Cardiac Amyloidosis: an Approach to Diagnosis and Management
Cardiac Amyloidosis: an Approach to Diagnosis and Management
Cardiac amyloidosis is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart. Cardiac amyloidosis has a wide spectrum of clinical manifestations but the most frequent presentation is heart failure. Differential diagnoses from other restrictive cardiomyopathies is important. A combination of clinical, electrocardiographic and imaging methods is commonly used to diagnose this disease. Definite diagnosis is based on endomyocardial biopsy and treatment of cardiac amyloidosis is a challenge. Heart transplantation, although controversial, has demonstrated survival benefit.
Amyloidosis is a clinical disorder caused by extracellular deposition of high-molecular-weight proteins. The typical fibrillar structure confers the characteristic resistance to proteolytic degradation. To date, at least 21 different proteins have been recognized as causative agents of amyloid diseases. Despite having heterogeneous structures and functions, all of these proteins can generate morphologically indistinguishable amyloid fibrils.
Cardiac involvement depends on the type of amyloid disease. Systemic amyloidosis occurs in more than 10 per million person-years in the USA. It accounts for 1 in 1000 deaths in the British population, and is probably underdiagnosed in the elderly population. Systemic amyloidosis is the most serious and commonly diagnosed form, with 2000–2500 new cases annually in the USA.
Amyloidosis is the most frequent cause of restrictive cardiomyopathy in the Western world. Unfortunately, this disease is generally misdiagnosed and its natural history and outcomes differ from other causes of heart failure. Cardiac amyloidosis portends a poor prognosis with a median survival of less than 6 months without treatment. However, if recognized early there are specific treatments available. Early diagnosis and treatment of patients with cardiac compromise is crucial.
Abstract and Introduction
Abstract
Cardiac amyloidosis is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart. Cardiac amyloidosis has a wide spectrum of clinical manifestations but the most frequent presentation is heart failure. Differential diagnoses from other restrictive cardiomyopathies is important. A combination of clinical, electrocardiographic and imaging methods is commonly used to diagnose this disease. Definite diagnosis is based on endomyocardial biopsy and treatment of cardiac amyloidosis is a challenge. Heart transplantation, although controversial, has demonstrated survival benefit.
Introduction
Amyloidosis is a clinical disorder caused by extracellular deposition of high-molecular-weight proteins. The typical fibrillar structure confers the characteristic resistance to proteolytic degradation. To date, at least 21 different proteins have been recognized as causative agents of amyloid diseases. Despite having heterogeneous structures and functions, all of these proteins can generate morphologically indistinguishable amyloid fibrils.
Cardiac involvement depends on the type of amyloid disease. Systemic amyloidosis occurs in more than 10 per million person-years in the USA. It accounts for 1 in 1000 deaths in the British population, and is probably underdiagnosed in the elderly population. Systemic amyloidosis is the most serious and commonly diagnosed form, with 2000–2500 new cases annually in the USA.
Amyloidosis is the most frequent cause of restrictive cardiomyopathy in the Western world. Unfortunately, this disease is generally misdiagnosed and its natural history and outcomes differ from other causes of heart failure. Cardiac amyloidosis portends a poor prognosis with a median survival of less than 6 months without treatment. However, if recognized early there are specific treatments available. Early diagnosis and treatment of patients with cardiac compromise is crucial.
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