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Amyloid Cardiomyopathy

An infiltrative cardiomyopathy caused by the deposition of amyloid fibrils within the myocardium.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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What is Amyloid Cardiomyopathy?

Cardiac amyloidosis is an infiltrative disease in which insoluble amyloid fibrils deposit in the myocardial extracellular space and cause restrictive physiology and heart failure. AL (light chain) and ATTR (transthyretin) are the most common types.

ATTR typically occurs in older men, often with a history of carpal tunnel syndrome and spinal stenosis, while AL is associated with multiple myeloma and monoclonal gammopathies. The disease presents with refractory HFpEF, arrhythmias and conduction disturbances.

Diagnosis is made by echocardiography, cardiac MRI, DPD/PYP scintigraphy and, when needed, endomyocardial biopsy. Newer targeted agents (tafamidis, patisiran, inotersen) are changing the natural history of the disease.

Symptoms

Exertional dyspnea
Orthopnea and paroxysmal nocturnal dyspnea
Edema and weight gain
Orthostatic hypotension
Syncope and bradycardia
History of carpal tunnel syndrome
Macroglossia in AL type

Risk Factors

Advanced age (for ATTR)
Familial TTR mutation
Multiple myeloma and monoclonal gammopathy (AL)
Carpal tunnel syndrome and spinal stenosis
Biceps tendon rupture
Biventricular thickening on imaging
Autonomic neuropathy features

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Refractory heart failure symptoms
  • Advanced LVH out of proportion to age
  • New-onset conduction disturbance
  • Macroglossia or amyloidosis-specific features
  • Mismatch between low-voltage ECG and thick walls

Treatment Methods

01
Careful diuretic use
02
Tafamidis for ATTR type
03
Oncology-based chemotherapy for AL type
04
Gene-silencing agents such as patisiran and inotersen
05
Device therapy for arrhythmias and conduction disease
06
Follow-up at a multidisciplinary amyloidosis center

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.