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Amyloid cardiomyopathy

Field	Value
synonyms
image	Echocardiographic findings of primary systemic AL amyloidosis.jpg
caption	Apparent left ventricular hypertrophy and “snowstorm” appearance of the myocardium are suggestive of an infiltrative cardiomyopathy.
specialty	Cardiology
symptoms	diastolic dysfunction, congestive heart failure, arrhythmia, cardiac nervous conduction block, fatigue, dyspnea.
complications	Congestive heart failure, atrial arrhythmia, ventricular arrhythmia, and blockage of cardiac nervous conduction.
causes
risks
diagnosis	Electrocardiography and Cardiac mri
treatment	Chemotherapy or liver transplant
prognosis	5-year survival rate of 10%
frequency
named after

Amyloid cardiomyopathy (stiff heart syndrome) is a condition resulting in the death of part of the myocardium (heart muscle). It is associated with the systemic production and release of many amyloidogenic proteins, especially immunoglobulin light chain or transthyretin (TTR). It can be characterized by the extracellular deposition of amyloids, foldable proteins that stick together to build fibrils in the heart.

Symptoms

Amyloid cardiomyopathy is associated with a number of symptoms:

diastolic dysfunction
congestive heart failure
arrhythmia
cardiac nervous conduction block
fatigue
dyspnea

Pathophysiology

Amyloid proteins are deposited in the myocardium. This limits ventricular filling during diastole, which increases end-diastolic volume. This can lead to a variety of cardiac issues, such as congestive heart failure, atrial arrhythmia, ventricular arrhythmia, and blocks to cardiac nervous conduction.

Diagnosis

Diagnosis is often delayed, because its symptoms are non-specific.

Electrocardiography can be used to identify low voltage and patterns similar to those of a heart attack.

Cardiac MRI can be used to distinguish it from hypertensive heart disease. This shows a thicker interventricular septum.

Treatment

Chemotherapy can treat amyloidosis if it is related to immunoglobulins. Liver transplant can treat amyloidosis if it is related to familial transthyretin.

Acoramidis (Attruby) was approved for medical use in the United States in November 2024, to treat adults with cardiomyopathy of wild-type or variant (hereditary) transthyretin-mediated amyloidosis (ATTR-CM) to reduce death and hospitalization related to heart problems.

Prognosis

Outcomes for amyloid cardiomyopathy are generally very poor, with fewer than 10% of patients surviving more than 5 years. Without treatment, few patients survive more than 6 months.

Epidemiology

In developed countries, amyloid cardiomyopathy is estimated to be involved in 0.1% of deaths.

References

"Cardiac amyloidosis: MedlinePlus Medical Encyclopedia".
(2017-06-09). "Amyloid Cardiomyopathy: Disease on the Rise". Circulation Research.
(2010-01-01). "Amyloid Heart Disease". Progress in Cardiovascular Diseases.
(2016-01-01). "Chapter 7 - Subtypes of Cancer Involving the Heart". Elsevier.
Benson, Merrill D.. (2013-01-01). "Chapter 79 - Amyloidosis and Other Protein Deposition Diseases". Academic Press.
(2012-01-01). "Chapter 43 - Infiltrative and Protein Misfolding Myocardial Diseases". Academic Press.
(2018-01-01). "Amyloid Cardiomyopathy". Elsevier.
(1 October 2024). "FDA approves drug for heart disorder caused by transthyretin-mediated".
Chen, Elaine. (2024-11-23). "BridgeBio wins FDA approval for heart disease drug".

Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

cardiomyopathy

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