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Published on: February 14, 2022
Amyloid diseases of the heart: current and future therapies
1Department of Cardiology, Hillingdon Hospital, Pield Heath Road, Uxbridge, Middlesex, UB8 3NN, UK. simon.dubrey@thh.nhs.uk
Insights
Amyloidosis, a heart disease from protein buildup, has many types. Accurate diagnosis is crucial for effective treatment of these amyloid cardiomyopathies.
Area of Science:
- Cardiology
- Internal Medicine
- Genetics
Background:
- Amyloid diseases arise from over 23 precursor proteins.
- Cardiologists commonly see light chain (AL) amyloidosis, senile systemic amyloidosis (SSA), and hereditary transthyretin amyloidosis.
- Secondary amyloidosis (AA) is prevalent in developing nations due to infections.
Purpose of the Study:
- To review the diverse spectrum of amyloid cardiomyopathies.
- To highlight the importance of accurate amyloid typing for treatment.
- To discuss various therapeutic approaches for cardiac amyloidosis.
Main Methods:
- Literature review of amyloidosis types and their cardiac manifestations.
- Analysis of clinical outcomes based on amyloid type and disease extent.
- Discussion of diagnostic and therapeutic strategies.
Main Results:
- Heart involvement in amyloidosis can have severe consequences.
- Clinical outcomes vary significantly based on amyloid type and systemic spread.
- Non-transthyretin variants and isolated atrial amyloid have different clinical implications.
Conclusions:
- Precise determination of amyloid type is essential for guiding therapy.
- Effective management of amyloid cardiomyopathies requires understanding the specific amyloid protein involved.
- This review outlines critical treatment approaches for various amyloidosis types affecting the heart.
Abstract:
Amyloid diseases in man are caused by as many as 23 different pre-cursor proteins already described. Cardiologists predominantly encounter three main types of amyloidosis that affect the heart: light chain (AL) amyloidosis, senile systemic amyloidosis (SSA) and hereditary amyloidosis, most commonly caused by a mutant form of transthyretin. In the third world, secondary amyloid (AA) is more prevalent, due to chronic infections and inadequately treated inflammatory conditions. Much less common, are the non-transthyretin variants, including mutations of fibrinogen, the apolipoproteins apoA1 and apoA2 and gelsolin. These rarer types do not usually cause significant cardiac compromise. Occurring worldwide, later in life and of less clinical significance, isolated atrial amyloid (IAA) also involves the heart. Heart involvement by amyloid often has devastating consequences. Clinical outcome depends on amyloid type, the extent of systemic involvement and the treatment options available. An exact determination of amyloid type is critical to appropriate therapy. In this review we describe the different approaches required to treat this spectrum of amyloid cardiomyopathies.
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