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Health condition · Clinically reviewed

Cardiomyopathy, hypertrophic, dilated and restrictive — modern care.

Diseases of the heart muscle — genetic, acquired or amyloid. Cardiac MRI, genetics and modern therapies (mavacamten, tafamidis, ICD) transform outcomes.

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A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced, not summarised

    Every claim is checked against NICE, ESC, Cardiomyopathy UK or a peer-reviewed source you can see at the end.

  • 03

    Updated for 2026

    Reflects current UK and European guidance, including mavacamten for obstructive HCM and tafamidis for ATTR amyloid.

Key facts

Cardiomyopathy at a glance.

The essentials — what it is, how common, the main variants, how it is diagnosed and the therapies that now transform outcomes.

  • What it is

    A structural or functional disease of the heart muscle — not caused by coronary, valve or hypertensive disease.

  • Most common form

    Hypertrophic cardiomyopathy (HCM) affects roughly 1 in 500 people — most inherited.

  • The main variants

    HCM, dilated (DCM), restrictive, arrhythmogenic (ARVC) and cardiac amyloid (ATTR and AL).

  • How it is diagnosed

    Cardiac MRI and genetic testing are central — echocardiography and ECG start the pathway.

  • Landmark therapies

    Mavacamten for obstructive HCM and tafamidis for ATTR amyloidosis have transformed outcomes.

  • Sudden death prevention

    An implantable cardioverter defibrillator (ICD) prevents sudden death in high-risk HCM, DCM and ARVC.

Why this guide matters

A new era in heart-muscle disease.

Cardiac MRI, genetic testing and disease-modifying drugs have redefined what living with cardiomyopathy looks like — for patients and their families.

  • Imaging that sees the muscle

    Cardiac MRI reveals scar, oedema and amyloid — years before symptoms become severe.

  • Genes that guide the family

    A pathogenic variant means cascade screening — finding relatives before they present.

  • Drugs that change the trajectory

    Mavacamten for obstructive HCM and tafamidis for ATTR amyloid have transformed prognosis.

How the diagnosis is made

From first suspicion to a specialist plan.

The pathway a UK cardiologist will normally follow — from ECG to genetics to specialist inherited cardiac clinic.

  1. 01

    Recognising

    Family and symptom history

    A careful look at breathlessness, palpitations, syncope and any family history of sudden cardiac death.

  2. 02

    Recognising

    ECG

    The first-line test — often abnormal in HCM, DCM and ARVC before symptoms appear.

  3. 03

    Recognising

    Transthoracic echocardiogram

    Bedside imaging of muscle thickness, chamber size and function.

  4. 04

    Confirming

    Cardiac MRI

    The gold standard for tissue characterisation — scarring, infiltration and amyloid detection.

  5. 05

    Confirming

    Genetic testing

    For HCM, DCM and ARVC — guides family screening and, sometimes, therapy.

  6. 06

    Managing

    24-hour or longer rhythm monitor

    Looking for arrhythmia that raises sudden death risk or drives symptoms.

  7. 07

    Managing

    Specialist inherited cardiac clinic

    Multidisciplinary review — cardiologist, geneticist and specialist nurse — for you and at-risk relatives.

Typical pathway: 4–12 weeks from first suspicion to a specialist plan.

Symptoms

What cardiomyopathy actually feels like.

Symptoms vary by type — from silent HCM picked up on family screening to progressive heart failure in DCM. Here is what to look for.

  • Breathlessness on exertion

    Often the first symptom — from stiff or poorly contracting muscle failing to keep up with demand.

  • Syncope on exertion

    A red flag in cardiomyopathy — a marker of sudden death risk that needs urgent cardiology review.

  • Palpitations

    A fluttering or racing heartbeat — arrhythmia is common in HCM, DCM and ARVC.

  • Chest pain

    Can occur even without coronary disease — from thickened muscle or microvascular dysfunction.

  • Family history / sudden death

    A first-degree relative with cardiomyopathy or unexplained sudden death is itself a diagnostic clue.

  • Ankle oedema (DCM)

    Swelling in the ankles or legs from fluid retention when the heart is dilated and failing.

  • Restrictive symptoms (amyloid)

    Breathlessness with preserved ejection fraction, carpal tunnel, low blood pressure — the amyloid pattern.

  • Red flag — exertional syncope

    Fainting during exertion in anyone with known or suspected cardiomyopathy needs urgent cardiology review — sudden death risk.

Treatment

How cardiomyopathy is treated in the UK.

Treatment is tailored by subtype — from beta-blockers and mavacamten in HCM to tafamidis in ATTR amyloid and ICDs where sudden death risk is high.

  • Beta-blocker

    First-line symptom control in HCM and DCM — slows the heart and lowers outflow obstruction.

  • Mavacamten (obstructive HCM)

    A cardiac myosin inhibitor that reduces obstruction and symptoms — landmark therapy for obstructive HCM.

  • Standard heart failure therapy

    For DCM: ACE inhibitor or ARNI, beta-blocker, MRA and SGLT2 inhibitor — the four pillars.

