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Prolapse of the antero-superior leaflet of the tricuspid valve secondary to congenital anomalies of the valvar and sub-valvar apparatus: a rare cause of severe tricuspid regurgitation

Published online by Cambridge University Press:  13 July 2005

Dominic J. R. Abrams ,
Philip Kilner ,
Janice A. Till ,
Darryl F. Shore ,
Babulal Sethia ,
Rodney C. G. Franklin  and
Alan G. Magee
Dominic J. R. Abrams
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Philip Kilner
Affiliation:
Department of Cardiac Magnetic Resonance, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Janice A. Till
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Darryl F. Shore
Affiliation:
Department of Cardiac Surgery, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Babulal Sethia
Affiliation:
Department of Cardiac Surgery, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Rodney C. G. Franklin
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
Alan G. Magee
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton & Harefield NHS Trust, London, United Kingdom
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Abstract

Congenital anomalies of the tricuspid valve, and/or its supporting apparatus, leading to severe tricuspid regurgitation are rare. Although well tolerated in early childhood, long-standing and progressive volume loading of the right heart leads to symptoms of decreased exercise tolerance, and may predispose to arrhythmias in the long term. We report three cases of severe tricuspid regurgitation related to anomalies of the cords supporting the antero-superior leaflet of the tricuspid valve. Shortened cords leading to tethering of the leaflet were seen in two cases, and hypoplasia of the leaflet in the other. In all cases, the regurgitant jet was directed posteriorly towards the coronary sinus and atrial septum. Surgical repair was possible in one case, while it proved necessary to replace the valve in a second. The third child is asymptomatic and under regular review.

Keywords

Atrioventricular valvetendinous cordschordae tendineaeanomaliesregurgitation
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 4 , August 2005 , pp. 417 - 421
Copyright
© 2005 Cambridge University Press

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