title: Prognostic role of clinical presentation in symptomatic patients with hypertrophic cardiomyopathy.
abstract: OBJECTIVE
  To evaluate the long-term prognostic impact of baseline symptoms in a cohort of hypertrophic cardiomyopathy (HCM) patients.
  
  
  RESULTS
  We considered 84 HCM patients symptomatic at diagnosis: 26 (31%) with heart failure (group 1), 34 (40%) with syncope/palpitations (group 2) and 24 (29%) with chest pain (group 3). During a median follow-up of 102 (53-187) months, 25 (30%) patients died/underwent heart transplant (HTx), 14 of 26 (54%) in group 1, 10 of 34 (29%) in group 2 and one of 24 (4%) in group 3. At 12, 60 and 120 months, HTx-free survival rates were 100, 79 and 52% in group 1, vs. 100, 97 and 69% in group 2, vs. 96, 96 and 96% in group 3, respectively (P = 0.008). At multivariate analysis, heart failure [hazard ratio (HR) 2.59, confidence interval (CI) 95% 1.09-6.17, P = 0.032] and left atrium diameter (HR 1.83, CI 95% 1.16-2.89, P = 0.009) emerged as independent predictors of death/HTx, with incremental prognostic power with respect to echo Doppler variables of left ventricular systolic and diastolic dysfunction [area under the curve (AUC) of receiver operating characteristics (ROC) curves at 5 years: 0.90 vs. 0.78, respectively, P = 0.03].
  
  
  CONCLUSIONS
  Clinical presentation emerged as a relevant prognostic tool in HCM patients symptomatic at onset, as heart failure was associated with a particularly poor outcome. Heart failure and left atrium diameter at diagnosis showed incremental prognostic power compared with echo Doppler assessment of left ventricular systolic and diastolic dysfunction.
authors.affiliation: Cardiovascular Department, Ospedali Riuniti and University of Trieste, Trieste, Italy. gherardobis@yahoo.it
authors.forename: Gherardo
authors.initials: G
authors.lastname: Finocchiaro
authors.forename: Bruno
authors.initials: B
authors.lastname: Pinamonti
authors.forename: Marco
authors.initials: M
authors.lastname: Merlo
authors.forename: Francesca
authors.initials: F
authors.lastname: Brun
authors.forename: Giulia
authors.initials: G
authors.lastname: Barbati
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000209:etiology; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D002317:Cardiovascular Agents / Q000627:therapeutic use; D002637:Chest Pain / Q000209:etiology; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D015150:Echocardiography, Doppler; D005260:Female; D006325:Heart Atria / Q000000981:diagnostic imaging; D006333:Heart Failure / Q000209:etiology / Q000503:physiopathology; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012307:Risk Factors; D013575:Syncope / Q000209:etiology; D013997:Time Factors; D016277:Ventricular Function, Left; D055815:Young Adult
chemical_list: D002317:Cardiovascular Agents