Hypertrophic cardiomyopathy

Red Flags

Sudden cardiac death survivor with HCM — secondary prevention ICD; cascade family screening^[1]
Syncope on exertion in HCM — high SCD risk; primary prevention ICD discussion^[1]
Symptomatic LVOT obstruction (gradient ≥50 mmHg) refractory to optimal medical therapy — septal reduction or cardiac myosin inhibitor^[1]
Family history of premature SCD with HCM phenotype — clinical and genetic cascade screening of first-degree relatives^[1]

First-line treatment

Interventions

Septal reduction therapy^[1]
Myectomy or alcohol septal ablation for symptomatic obstructive HCM with LVOT gradient ≥50 mmHg and persistent NYHA III–IV despite optimal medical therapy
Implantable cardioverter-defibrillator^[1]
Secondary prevention after cardiac arrest or sustained VT. Primary prevention guided by HCM Risk-SCD calculator integrated with LGE burden, apical aneurysm, and family history
Genetic counselling and cascade screening^[1]
All probands with HCM; first-degree relatives offered clinical screening every 1–2 years and cascade genetic testing

First-line drug therapy

Drug	Class	Adult	Paediatric	Notes
Mavacamten^[1]	Cardiac myosin inhibitor	5 mg PO once daily, titrate per LVOT gradient and LVEF (range 2.5–15 mg)	—	Symptomatic obstructive HCM with insufficient response to beta-blocker; monitor LVEF every 4 weeks during titration; reduces need for septal reduction in EXPLORER-HCM and VALOR-HCM
Aficamten^[1]	Cardiac myosin inhibitor (next-generation)	5–20 mg PO once daily per dose-titration protocol	—	SEQUOIA-HCM data: improved exercise capacity in symptomatic obstructive HCM; emerging alternative to mavacamten
Bisoprolol or metoprolol succinate^[1]	Beta-blocker (first-line)	Bisoprolol 1.25–10 mg daily; metoprolol succ 25–200 mg daily	—	First-line in obstructive and symptomatic non-obstructive HCM
Disopyramide^[1]	Class IA antiarrhythmic (negative inotrope)	100–250 mg PO QID (or sustained-release equivalent)	—	Alternative for symptomatic obstructive HCM intolerant or refractory to beta-blocker; useful before mavacamten or septal reduction
Verapamil^[1]	Non-DHP calcium channel blocker	120–480 mg PO daily (sustained release)	—	Alternative when beta-blocker contraindicated; AVOID in severe LVOT obstruction with elevated PCWP

Mavacamten^[1]

Cardiac myosin inhibitor

Adult: 5 mg PO once daily, titrate per LVOT gradient and LVEF (range 2.5–15 mg)
Paediatric: —

Symptomatic obstructive HCM with insufficient response to beta-blocker; monitor LVEF every 4 weeks during titration; reduces need for septal reduction in EXPLORER-HCM and VALOR-HCM

Aficamten^[1]

Cardiac myosin inhibitor (next-generation)

Adult: 5–20 mg PO once daily per dose-titration protocol
Paediatric: —

SEQUOIA-HCM data: improved exercise capacity in symptomatic obstructive HCM; emerging alternative to mavacamten

Bisoprolol or metoprolol succinate^[1]

Beta-blocker (first-line)

Adult: Bisoprolol 1.25–10 mg daily; metoprolol succ 25–200 mg daily
Paediatric: —

First-line in obstructive and symptomatic non-obstructive HCM

Disopyramide^[1]

Class IA antiarrhythmic (negative inotrope)

Adult: 100–250 mg PO QID (or sustained-release equivalent)
Paediatric: —

Alternative for symptomatic obstructive HCM intolerant or refractory to beta-blocker; useful before mavacamten or septal reduction

Verapamil^[1]

Non-DHP calcium channel blocker

Adult: 120–480 mg PO daily (sustained release)
Paediatric: —

Alternative when beta-blocker contraindicated; AVOID in severe LVOT obstruction with elevated PCWP

Safety-net

Inform first-degree relatives of your diagnosis — they may need echocardiographic and genetic screening^[1]
Avoid intense competitive sport without explicit cardiology approval — sudden cardiac death risk^[1]
New syncope, chest pain, or palpitations — same-day cardiology review^[1]

Referral criteria

Confirmed HCM or strong clinical suspicion (LV wall thickness ≥15 mm or ≥13 mm with family history)Inherited cardiac conditions clinic with HCM expertise^[1]
Symptomatic obstructive HCM despite optimal medical therapyHCM centre for mavacamten/aficamten or septal reduction evaluation^[1]
Family history of premature SCD or sudden unexplained deathInherited cardiac conditions clinic for cascade screening^[1]

Clinical summary

HCM-focused diagnosis, sudden death risk stratification, and pharmacological + interventional management with cardiac myosin inhibitors and septal reduction.

References

1.EJHF Expert Consensus Statement on the diagnosis and management of hypertrophic cardiomyopathy (2026); 2023 ESC Cardiomyopathies; 2024 AHA/ACC HCM (2026)