Tricuspid valve agenesis: a case report of a rare cause of tricuspid regurgitation in an adult

Abstract

Background Tricuspid valve agenesis (TVA) is an exceptionally rare congenital cardiac disease characterized by the incomplete formation or complete absence of one or more tricuspid leaflets. It is commonly diagnosed during childhood due to the development of heart failure symptoms. Case summary We report the case of 62-year-old women admitted for worsening of heart failure symptoms (New York Heart Association functional class IV at admission). Standard transthoracic echocardiogram was performed and showed severe right heart dilatation and severe tricuspid regurgitation. Three-dimensional echocardiogram revealed the absence of the posterior tricuspid leaflet. The patient underwent an uncomplicated surgical bioprosthetic valve replacement with a favorable outcome and partial recovery of right ventricular function. In our case, valve repair was not favored due to the additional mild hypoplasia of the anterior leaflet. The 3-month postoperative evolution of the patient was favorable, with significant symptom relief. Discussion The diagnosis of tricuspid agenesis during adulthood is uniquely uncommon. Three-dimensional echocardiography can provide accurate preprocedural insight into the valve anatomy, allowing to plan for either valve repair when the anatomy is suitable or valve replacement.