Amyloid cardiomyopathy

Amyloid cardiomyopathy

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  Echocardiographic findings of primary systemic AL amyloidosis

Amyloid Cardiomyopathy

Amyloid cardiomyopathy is a form of heart disease caused by the deposition of amyloid proteins in the myocardium, the muscular tissue of the heart. This condition is a type of restrictive cardiomyopathy, which is characterized by the stiffening of the heart walls, leading to impaired filling of the heart chambers and reduced cardiac output.

Pathophysiology

Amyloid cardiomyopathy occurs when misfolded amyloid proteins accumulate in the heart tissue. These proteins can be of different types, but the most common in cardiac involvement are light chain amyloidosis (AL amyloidosis) and transthyretin amyloidosis (ATTR amyloidosis). In AL amyloidosis, the amyloid proteins are derived from immunoglobulin light chains, while in ATTR amyloidosis, they are derived from the transthyretin protein.

The deposition of amyloid proteins in the myocardium leads to increased stiffness of the heart walls, which impairs diastolic filling and can eventually lead to heart failure. The condition can also affect the electrical conduction system of the heart, leading to arrhythmias.

Clinical Presentation

Patients with amyloid cardiomyopathy may present with symptoms of heart failure, such as dyspnea, fatigue, and edema. Other symptoms may include orthostatic hypotension, syncope, and palpitations. The disease can progress to cause severe heart failure and may be associated with other systemic manifestations of amyloidosis, depending on the type.

Diagnosis

The diagnosis of amyloid cardiomyopathy involves a combination of clinical evaluation, imaging studies, and laboratory tests. Echocardiography is often used to assess cardiac structure and function, revealing thickened ventricular walls and a "sparkling" appearance of the myocardium. Cardiac MRI can provide further detail on tissue characteristics. A definitive diagnosis often requires a biopsy of the heart tissue, demonstrating amyloid deposits.

Laboratory tests may include serum and urine protein electrophoresis, immunofixation, and measurement of serum free light chains to identify AL amyloidosis. Genetic testing may be used to identify mutations associated with hereditary ATTR amyloidosis.

Treatment

The treatment of amyloid cardiomyopathy depends on the type of amyloidosis. For AL amyloidosis, treatment focuses on reducing the production of amyloidogenic light chains, often using chemotherapy agents similar to those used in multiple myeloma. For ATTR amyloidosis, treatment may involve the use of transthyretin stabilizers, such as tafamidis, or gene-silencing therapies.

Supportive care for heart failure symptoms is also important and may include the use of diuretics, beta-blockers, and other heart failure medications. In some cases, heart transplantation may be considered for eligible patients.

Prognosis

The prognosis of amyloid cardiomyopathy varies depending on the type of amyloidosis and the extent of cardiac involvement. AL amyloidosis generally has a poorer prognosis compared to ATTR amyloidosis, but advances in treatment have improved outcomes for many patients.

Related Pages

• Cardiomyopathy
• Heart failure
• Light chain amyloidosis
• Transthyretin amyloidosis
• Echocardiography

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