From the Guidelines
Hypertrophic cardiomyopathy (HCM) management should prioritize a combination of lifestyle modifications, medications, and surgical/catheter interventions, as recommended by the 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR guideline 1. The goal of treatment is to reduce symptoms, prevent sudden cardiac death, and improve quality of life.
Key Management Strategies
- Beta-blockers like metoprolol (25-100 mg twice daily) or calcium channel blockers such as verapamil (120-360 mg daily) are commonly used to reduce heart rate, improve diastolic filling, and decrease outflow obstruction 1.
- Patients should avoid dehydration and strenuous exercise, particularly competitive sports.
- First-degree relatives should undergo screening with echocardiography and ECG to identify affected relatives 1.
- For severe cases with outflow tract obstruction, surgical myectomy or alcohol septal ablation may be considered, with intraoperative transesophageal echocardiography (TEE) and transthoracic echocardiography (TTE) guiding the procedures 1.
- Patients with high risk factors (family history of sudden death, severe hypertrophy >30mm, unexplained syncope, abnormal blood pressure response to exercise) may require implantable cardioverter-defibrillators for sudden death prevention.
Importance of Regular Follow-up
Regular cardiology follow-up with echocardiography is essential to monitor disease progression, assess for changes in left ventricular (LV) systolic and diastolic function, wall thickness, chamber size, LV outflow tract obstruction (LVOTO), and concomitant valvular disease 1.
Diagnostic Imaging
Comprehensive 2D echocardiography has a primary role in establishing the diagnosis of HCM, determining hypertrophy pattern, presence of LV apical aneurysms, LV systolic and diastolic function, mitral valve function, and presence and severity of LVOTO 1. Provocative maneuvers such as sustained Valsalva or squat-to-stand maneuvers can help uncover the presence of LVOTO, which may inform the care of the individual 1.
From the Research
Hypertrophic Cardiomyopathy Treatment
- Hypertrophic cardiomyopathy (HCM) is a genetic heart disease that can cause sudden cardiac death (SCD) and is the most common genetic heart disease 2.
- Treatment of obstructive HCM symptoms and gradient resistant to first-line therapy with β-blockade or verapamil is a topic of controversy 3.
- A study of 737 patients with HCM found that 41% required further therapy for obstruction, with 74% of these patients being treated with disopyramide, resulting in pharmacological control of symptoms in 64% of patients 3.
Treatment Options
- β-blockade or verapamil are commonly used as first-line therapy for HCM, but some patients may not respond to these treatments 3, 2.
- Disopyramide can be added to β-blockade or verapamil in patients who do not respond to first-line therapy, with 64% of patients achieving pharmacological control of symptoms 3.
- Surgical myectomy is also an option for patients with obstruction and symptoms resistant to initial pharmacological therapy, with 91% of patients undergoing this procedure in one study 3.
- Metoprolol and verapamil have been shown to be effective in reducing left ventricular outflow tract (LVOT) gradients and improving quality of life in patients with HCM, with metoprolol being better tolerated in one study 2.
Outcomes
- A study of 299 patients with HCM found that stepped management, including the addition of disopyramide and surgical myectomy, resulted in meaningful symptom relief and low mortality, with a 10-year survival rate of 88% 3.
- The annual rate of sudden death in this study was 0.06%/y, which is low compared to other studies 3.
- Metoprolol and verapamil have been shown to be safe to use with a good side effect profile, although metoprolol was better tolerated in one study 2.