Red Flags
- Sudden cardiac death survivor with structural heart disease — secondary prevention ICD; cascade family screening[1]
- Hypertrophic cardiomyopathy with syncope and high SCD risk score — primary prevention ICD discussion[1]
- New severe LV dysfunction in pregnancy or peripartum (PPCM) — admit; HF therapy compatible with pregnancy/lactation; multidisciplinary care[1]
- Family history of premature SCD or unexplained cardiac arrest — clinical and genetic screening of first-degree relatives[1]
First-line treatment
Interventions
- Implantable cardioverter-defibrillator (ICD)[1]Secondary prevention after cardiac arrest or sustained VT. Primary prevention per validated risk scores (HCM Risk-SCD, DCM-LVEF + LGE, ARVC TF risk)
- Septal reduction (myectomy or alcohol ablation)[1]Symptomatic obstructive HCM with LVOT gradient ≥50 mmHg and persistent NYHA III–IV despite optimal medical therapy
- Genetic counselling and family screening[1]All probands with confirmed cardiomyopathy of suspected genetic aetiology; cascade clinical and genetic screening of first-degree relatives
First-line drug therapy
| Drug | Class | Adult | Paediatric | Notes |
|---|---|---|---|---|
| Mavacamten[1] | Cardiac myosin inhibitor | 5 mg PO once daily, titrate per echocardiographic LVOT gradient and LVEF | — | Symptomatic obstructive HCM with insufficient response to beta-blocker; monitor LVEF every 4 weeks during titration |
| Bisoprolol or metoprolol[1] | Beta-blocker | Bisoprolol 1.25–10 mg daily; metoprolol succ 25–200 mg daily | — | First-line in obstructive HCM; symptom-driven dose titration |
| Disopyramide[1] | Class IA antiarrhythmic | 100–250 mg PO QID (or sustained release equivalent) | — | Alternative for symptomatic obstructive HCM intolerant or refractory to beta-blocker |
| Tafamidis[1] | Transthyretin stabiliser | 61 mg PO once daily | — | ATTR cardiac amyloidosis; reduces all-cause mortality and CV hospitalisation |
Mavacamten[1]
Cardiac myosin inhibitor
- Adult
- 5 mg PO once daily, titrate per echocardiographic LVOT gradient and LVEF
- Paediatric
- —
Symptomatic obstructive HCM with insufficient response to beta-blocker; monitor LVEF every 4 weeks during titration
Bisoprolol or metoprolol[1]
Beta-blocker
- Adult
- Bisoprolol 1.25–10 mg daily; metoprolol succ 25–200 mg daily
- Paediatric
- —
First-line in obstructive HCM; symptom-driven dose titration
Disopyramide[1]
Class IA antiarrhythmic
- Adult
- 100–250 mg PO QID (or sustained release equivalent)
- Paediatric
- —
Alternative for symptomatic obstructive HCM intolerant or refractory to beta-blocker
Tafamidis[1]
Transthyretin stabiliser
- Adult
- 61 mg PO once daily
- Paediatric
- —
ATTR cardiac amyloidosis; reduces all-cause mortality and CV hospitalisation
Safety-net
Referral criteria
- Confirmed cardiomyopathy or strong clinical suspicionCardiology specialist (preferably inherited cardiac conditions clinic)[1]
- Family history of premature SCD or sudden unexplained deathInherited cardiac conditions clinic for cascade screening[1]
- Symptomatic obstructive HCM despite medical therapyHCM centre for septal reduction or mavacamten initiation[1]
- Peripartum cardiomyopathyMultidisciplinary cardiology + obstetrics team[1]
Clinical summary
Diagnosis and management of hypertrophic, dilated, restrictive, arrhythmogenic, and non-classified cardiomyopathies, including genetic and pregnancy considerations.