Introduction: Hypertrophic cardiomyopathy (HCM) is a genetic, heterogenous, disease with a prevalence of 1/500. Symptoms are exertional dyspnea, angina, dizziness or syncope. In some patients there seem to be an increased risk of sudden cardiac death. Life threatening ventricular arrhythmias can be treated by an ICD. However it is a progressive disease with a risk of congestive heart failure.
Methods: : From The Swedish ICD-Registry we found 370 patients with an ICD due to HCM between 1995 and 2012. A total of 50 patients died during the same period. The Cause of Death Registry classified the main cause of death in 42 of these until 2010. Main cause of death. N= numder of patients. Cardiac causes were HCM (n=23), acute myocardial infarction (n=4) and ischemic heart disease (n=2) Age at time of death of the total 50 patients with an ICD due to hypertrophic cardiomyopathy. Remaining life expectancy for a 50 year old man is currently 31.5 years and for a women 34.5 (Statistics, Sweden 2012).
Results: In the Swedish Cause of Death Registry we found cardiac causes as the main cause in 71% of patients with HCM and ICD. In 93% (39/42) this was a contributing cause of death. Average age at death was 67.5 yr (median 69) compared to normal population 81.5 yr for men and 84.6 yr for women
Conclusions: Cardiac causes of death are still the limit for a normal life expectancy in HCM patients with an ICD.