Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration.
OBJECTIVE
This study evaluates the clinical course and identifies risk factors for sudden cardiac death (SCD) and clinical deterioration in hypertrophic cardiomyopathy (HCM) in a large community-based population. Comparison was made with data from six tertiary referral and six nonreferral institutions.


BACKGROUND
Hypertrophic cardiomyopathy is a disease with marked heterogeneity in clinical presentation and prognosis. Risk factors for SCD are not well defined in patients free of referral bias.


METHODS
Between 1970 and 1999, 225 consecutive patients (mean age [+/-SD] 41+/-16 years) were examined and followed at yearly intervals.


RESULTS
Forty-four deaths were recorded of which 27 cases were cardiovascular. Fourteen patients died suddenly, six were successfully resuscitated, and seven patients died of congestive heart failure. The annual mortality, annual cardiac mortality, and annual mortality due to sudden death were 1.3%, 0.8%, and 0.6%, respectively. At least one New York Heart Association (NYHA) functional class deterioration was reported in 33% of the patients with a significant (> or =50 mm Hg) left ventricular outflow tract (LVOT) gradient in contrast to 7% without obstruction. The presence of syncope was related to SCD (p < 0.05). Younger age and more severe functional limitation distinguishes patients from in hospital-based centers from the ones in community-based centers.


CONCLUSIONS
Hypertrophic cardiomyopathy is a benign disease in an unselected population with a low incidence of cardiac death. Syncope was associated with a higher incidence of SCD and patients with a significant LVOT obstruction were more susceptible to clinical deterioration.
Kofflard
Marcel J M
MJ
Department of Cardiology, Albert Schweitzer Hospital, Dordrecht, The Netherlands.
Ten Cate
Folkert J
FJ
van der Lee
Chris
C
van Domburg
Ron T
RT
D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000453:epidemiology*; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006761:Hospitals / Q000706:statistics & numerical data*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care / Q000706:statistics & numerical data*; D012111:Residence Characteristics / Q000706:statistics & numerical data*; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate; D013997:Time Factors