TRICUSPID VALVE DYSPLASIA  

 

Tricuspid valve dysplasia, absence of the tricuspid valve leaflets, Uhl's and Ebstein's anomaly are a spectrum of diseases characterized by dysplasia, hypoplasia or aplasia of the tricuspid valve or right ventricular myocardium or both.
 

Case 1

 

Case 2

Subcostal view – large right atrium (RA), normal right ventricle (RV) and left ventricle (LV).

        Sagittal view – large right atrium

Normal position of mitral and tricuspid valves. This excludes Ebstein’s anomaly as the cause of RA enlargement

       Normal LV and aortic outflow tract

Large RA and normal size RV

Mitral and tricuspid valves at normal levels (no apical displacement of tricuspid valve)

Marked tricuspid regurgitation due to valvular dysplasia

Normal pulmonary vein draining into LA. This excludes total anomalous pulmonary venous return

 

 

 

 

Video clip of Tricuspid Valve Dysplasia

 

DIFFERENTIAL DIAGNOSIS

 

Tricuspid valve dysplasia

Valve leaflets are thickened and may be redundant or hypoplastic.
Leaflets attach normally in the AV groove.
M-mode of tricuspid valve dysplasia.

Left cardiac axis deviation.

Ebstein's anomaly

Attachment of the septal and posterior leaflets are displaced to the right
Tricuspid valve may be dysplastic.

Absent tricuspid valve
leaflet (unguarded tricuspid valve)

No tricuspid valve leaflets are visible.

Uhl's anomaly

Normally formed and attached leaflets
Right atrial dilatation and thinned right ventricular myocardium.

Tricuspid valve incompetence

 

 

 

REFERENCES
  1. Hornberg LK, Sahn DJ, Kleinman CS et.al. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: Diagnosis and outcome. J Am Coll Cardiol 1991;17:167.
  2. Benson CB, Brown DL, Roberts DJ. Uhl's anomaly of the heart mimicking Ebstein's anomaly in utero. J Ultrasound Med 1995;14:781-783.