title: The progression of left ventricular systolic and diastolic dysfunctions in hypertrophic cardiomyopathy: clinical and prognostic significance.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  One hundred one HCM patients were studied by echo-Doppler at baseline and during follow-up.
  
  
  RESULTS
  During a follow-up of 109 + or - 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.
  
  
  CONCLUSIONS
  Left ventricular diastolic and/or systolic dysfunction at follow-up are relatively frequent in HCM and are associated with a poor prognosis.
authors.affiliation: Cardiovascular Department, Ospedali Riuniti and University of Trieste, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Pinamonti
authors.forename: Marco
authors.initials: M
authors.lastname: Merlo
authors.forename: René
authors.initials: R
authors.lastname: Nangah
authors.forename: Renata
authors.initials: R
authors.lastname: Korcova
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Lenarda
authors.forename: Giulia
authors.initials: G
authors.lastname: Barbati
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D003971:Diastole; D018450:Disease Progression; D015150:Echocardiography, Doppler; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007558:Italy; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D016016:Proportional Hazards Models; D018570:Risk Assessment; D012307:Risk Factors; D016019:Survival Analysis; D013599:Systole; D013997:Time Factors; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D016277:Ventricular Function, Left; D055815:Young Adult