Long-term clinical outcome of patients with tricuspid atresia. I. "Natural history"

The clinical course and outcome of 111 patients with tricuspid atresia, seen during 1972-1982, were reviewed. All clinical and investigation data, progress and management were analysed. The mean age at presentation was 21.9 +/- 8.8 days. Patients with reduced pulmonary blood flow presented earlier. Patients with normal or increased pulmonary blood flow usually presented in heart failure. "True" natural history was observed in 13 unoperated patients. Six of these died at the age of 7 days to 8 months (mean 32.2 days). Seven patients survived without surgery for 11 months to 24 years, the older ones developing pulmonary vascular obstructive disease. The overall mortality in the whole series was 32.4% (36/111), the mean age at death was 3.1 years (range 3 days-48 years). The mortality rate within the first year of life was 22.5%. The mean age of survivors at follow-up was 10.7 +/- 0.9 years (range 11 months-27 years).