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CLINICAL STUDY: HYPERTROPHIC CARDIOMYOPATHY

Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration

Marcel J. M. Kofflard, MD, Folkert J. Ten Cate, MD, FACC^*,*, Chris van der Lee, MD^* and Ron T. van Domburg, MSc^*

^* Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands
Albert Schweitzer Hospital Department of Cardiology, Dordrecht, The Netherlands

Manuscript received June 11, 2002; revised manuscript received October 17, 2002, accepted October 31, 2002.

^* Reprint requests and correspondence: Dr. Folkert J. Ten Cate, Thoraxcenter Ba 304, Erasmus MC, Dr. Molewaterplein 40, 3015 GD Rotterdam, The Netherlands.
tencate{at}card.azr.nl

OBJECTIVES: 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 (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.

Abbreviations and Acronyms   AF   atrial fibrillation   CI   confidence interval   HCM   hypertrophic cardiomyopathy   LVOT   left ventricular outflow tract   NYHA   New York Heart Association   RR   relative risk   SCD   sudden cardiac death   VT   ventricular tachycardia

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