Cardiomyopathie hypertrophique

Hypertrophic cardiomyopathy is characterized by an asymmetric hypertrophy of the left ventricle, especially in the interventricular septum. An outflow gradient in the left ventricle is present in twenty-five percent of cases. The disease is usually hereditary and genes responsible for the disease encode for the sarcomere proteins. The natural course is generally favourable but sometimes complications may occur, such as sudden death or congestive heart failure. Treatment remains difficult. If symptoms are not controlled by beta-blockers or verapamil, and if a pressure gradient is present, surgery with myotomy-myectomy may be proposed. Other treatments in such case are dual-chamber pacing or non-surgical septum reduction (alcohol injection). In patients with high-risk of sudden death, amiodarone or implantable cardioverter-defibrillator can be proposed.