Classification of Ebstein anomaly

There are several classification systems for Ebstein anomaly, the most commonly used are the Carpentier classification (anatomical classification) and the Celermajer index or the Great Ormond Street Echocardiography (GOSE) score for neonates.

These classification systems are crucial for determining the severity of the Ebstein anomaly.

Carpentier classification

• type A: mild

  • mild apical displacement of the septal and posterior tricuspid valve leaflets

  • small "atrialised" ventricle

  • adequate function of the functional right ventricle

• type B: moderate

  • moderate apical displacement of the septal and posterior tricuspid valve leaflets

  • abnormal attachment of the otherwise freely mobile anterior leaflet

  • moderately sized "atrialised" ventricle with reduced function

  • reduced volume but adequate function of the functional right ventricle

• type C: severe

  • severe apical displacement of the septal and posterior tricuspid valve leaflets

  • abnormal attachments restricting the mobility of the anterior leaflet and potentially obstructing the right ventricular outflow tract

  • large "atrialised" ventricle with reduced function

  • small functional right ventricle

• type D: extensive (tricuspid valve sac)

  • complete non-delamination of the tricuspid valve leaflets

  • multiple abnormal attachments

  • almost complete "atrialisation" of the right ventricle with the exception of a small insufficient infundibular component

Great Ormond Street Echocardiography (GOSE) score

The GOSE score uses the ratio of the combined area of the right atrium (RA) and atrialised right ventricle (aRV) versus the combined area of the functional right ventricle (fRV), left atrium (LA), and left ventricle (LV):

GOSE score: area (RA+aRV) / area (fRV+LA+LV)

• grade 1: < 0.5

• grade 2: 0.5-0.99

• grade 3: 1-1.49

• grade 4: ≥ 1.5

History and etymology

In 1988, the French cardiac surgeon Alain Frédéric Carpentier and colleagues established a classification of Ebstein anomaly based on the anatomy of the right ventricle and the tricuspid valve ³.

The GOSE scoring system was developed by the British cardiologist David Celermajer during research on 50 individuals with neonatal Ebstein anomalies from 1961 to 1990 ⁵.