Hostname: page-component-7479d7b7d-m9pkr Total loading time: 0 Render date: 2024-07-10T11:28:27.564Z Has data issue: false hasContentIssue false
  • English
  • Français

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
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Tabatabaei, N, Katanyuwong, P, Breen, JF, et al. Uncommon variant of Ebstein anomaly with tricuspid stenosis. Circulation 2009; 120: e1–e2.CrossRefGoogle ScholarPubMed
2.Morgan, ML, Dearani, JA, Danielson, GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135: 1120–1136.Google Scholar
3.Dearani, JA, Bacha, E, da Silva, JP. Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Op Tech Thorac Cardiovasc Surg 2008; 13: 109–125.CrossRefGoogle Scholar
4.Quinonez, LG, Dearani, JA, Puga, FJ, et al. Results of the 1.5 ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007; 133: 1303–1310.CrossRefGoogle ScholarPubMed