age of 56 after which the patient went into progressive congestive failure and atrial fibrillation. He died at the age of 60, without ever having surgical intervention. Autopsy confirmed the presence of tricuspid atresia due to absence of the right atrioventricular connection, and also revealed partial left juxtaposition of the atrial appendages, an atrial septal defect in the oval fossa, rudimentary right ventricle, double outlet left ventricle with anterior aorta (anatomically corrected malposition), subvalvar and valvar pulmonary stenosis, dilation of the pulmonary arteries, patent arterial duct and major systemic to pulmonary collateral arteries.

It was considered that the development of the systemic to pulmonary arterial anastomoses probably accounted for the initial remission of symptoms, whilst the obstruction of the pulmonary outflow tract prevented the development of any significant degree of pulmonary hypertension in the presence of double outlet left ventricle.

Keywords

Absent right atrioventricular connectionabnormal ventriculo-arterial relationsanatomically corrected malposition
Type
Clinico-Pathological Correlations
Information
Cardiology in the Young , Volume 8 , Issue 2 , April 1998 , pp. 275 - 280
Copyright
Copyright © Cambridge University Press 1998

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