Ebsteins malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair

Rami N. Khouzam, Joseph A. Dearani, Paul R. Julsrud

Research output: Contribution to journalArticlepeer-review

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 Ebsteins 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.

Original languageEnglish (US)
Pages (from-to)80-85
Number of pages6
JournalCardiology in the young
Volume20
Issue number1
DOIs
StatePublished - Feb 1 2010

Keywords

  • Congenital cardiac disease
  • Ebsteins malformation
  • Right ventricular outflow tract obstruction
  • Thrombus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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