5th July 2026

Management

General Management 

• Avoid dehydration, excessive alcohol consumption and exposure to drastic weather conditions (humidity, heat). 
• Weight loss and regular low- to moderate-intensity exercise are advisable in all individuals with cardiomyopathy. 
• High-intensity exercise and competitive sports are not recommended in high-risk individuals and in individuals with LVOTO and exercise-induced ventricular arrhythmia. 
• Cardiopulmonary exercise testing should be performed every 2-3 years in stable patients, or sooner in those with progressive symptoms.

Medical Management 

• Beta-blockers or non-dihydropyridine calcium channel blockers are first-line therapies to improve diastolic filling and reduce outflow tract gradient. 
• Disopyramide, a class Ia antiarrhythmic agent, can be added in HCM patients with refractory LVOTO symptoms. 
• Mavacamten, a novel cardiac myosin ATPase inhibitor, has also demonstrated an improvement in LVOT gradient and exercise performance in patients with LVOTO in a recent phase III, double-blind, placebo-controlled trial. 
• HCM patients with LV systolic dysfunction should be started on guideline-directed medical therapy for HF. 
  • It is recommended that diuretics are started cautiously in patients with LVOTO to avoid exacerbating symptoms. 
• Amiodarone can be used to suppress atrial and ventricular arrhythmias in HCM patients refractory to beta-blockers. 
• All HCM patients with atrial fibrillation must be started on oral anticoagulants irrespective of CHA₂DS₂-VA to reduce thromboembolic risk.

Surgical Management 

• Device therapy
  • Implantable cardiac defibrillator (ICD) is recommended in HCM patients who have survived a cardiac arrest due to a ventricular arrhythmia, or who have sustained VT resulting in haemodynamic compromise. 
  • The HCM Risk-SCD calculator should be utilised as a tool to risk-stratify HCM patients over the age of 16 years for sudden cardiac death (SCD) at 5 years. It encompasses family history of SCD, NSVT, age, history of unexplained syncope, LVOT gradient, LV wall thickness and LA diameter.  
  • Primary prevention ICD is indicated in patients with a risk score ≥6% with life expectancy over one year and should be considered on an individual basis if 4-6%. 
  • Cardiac resynchronisation therapy (CRT) can be assessed in individuals with persistent LV dysfunction with left bundle branch block and QRS interval ≥130ms.
• Surgery
  • Surgical myomectomy ought to be considered in patients with obstructive symptoms refractory to medical therapy and is performed in combination with mitral valve repair. 
  • Alcohol septal ablation is an alternative to surgical myomectomy to reduce LVOT gradient. 
  • Cardiac transplantation is only considered in eligible patients with NYHA class III-IV symptoms or ventricular arrhythmias refractory to medical and device therapy.