DISEASE SCANNER

Global Incurable Diseases Tracker

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Cardiac Disease

Cardiac Amyloidosis

HIGH SEVERITY

Buildup of abnormal amyloid protein in the heart tissue causing stiffening and restrictive cardiomyopathy. Two main types: ATTR (transthyretin, age-related or hereditary) and AL (light chain, associated with multiple myeloma). Increasingly recognized cause of heart failure with preserved ejection fraction.

Global Affected

500.0K

Countries

89

Symptoms

Shortness of breath
Fatigue
Swelling in legs
Arrhythmias (especially AF)
Dizziness/syncope
Chest pain
Orthostatic hypotension
Carpal tunnel syndrome (ATTR)

Treatment Options

Tafamidis (ATTR)
Daratumumab (AL)
Chemotherapy (AL)
Autologous stem cell transplant (AL)
Heart transplant
Liver transplant (hereditary ATTR)
Diuretics
AF management
Anticoagulation

Risk Factors

1Age >65 (wild-type ATTR)
2Genetic mutations (hereditary ATTR)
3Multiple myeloma (AL)
4Male gender
5African ancestry (V122I variant)
6Family history (hereditary)

Diagnostic Methods

  • 1Cardiac MRI
  • 2Technetium pyrophosphate scan (ATTR)
  • 3Serum free light chains (AL)
  • 4Endomyocardial biopsy (gold standard)
  • 5Echocardiogram
  • 6Genetic testing
  • 7Fat pad biopsy

Prognosis

Poor if untreated. ATTR: median survival 2-5 years without tafamidis, improved with treatment. AL: depends on underlying plasma cell disorder. Early treatment significantly improves outcomes.

Prevention

  • Genetic counseling (hereditary forms)
  • Early diagnosis
  • Management of underlying conditions
  • Regular cardiac monitoring (at-risk)

Research Status

Tafamidis stabilizes transthyretin and improves survival in ATTR. Daratumumab for AL amyloidosis. Heart transplant for end-stage disease. Liver transplant for hereditary ATTR. Early diagnosis critical for treatment benefit.

Affected Countries

United StatesUnited KingdomCanadaAustraliaGermanyFranceItalySpainJapanBrazilMexicoRussiaChinaIndiaSwedenNorwayFinlandNetherlandsBelgiumSwitzerlandAustriaDenmarkPolandCzech RepublicHungaryRomaniaBulgariaCroatiaSloveniaSlovakiaLTLVEEIrelandPortugalGreeceCYMTLUIcelandNew ZealandSouth KoreaTaiwanSingaporeHong KongMalaysiaThailandIndonesiaPhilippinesVietnamSouth AfricaEgyptTurkeySaudi ArabiaUAEQatarKuwaitBHOmanJordanLebanonSyriaIraqIranPakistanBangladeshLKMMKHLAMNKPUzbekistanKazakhstanKGTJTMAzerbaijanAMGEUkraineBelarusMDALBAMKMESerbiaXK

Sources

  • https://www.mayoclinic.org/diseases-conditions/amyloidosis
  • https://www.heart.org/en/health-topics/cardiomyopathy
  • https://my.clevelandclinic.org/health/diseases/22642-cardiac-amyloidosis

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.