Clinical and necropsy findings are described in 56 patients with mitral valve prolapse (MVP): 15 patients, aged 16 to 69 years (mean 39), died suddenly and MVP was the only cardiac condition found at necropsy (hereafter called isolated MVP); the remaining 41 patients had other conditions that were capable of being fatal. Of the latter 41 patients had other conditions that were capable of being fatal. Of the latter 41 patients, 7 aged 17 to 59 years (mean 45), had associated congenital heart disease, and 34 patients, aged 17 to 70 years (mean 52), had no associated congenital cardiac abnormalities. Compared with the 34 patients without associated congenital heart disease and with non-MVP conditions capable in themselves of being fatal, the 15 patients who died suddenly with isolated MVP were younger (mean age 39 +/- 17 versus 52 +/- 15 years; p = 0.01), more often women (67% versus 26%; p = 0.008) and had a lower frequency of mitral regurgitation (7% versus 38%; p = 0.02). The 15 patients dying suddenly with isolated MVP also were less likely to have evidence of ruptured chordae tendineae (29% versus 67%; p = 0.04).