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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
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

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.

Type
Original Article
Copyright
© 2005 Cambridge University Press

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References

Becker AE, Becker MJ, Edwards JE. Pathological spectrum of dysplasia of the tricuspid valve. Arch Pathol 1971; 91: 167178.Google Scholar
McElhinney DB, Silverman NH, Brook MM, Hanley FL, Stanger P. Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valve dysplasia in the era of color flow echocardiography. Cardiol Young 1999; 9: 300304.Google Scholar
Reddy VM, McElhinney DB, Brook MM, Silverman NH, Stanger P, Hanley FL. Repair of congenital tricuspid valve abnormalities with artificial chordae tendinae. Ann Thorac Surg 1998; 66: 172176.Google Scholar
Katogi T, Aeba R, Ito T, et al. Surgical management of isolated congenital tricuspid regurgitation. Ann Thorac Surg 1998; 66: 15711574.Google Scholar
Kobza R, Kurz DJ, Oechslin EN, et al. Aberrant tendinous cords with tethering of the tricuspid leaflets: a congenital anomaly causing severe tricuspid regurgitation. Heart 2004; 90: 319323.Google Scholar
Dearani J, Danielson GK. Congenital heart surgery nomenclature and database project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69: S106S117.Google Scholar
D'Cruz IA, Taylor J, Kaiser J. Retrograde systolic flow in the coronary sinus due to selective tricuspid regurgitant jet entry. Echocardiography 2000; 17: 341343.Google Scholar
Messika-Zeitoun D, Thomson H, Bellamy M, et al. Medical and surgical outcome of tricuspid regurgitation caused by flail leaflets. J Thorac Cardiovasc Surg 2004; 128: 296302.Google Scholar
Hornberger LA, Sahn DJ, Kleinman CS, Copel JA, Reed KL. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991; 17: 167173.Google Scholar
Morton JB, Sanders P, Vohra JK, et al. Effect of chronic atrial stretch on atrial electrical remodeling in patients with an atrial septal defect. Circulation 2003; 107: 17751782.Google Scholar