The Congenital Tricuspid Valve Spectrum: From Ebstein to Dysplasia

Elizabeth H. Stephens, Joseph Dearani, Muhammad Yasir Qureshi, Naser Ammash, Joseph J. Maleszewski

Research output: Contribution to journalReview articlepeer-review


Ebstein anomaly has a breadth of presentations, including “typical” and “atypical,” and can be confused with congenital tricuspid dysplasia. We summarize how to differentiate within this spectrum of disease. Both typical and atypical Ebstein have an underlying failure of delamination, but atypical Ebstein does not have ≥8mm/m2 apical septal leaflet displacement. In congenital tricuspid dysplasia, delamination is normal, while the leaflets and subvalvar apparatus are abnormal. To summarize, the sine qua non feature of Ebstein anomaly, present in both typical and atypical, is the failure of delamination. These are distinct from congenital tricuspid valve dysplasia in which the pathology is in the leaflet itself.

Original languageEnglish (US)
Pages (from-to)783-791
Number of pages9
JournalWorld Journal for Pediatric and Congenital Heart Surgery
Issue number6
StatePublished - Nov 2020


  • congenital tricuspid regurgitation
  • Ebstein anomaly
  • echocardiography
  • imaging
  • pathology
  • tricuspid valve

ASJC Scopus subject areas

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


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