  • Septal myectomy or alcohol ablation

    For severe outflow obstruction in HCM when medication is not enough.

  • ICD for high-risk patients

    Prevents sudden cardiac death in HCM, DCM or ARVC when risk stratification indicates.

  • Tafamidis for ATTR amyloidosis

    A transthyretin stabiliser that slows disease progression and improves survival in ATTR cardiac amyloid.

  • Anticoagulation for AF

    Atrial fibrillation is common — stroke risk is high in cardiomyopathy and warrants anticoagulation.

  • Heart transplant (end-stage)

    For advanced disease unresponsive to medical and device therapy — a small number of patients each year.

What this guide is based on

The sources behind every claim on this page.

UK and European guidance, specialist society standards and reputable patient charities, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your cardiologist knows your imaging, genetics and history and can tell you which parts apply to you.

  • National Institute for Health and Care Excellence (NICE). Hypertrophic cardiomyopathy — technology appraisals and guidance.

  • European Society of Cardiology (ESC). Guidelines on the management of cardiomyopathies.

  • Cardiomyopathy UK. Patient information on HCM, DCM, ARVC and cardiac amyloid.

  • British Society for Heart Failure. Position statements and clinical standards.

Red flags

When cardiomyopathy becomes an emergency.

Most cardiomyopathy is managed in clinic — but these situations need urgent specialist attention.

  • Exertional syncope

    Fainting during exertion in known or suspected cardiomyopathy — sudden death risk, urgent cardiology review.

  • Family history of sudden cardiac death

    A first-degree relative under 40 who died suddenly — you and your family need cascade screening.

  • Rapidly progressive heart failure

    New or worsening breathlessness, oedema and fatigue over days to weeks — same-week cardiology assessment.

  • Life-threatening arrhythmia

    Sustained ventricular tachycardia or resuscitated cardiac arrest — inpatient assessment for an ICD.

  • Post-ICD shock

    Any shock delivered by your ICD needs prompt device-clinic review, even if you feel well.

  • Amyloid multisystem features

    Carpal tunnel, autonomic symptoms, low BP or nephrotic syndrome with heart failure — think amyloid, refer.

  • Pregnancy planning

    Some cardiomyopathies carry major pregnancy risk — pre-pregnancy counselling in a specialist clinic is essential.

  • Post-transplant complications

    Rejection, infection or graft dysfunction — contact the transplant team immediately.

  • Genetic cascade needed

    A pathogenic variant identified in you means first-degree relatives should be offered screening.

Living with it

A lifelong condition, but far more treatable than it used to be.

Four things that make the biggest difference — activity, monitoring, medication and family screening.

A quiet reminder

You are not doing this alone.

Specialist inherited cardiac clinics look after you and your family together — cardiology, genetics and specialist nursing in one team.

  1. 01 Daily habits

    Activity, tailored to you

    Regular moderate activity is safe in most cardiomyopathies — competitive sport in HCM and ARVC needs individual advice.

  2. 02 Monitoring

    Regular specialist review

    Annual echocardiogram, ECG and rhythm monitor for most patients — MRI at longer intervals.

  3. 03 Medication

    Take it as prescribed

    Beta-blockers, HF drugs, mavacamten or tafamidis all work best when taken consistently, at the same time each day.

  4. 04 Family

    Screen the people you love

    First-degree relatives should be offered ECG, echocardiogram and, where indicated, genetic testing.

Frequently asked

Everything we get asked about cardiomyopathy.

Quick answers on subtype, mavacamten and tafamidis, ICDs and family screening.

  • What is cardiomyopathy?

    A disease of the heart muscle itself — not caused by coronary artery disease, valve disease or high blood pressure. The main forms are hypertrophic (HCM), dilated (DCM), restrictive, arrhythmogenic (ARVC) and cardiac amyloid.

  • How common is hypertrophic cardiomyopathy?

    HCM affects roughly 1 in 500 people in the UK, making it the most common inherited cardiac condition. Many people have no symptoms and are diagnosed through family screening.

  • What is mavacamten?

    Mavacamten is a first-in-class cardiac myosin inhibitor licensed for symptomatic obstructive hypertrophic cardiomyopathy. It reduces outflow tract obstruction, improves symptoms and can avoid the need for septal reduction therapy in many patients.

  • What is tafamidis and who needs it?

    Tafamidis is a transthyretin stabiliser used to treat cardiac amyloidosis caused by transthyretin (ATTR) deposits. It slows progression and improves survival — but only works in ATTR amyloid, not AL amyloid.

  • Do I need an ICD?

    An implantable cardioverter defibrillator prevents sudden cardiac death in patients whose risk score, family history, MRI scar burden or documented arrhythmia crosses a defined threshold. Your cardiologist will use a validated calculator to guide the decision.

  • Should my family be tested?

    Yes — first-degree relatives of anyone with an inherited cardiomyopathy should be offered ECG, echocardiogram and, where a pathogenic variant is found, targeted genetic testing. This cascade screening finds people early, before symptoms.

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