Congenital tricuspid regurgitation is an extremely rare condition. The morphologic heterogeneity makes it difficult to derive any conclusions regarding the best strategy for management of this condition.

We analysed the published literature on “congenital tricuspid regurgitation” using MEDLINE. In addition, the reference sections of all relevant articles were searched to identify additional cases. Studies published till June 2009 are included.

As far as we could establish, there were 123 cases of congenital tricuspid regurgitation. Of these, 63 were documented during surgery, 38 during autopsy, and 22 were diagnosed by echocardiography or cardiac catheterisation. The mean age was 22.57 years, with a standard deviation of 23.42 years, and the age ranged from 1 day to 80 years, with a median of 16 years. There was a male predominance, with a male-to-female ratio of approximately 3:2.

Congenital tricuspid regurgitation is an uncommon clinical entity with wide anatomical variations. The severity of disease dictates the presentation in infancy, childhood, or adulthood. Tricuspid valve repair is the ideal treatment whenever feasible, especially in children. We propose a new classification for congenital tricuspid regurgitation, which not only includes the anatomical variations, but can also help the surgeon in deciding on the best strategy for management.