HOCM Flashcards
What is the cause of Hypertrophic Cardiomyopathy (HCM)?
mutations in sarcomere and sarcomere-related protein genes that affect the contractile machinery of the heart.
What is the most common identifiable cause of sudden cardiac death in healthy individuals under 35?
HCM is the most common identifiable cause of sudden cardiac death in this population.
What percentage of HCM patients have a known gene mutation?
About 60% of patients with HCM have a known gene mutation of the sarcomere or sarcomere-related genes.
What are the most commonly identified gene mutations in HCM patients?
β-myosin heavy chain (MYH7) and myosin-binding protein C3 (MYBPC3), which account for about 75% of variant-positive patients.
What is the commonest presentation of HCM in terms of age?
HCM is most commonly presented in the third decade of life, though it can occur at any age.
What is considered a hemodynamically significant gradient for LVOTO in HCM?
A gradient of 30 mm Hg or more is considered hemodynamically significant, with a gradient of 50 mm Hg or more being the threshold for surgical intervention.
What is the most common arrhythmia in HCM?
Atrial fibrillation is the most common arrhythmia in HCM, present in about 25% of patients.
A “jerky” pulse with rapid upstroke and downstroke is characteristic of
HCM.
How does chest pain present in HCM patients?
Chest pain can present as typical or atypical angina. It may worsen after a heavy meal (postprandial exacerbation) in individuals with LVOTO.
What physical examination findings are associated with HCM?
Physical exam findings include a “jerky” pulse, normal S1 and split S2, an S3 (due to decompensated heart failure), prominent “a wave” of jugular venous pressure, laterally displaced apical impulse, double carotid pulse, and an S4 (due to left ventricular hypertrophy).
What type of murmur may be present if mitral regurgitation is seen in HCM?
A holosystolic, blowing murmur may be loudest at the apex if mitral regurgitation is present.
What is the first-line treatment for symptomatic HCM?
Pharmacological therapy, including beta blockers, nondihydropyridine calcium channel blockers, and disopyramide.
What is Mavacamten, and how does it help treat obstructive HCM?
Mavacamten is a first-in-class drug approved in 2022. It works by inhibiting the interaction between myosin and actin in cardiac muscle cells, reducing left ventricular outflow tract (LVOT) obstruction and alleviating symptoms of obstructive HCM.
What is the role of beta-blockers in managing HCM?
Beta-blockers are the first line of treatment for most HCM cases. They should be titrated to a therapeutic dose that provides symptom relief, ensuring optimal treatment while minimizing adverse effects.
What are common side effects of Mavacamten?
Common side effects include dizziness, fainting, shortness of breath, and potential heart failure requiring dosage adjustment if left ventricular ejection fraction drops below 50%.
