Introduction
sudden death, syncope, chest pain, intolerance, palpitations, breathlessness, obstructive HCM, heart muscle disease
SUDDEN DEATH, CARDIAC DISORDER, HEART MUSCLE DISEASE, HEART, HYPERTROPHIC CARDIOMYOPATHY (HCM)
Hypertrophic cardiomyopathy (HCM), the most common inherited cardiac disorder, is a heart muscle disease most often caused by variants in one or more sarcomeric genes.Clinical manifestations are variable but can be profound. Two-thirds of patients have symptoms at diagnosis, including breathlessness, chest pain, effort intolerance, palpitations and syncope.Management traditionally comprises therapies to palliate symptoms and implantable cardioverter–defibrillators to prevent sudden death in patients deemed to be at high risk. More recently, trials of Mavacamten, an allosteric inhibitor of β-cardiac myosin ATPase activity, have demonstrated reduced LV outflow tract gradient and improved symptoms, exercise performance and health status in patients with symptomatic obstructive HCM (LV outflow tract gradient of 50 mm Hg or greater and New York Heart Association (NYHA) class II and III symptoms).