To assess the prevalence of ventricular arrhythmias and late potentials (LPs) in mitral valve prolapse (MVP) and to identify clinical, ECG, and echocardiographic markers of spontaneous ventricular arrhythmias, we studied 58 consecutive patients (mean age 46.6 +/- 17.8 years; 29 males, 29 females) with MVP diagnosed by echocardiography. Patients underwent ambulatory ECG recording (n = 58), exercise stress test (n = 56), signal-averaged ECG (n = 58), and programmed ventricular stimulation (n = 52). Ten patients (17.2%) had spontaneous nonsustained ventricular tachycardia (NSVT), 26 patients (44.8%) had premature ventricular contractions (PVCs), Lown grade > or = 3 during 24-hour ECG, and 19 had Lown grade > or = 3 PVCs during exercise stress test; 13 patients had LPs (22.4%). We provoked sustained VT in one case and NSVT in ten cases. Patients with complex ventricular arrhythmias during 24-hour ECG and exercise stress test were older and more often had mitral regurgitation. There was a statistical correlation between the presence of LPs and spontaneous VT (46.1% vs 8.9%; P < 0.005) and induced ventricular arrhythmias (50% vs 12.8%; P < 0.005). No correlation was found between spontaneous ventricular arrhythmias and thickness or posterior displacement of the mitral valve. In conclusion, complex ventricular arrhythmia (especially VT) and LPs are frequent in MVP. Patient age and mitral regurgitation seem to be determinant factors of complex ventricular arrhythmias in MVP. On signal-averaged ECG, absence of LPs seems to be a good additional marker to identify MVP patients without spontaneous VT. On the other hand, programmed ventricular stimulation does not appear valuable in determining a MVP subgroup with a high risk of ventricular arrhythmias.