Tricuspid valve – unguarded

Tricuspid valve – unguarded

Description

In this very rare condition, the tricuspid valve leaflets are rudimentary. The condition is usually fatal in utero or in early neonatal life. Tricuspid valve replacement is a theoretical possibility if the neonate survives to a surgical procedure. Valve replacement is problematic in children as an artificial valve has to be replaced with growth and anticoagulants are necessary.

Diagnosis

No tricuspid valve leaflets are seen in the site of the tricuspid valve. There is free tricuspid regurgitation with dilatation of the right atrium and ventricle. There is often progression to hepatic congestion and hydrops. The cardiothoracic ratio is increased.

Differential Diagnosis

The condition could be mistaken for Ebstein’s malformation but the degree of incompetence is likely to be much more severe in an unguarded orifice.

Sonographic Features

No tricuspid leaflets seen.

Severe tricuspid incompetence.

Right atrial dilatation.

Increased C/T ratio.

Associated Syndromes

References

Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia Circulation 30:911-917