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The significance of the progression of systolic and diastolic dysfunctions in hypertrophic cardiomyopathy (HCM) is still an open issue. We sought to evaluate the clinical and prognostic implications of the progression of left ventricular systolic and diastolic dysfunction in HCM.One hundred one HCM patients were studied by echo-Doppler at baseline and during follow-up.During a follow-up of 109 ± 67 months, 28% of patients showed a progression to left ventricular diastolic dysfunction, defined as restrictive filling pattern (RFP), and 16% to left ventricular systolic dysfunction (left ventricular ejection fraction <50%). The 10-year heart transplant-free survival rate was 45% in patients with RFP at follow-up vs. 82% in the patients without RFP (P < 0.001), and 52% in patients with left ventricular systolic dysfunction at follow-up vs. 75% in the patients with left ventricular ejection fraction of at least 50% (P = 0.001). Baseline predictors of death/transplantation were New York Heart Association class III–IV, indexed left atrial diameter, and RFP. When RFP and left ventricular systolic dysfunction were added at follow-up, both emerged as prognostic predictors (RFP: hazard ratio 8.92, 95% confidence interval 2.5–31.86; systolic dysfunction: hazard ratio 25.35, 95% confidence interval 3.57–179.88) with a significant increase of area under the receiver-operating characteristic curves (0.81 vs. 0.70, P = 0.03) with respect to the baseline model.Left ventricular diastolic and/or systolic dysfunction at follow-up are relatively frequent in HCM and are associated with a poor prognosis.