Describes a rare case of congenitally unguarded tricuspid valve orifice with right ventricular apical isolation presenting with right heart failure and atrial fibrillation in an adult.
Consider in adult right heart failure differentials; leaves open prevalence, natural history, and management.
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.
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Mohan et al. (2015) studied this question.
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