Cardiomyopathies

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

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

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.

References

1.2023 ESC Guidelines for the management of cardiomyopathies (2023)