Ebstein's Anomaly
Definition
A congenital malformation of the tricuspid valve in which attachments of one, two or all three leaflets are displaced downward from the annulus to the right ventricular wall. The portion of the right ventricle above the displaced leaflets is composed of atrial tissue: referred to as the "atrialized" portion of the right ventricle
Associated Anomalies
- Atrial septal defect (75% with a right-to-left shunt)
- Ventricular septal defect
- Pulmonic stenosis (valvular)
- Endocardial cushion defects
- Mitral stenosis
- Tetralogy of Fallot
- D-transposition of the great arteries
- Patent ductus arteriosus
Signs and Symptoms
- Dyspnea
- Fatigue
- Cyanosis
- Palpitations (especially if associated with accessory bypass tracts)
Complication
- Right-sided congestive heart failure
Cardiac Auscultation
- Widely split S1
- "Sail" sound
- Loud S2
- Tricuspid regurgitation
Electrocardiogram
- Wolff-Parkinson-White type B
- Atrial and or ventricular arrhythmias
Chest X-ray
- Cardiomegaly
Treatment
- Plication with closure of atrial septal defect
- Annuloplasty for tricuspid regurgitation
M-Mode / 2-D
- Delayed closure of the tricuspid valve as compared with the mitral valve ≥80 msec
- Increased excursion of the anterior tricuspid valve leaflet
- Right ventricular overload pattern (right ventricular enlargement with paradoxical septal motion)
- The apical four-chamber view reveals the extent of the tricuspid valve displacement; Ebstein's is diagnosed when the level of the mitral valve annulus to the displaced tricuspid septal leaflet measures ≥ 20 mm or ≥ 8 mm/m2
Doppler
- Tricuspid regurgitation
- Tricuspid stenosis
- Patent foramen ovale
- Atrial septal defect (predominantly right-to-left shunt)
- Valvular pulmonic stenosis/ atresia