Ebstein's Anomaly Flashcards
What structures are commonly affected?
Tricuspid valve and RA/RV
RV may be hypoplastic. TV has varying degrees of regurgitation. RA is usually enlarged to varying degrees.
What leaflets are apically displaced in the tricuspid valve?
Typically the septal and posterior leaflets are below the AV junction in the right ventricle. The leaflets may adhere to the underlying myocardium (failure of delamination).
What 3 processes typically affect the anterior tricuspid leaflet?
Redundancy, tethering, and fenestrations of the anterior
tricuspid valve leaflet
What happens to the RV inflow?
Atrialization and dilation (to varying degrees)
What are the typical EKG findings?
“Splintered” RBBB pattern (rsrsr’ v1 or v2), Himalayan p waves, QR pattern V1 (may extend to V4), pre-excitation
What associated defect may lead to varying degrees of cyanosis?
More than 50% of patients have a shunt at the atrial level
with either a PFO or secundum ASD, which results in
varying degrees of cyanosis; VSDs also may occur
What conduction abnormality is commonly cited?
One or more accessory conduction pathways, increasing
the risk of atrial tachycardias (approximately 25%)
How is SCD commonly reported in Ebstein’s?
Sudden cardiac death may occur and has been attributed to atrial fibrillation with accelerated conduction through an accessory pathway or from ventricular arrhythmias.
What are some possible (usually mild) physical exam findings in EA?
- Low CO leading to Low pulse volume and peripheral cyanosis
- RV lift
- On auscultation, the first sound is loud, and there may be 1 or more systolic clicks. The murmur of TR is holosystolic at the lower left sternal border and increases on inspiration. End-stage disease with severe TR and ventricular dysfunction may manifest as right-sided heart failure.
What are some possible (usually mild) physical exam findings in EA?
- Low CO leading to Low pulse volume and peripheral cyanosis
- RV lift
- On auscultation, the first sound is loud, and there may be 1 or more systolic clicks. The murmur of TR is holosystolic at the lower left sternal border and increases on inspiration. End-stage disease with severe TR and ventricular dysfunction may manifest as right-sided heart failure.
What is the key echo finding to diagnose EA?
Ebstein’s anomaly is characterized by apical displacement of the septal tricuspid leaflet of more than 8 mm per m2 and the presence of a redundant, elongated anterior tricuspid leaflet.
