Congenitally unguarded tricuspid valve orifice with multiple other defects in a child with refractory heart failure.

Congenitally unguarded tricuspid valve orifice, a variant of tricuspid valve dysplasia, is a rare malformation with protean manifestations. This report describes a 5-year old child with refractory heart failure who, on echocardiography, was found to have unguarded tricuspid valve orifice with severe tricuspid regurgitation, pulmonary valve dysplasia with severe pulmonary regurgitation , cor triatriatum dexter , myxomatous mitral valve , perimembranous ventricular septal defect and a diverticulum of the right ventricular outflow tract. The management options for this child are discussed.