Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: Dutch cardiologists and the care of mutation carriers Background. Patients with hypertrophic cardiomyopathy (HCM) and HCM mutation carriers are at risk of sudden cardiac death (SCD). Both groups should therefore be subject to regular cardiological testing -including risk stratification for SCD -according to international guidelines. We evaluated Dutch cardiologists' knowledge of and adherence to international guidelines on risk stratification and prevention of SCD in mutation carriers with and without manifest HCM. Methods. A questionnaire was sent to 1109 Dutch cardiologists (in training) containing case-based questions. Results. The response rate was 21%. Own general knowledge on HCM care was rated as insufficient by 63% of cardiologists. The percentage of correct answers (i.e. in agreement with international guidelines), on the case-based questions ranged from 37 to 96%, being lowest in cases with an unknown number of risk factors for SCD. A substantial portion of correct answers was based on the correct answer 'ask an expert opinion'. Significantly more correct answers were provided in cases with manifest HCM. There was little difference between the answers of cardiologists with different self-reported levels of knowledge, with different numbers of HCM patients in their practice or with different numbers of carriers without manifest HCM. Conclusion. Knowledge on risk stratification and preventive therapy was mediocre, and knowledge gaps exist, especially on HCM mutation carriers without manifest disease. Fortunately, experts are frequently asked for their opinion which might bring patient care to an adequate level. Hopefully, our results will stimulate cardiologists to follow developments in this field, thereby increasing quality of care for HCM patients and mutation carriers. (Neth Heart J 2009:17:464-9.).Keywords: Hypertrophic cardiomyopathy, risk stratification, sudden cardiac death, questionnaire With a prevalence of about one in 500, hypertrophic cardiomyopathy (HCM) is one of the most common genetic diseases.1 HCM is inherited as an autosomal dominant trait caused by a large variety of mutationsaround 450 different mutations in more than 11 genes have been reported.2 HCM is clinically classified as a (usually asymmetric) thickening of the myocardial wall of the left ventricle, often involving the interventricular septum, which cannot be accounted for by other clinical diseases. 3,4 Most patients experience few if any symptoms. If present, symptoms are diverse. Common symptoms are chest pain during exertion and dyspnoea.
5The most feared complication of HCM is sudden cardiac death (SCD) with an annual incidence of 1% in unselected HCM patients. 6 Although SCD in HCM predominantly occurs in young asymptomatic adults According to a consensus document from the American College of Cardiology (ACC) and the European Society of Cardiology (ESC), HCM patients (with manifest disease) and mutation carriers (with or without manifest disea...