SJS July-03
EBSTEIN’S ANOMALY
Brickner ME, et al. Congenital heart disease in adults. Second of two parts. N Engl J Med. 2000 Feb 3;342(5):334-42..
Take home points:
1. Ebstein’s anomaly results in an abnormally placed tricuspid valve and “atrialization” of the RV with
decreased RV outflow
2. 80% of patients with Ebstein’s anomaly have RÆL shunt(s) so cyanosis usually occurs
3. In adults with Ebstein’s anomaly, Eisenmenger’s syndrome and WPW can be complications
Anatomy of Ebstein’s anomaly: a problem with the tricuspid valve apparatus
• The septal leaflet (and often posterior leaflet) of the tricuspid valve are displaced into the RV
• The anterior leaflet is abnormal, large, and adherent to the RV free wall
• A large portion of the RV is “atrialized” so that the functional RV is very small
• Tricuspid valve usually regurgitant (but may be stenotic)
• 80% of patients have associated ASD and/or PFO through which RÆL shunting may occur
Clinical manifestations:
• Presents with cyanosis very early in life due to RÆL shunt
• Differential diagnosis of cyanotic congenital heart disease: remember the T’s…
Tetralogy of Fallot, Transposition of the great vessels, Total anomalous pulmonary venous return, Tricuspid
problems (Ebstein’s anomaly, tricuspid atresia)
• Severity of hemodynamic derangements depends on how much of the RV is left functional; if the anomaly is minor,
patients may present later in life (adulthood) with Eisenmenger’s syndrome
• Predictors of outcome in adults with Ebstein’s anomaly:
- NYHA functional class
- Heart size
- Presence or absence of cyanosis
- Presence or absence of paroxysmal atrial tachycardias
• Physical findings: TR murmur, hepatomegaly, widely split S2
• ECG findings: tall, broad P waves, RBBB, 1°AVB; 20% of patients have WPW so look for delta wave
• CXR findings: cardiomegaly (due to huge RA), decreased pulmonary vascular markings (RÆL shunt)
Management:
•
In adults, definitive therapy involves
repairing the tricuspid valve
• Medical man