Congenitally unguarded tricuspid valve orifice with right ventricular apical isolation in an adult

Abstract

Congenitally unguarded tricuspid valve (TV) orifice, a variant of TV dysplasia, is a rare malformation with protean manifestations. This report describes a symptomatic adult male with gross right heart failure and atrial fibrillation, who was found to have an unguarded TV orifice with isolation of the trabecular apical cavity of the right ventricle (RV) and muscular ridges separating outflow tract (forme-fruste of the double-chambered RV). The right ventricular outflow tract remained patent.

Ebstein Anomaly With Right Atrial Clot

“…A close differential diagnosis was unguarded TV orifice which is associated with severe dysplasia of tricuspid leaflets and absence of TV leaflets and absent chordae [ 2 , 3 ] (presence of septal and anterior leaflets of TV and associated chordae attached to ATL in our case helped in ruling out this). Previous case reports have shown large clot in RA in the setting of unguarded TV orifice [ 2 , 4 ].…”

“…A close differential diagnosis was unguarded TV orifice which is associated with severe dysplasia of tricuspid leaflets and absence of TV leaflets and absent chordae [ 2 , 3 ] (presence of septal and anterior leaflets of TV and associated chordae attached to ATL in our case helped in ruling out this). Previous case reports have shown large clot in RA in the setting of unguarded TV orifice [ 2 , 4 ]. Other differential diagnoses which included pulmonary atresia with intact interatrial septum with TV dysplasia [ 2 ] (presence of patent pulmonary valve in our case) and UHL’s anomaly [ 4 ] (presence of abnormal TV in our case) were similarly excluded.…”

“…Previous case reports have shown large clot in RA in the setting of unguarded TV orifice [ 2 , 4 ]. Other differential diagnoses which included pulmonary atresia with intact interatrial septum with TV dysplasia [ 2 ] (presence of patent pulmonary valve in our case) and UHL’s anomaly [ 4 ] (presence of abnormal TV in our case) were similarly excluded. Right atrial clots are usually seen in association with central venous lines, or indwelling catheters.…”

A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult

“…Dysplasia of the leaflets along with displacement of the septal leaflet but not absence is an integral part of Ebstein malformation. TV is structurally normal in Uhl's anomaly . Patients with unguarded TV orifice and mild degree of right ventricular dysfunction survive to adulthood and even reach old age .…”

“…Tricuspid valve dysplasia is the most common cause of isolated tricuspid regurgitation, and the unguarded TV orifice is the most extreme form with a very variable natural history . Differential diagnosis of unguarded TV is TV dysplasia in association with pulmonary atresia and intact ventricular septum, and Ebstein's and Uhl's anomaly.…”