7th March 2024

Treatment

The management of hypertrophic cardiomyopathy is very dependent upon the extent of the pathology and the consequent symptoms.

For patients with significant arrhythmias, these can be treated as normal, though in the context of atrial fibrillation, drugs such as flecainide are typically avoided.

For patients with left ventricular outflow tract (LVOT) gradients, beta blockers, or rate limiting calcium channel blockers are effective at reducing this by acting as negative inotropes which reduces the risk of outflow tract obstruction.

Mavacamten is a myosin-actin cross-bridge inhibitor, a novel agent which, at the time of writing, is not yet approved for use in HCM but appears to have a positive impact on LVOT gradient, NYHA functional class, dyspnoea scores and NT pro-BNP levels in symptomatic HCM patients.

Patients with symptoms due to outflow tract obstruction refractory to drug therapy can be considered for a surgical myomectomy or alcohol septal ablation to reduce the outflow tract gradient.

Online calculators exist to predict 5-year risk of sudden cardiac death. For patients with a 5-year risk of SCD <4%, an ICD is generally not indicated, in patients with a risk of 4 to less than 6%, an ICD may be considered and in patients with a 5-year risk ≥6%, an ICD should be considered.

First degree relatives of patients with HCM should be screened with echocardiography and ECG to look for the typical abnormalities as described above. The children of affected children should be screened routinely until the age of 20. If HCM is not discovered by this stage, then it is unlikely to occur in later life.