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Ebstein’s malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair

Published online by Cambridge University Press:  24 February 2010

Rami N. Khouzam ,
Joseph A. Dearani  and
Paul R. Julsrud
Rami N. Khouzam*
Affiliation:
Farmington Heart Center, Farmington, New Mexico
Joseph A. Dearani
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, United States of America
Paul R. Julsrud
Affiliation:
Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States of America
*
Correspondence to: Rami Khouzam, 92 Fleet Place, Mineola, NY 11501. Tel: (516) 780 7124; Fax: (505) 327 1134; E-mail: khouzamrami@yahoo.com
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Abstract

This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

Keywords

Ebstein’s malformationright ventricular outflow tract obstructionthrombuscongenital cardiac disease
Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 20 , Issue 1 , February 2010 , pp. 80 - 85
Copyright
Copyright © Cambridge University Press 2010

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References

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