The natural history of isolated congenital pulmonary valve incompetence: surgical implications.

The natural history of isolated congenital pulmonary valve incompetence has been determined by actuarial and parametric techniques from 72 cases collected from the literature. Symptoms developed within 20 years in 6% (CL 4% to 9%) of the patients and within 40 years in 29% (CL 22% to 36%). The hazard function of the development of symptoms demonstrates a risk increasing in time, particularly evident after 40 years. Death followed the appearance of symptoms after an average of 39 months in 3 patients who died. The surgical implications as regards the use of transannular patches and valveless extracardiac conduits are discussed.