title: Sudden death in hypertrophic cardiomyopathy: identification of high risk patients.
abstract: OBJECTIVE
  We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD).
  
  
  BACKGROUND
  Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers.
  
  
  METHODS
  We studied 368 patients (14 to 65 years old, 239 males) with HCM. There were five variables: nonsustained ventricular tachycardia (NSVT), syncope, exercise blood pressure response (BPR), family history of sudden death (FHSD) and left ventricular wall thickness (LVWT).
  
  
  RESULTS
  During follow-up (3.6+/-2.5 years [range 2 days to 9.6 years]), 36 patients (9.8%) died, 22 of them suddenly. Two patients received heart transplants. The six-year SD-free survival rate was 91% (95% confidence interval [CI] 87% to 95%). In the Cox model, there was a significant pairwise interaction between FHSD and syncope (p = 0.01), and these were subsequently considered together. The multivariate SD risk ratios (with 95% CIs) were 1.8 for BPR (0.7 to 4.4) (p = 0.22); 5.3 for FHSD and syncope (1.9 to 14.9) (p = 0.002); 1.9 for NSVT (0.7 to 5.0) (p = 0.18) and 2.9 for LVWT (1.1 to 7.1) (p = 0.03). Patients with no risk factors (n = 203) had an estimated six-year SD-free survival rate of 95% (95% CI 91% to 99%). The corresponding six-year estimates (with 95% CIs) for one (n = 122), two (n = 36) and three (n = 7) risk factors were 93% (87% to 99%), 82% (67% to 96%) and 36% (0% to 75%), respectively. Patients with two or more risk factors had a lower six-year SD survival rate (95% CI) compared with patients with one or no risk factors (72% [56% to 88%] vs. 94% [91% to 98%]) (p = 0.0001).
  
  
  CONCLUSIONS
  This study demonstrates that patients with multiple risk factors have a substantially increased risk of SD sufficient to warrant consideration for prophylactic therapy.
authors: Department of Cardiological Sciences, St. George's Hospital Medical School, London, United Kingdom. pelliott@sghms.ac.uk
authors: P M
authors: PM
authors: Elliott
authors: J
authors: J
authors: Poloniecki
authors: S
authors: S
authors: Dickie
authors: S
authors: S
authors: Sharma
authors: L
authors: L
authors: Monserrat
authors: A
authors: A
authors: Varnava
authors: N G
authors: NG
authors: Mahon
authors: W J
authors: WJ
authors: McKenna
mesh_terms: D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000401:mortality*; D016757:Death, Sudden, Cardiac; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018570:Risk Assessment; D012307:Risk Factors; D016019:Survival Analysis