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Hypertrophic cardiomyopathy (HCM) is a relatively common disease of the cardiac sarcomere with broad diversity in terms of disease-causing gene mutation, phenotypic expression, therapy, and prognosis. The heterogeneity of the disease is featured by the fact that it burdens patients of all ages. HCM is the most prevalent cause of sudden death in athletes, but several technological advancements in implantable defibrillators and pacemakers have offered new therapeutic options for HCM patients and reduced mortality to 0.5% per year. In addition, rapid advances in our knowledge of the molecular defects accountable for HCM have strengthened our awareness of the disorder and recommended new approaches to the assessment of prognosis. Despite all these evolutions, a small subgroup of patients with HCM will experience sudden cardiac death, and risk stratification remains a critical challenge. This review provides a practical guide to the updated recommendations for patients with HCM, including clinical updates for diagnosis, family screening, clinical imaging, risk stratification, and management. |
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