Congenital tricuspid valve regurgitation (TR) is a relatively uncommon condition that includes a heterogeneous group of lesions with a unique management strategy. There are wide anatomic variations that lead to congenital TR in patients without Ebstein malformation. Possible etiologies may include primary valve abnormalities (eg, congenital absence of chordae) or other forms of tricuspid valve dysplasia as in congenitally unguarded tricuspid valve, and patients with pulmonary atresia and intact ventricular septum, which can be similar to Ebstein's valves or secondary regurgitation in association with other anomalies as in atrioventricular septal defects, right ventricular outflow tract obstructive lesions (pulmonary stenosis or atresia with ventricular septal defect [VSD]), tricuspid valve annular dilatation in association with right ventricular volume overload lesions as in congenital coronary arterial fistula with secondary right ventricular enlargement, and Uhl's anomaly. Iatrogenic etiologies in the congenital population include TR secondary to previous VSD closure (chordal or leaflet injury), pacemaker or internal cardiac defibrillator lead-induced TR, and traumatic TR (ruptured chordae). Presentation depends on the severity of the disease and may be apparent in infancy, childhood, or adulthood.
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