From the Guidelines
Amyloid infiltration of the heart, also known as cardiac amyloidosis, is a serious condition where abnormal protein deposits called amyloid fibrils accumulate in heart tissue, causing the heart to become stiff and thickened, which impairs its ability to contract and relax properly. This condition is more common than previously thought and its diagnosis and management are paramount for every cardiovascular specialist 1. The primary goals of care for all patients with cardiac amyloidosis are improved survival and maximized quality of life through each patient’s individual disease journey, which requires a team-based approach to achieve optimal outcomes 1.
Types and Symptoms of Cardiac Amyloidosis
There are several types of cardiac amyloidosis, with the most common being light chain (AL) amyloidosis associated with bone marrow disorders, and transthyretin amyloidosis (ATTR), which can be either hereditary or age-related (wild-type) 1. Symptoms typically include heart failure, irregular heartbeat, fatigue, shortness of breath, swelling in the legs, and low blood pressure.
Diagnosis and Treatment
Diagnosis requires a combination of imaging tests like echocardiography, cardiac MRI, nuclear imaging, and often tissue biopsy to confirm the presence of amyloid 1. Treatment depends on the type of amyloidosis and may include chemotherapy for AL amyloidosis, medications like tafamidis for ATTR, or in some cases, heart transplantation 1. Anticoagulation is a reasonable treatment strategy to reduce the risk of stroke in patients with cardiac amyloidosis and atrial fibrillation (AF) 1.
Management Considerations
The management of cardiac amyloidosis involves physicians and advanced-practice providers across a wide array of specialties, including primary care, cardiology, nephrology, hematology, neurology, gastroenterology, and palliative care 1. The treatment of patients with amyloidosis is complex and requires a team-based approach to achieve optimal outcomes 1. Early diagnosis is crucial as the condition is progressive and can lead to severe heart failure if left untreated. The use of established guidelines for the management of heart failure in conjunction with specific strategies for diagnosis and treatment of cardiac amyloidosis is recommended 1.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Definition of Amyloid Infiltration of the Heart
Amyloid infiltration of the heart, also known as cardiac amyloidosis, occurs due to the deposition of abnormal proteins (amyloid) in the cardiac tissue 2, 3, 4, 5, 6.
Causes and Types
There are three main types of amyloidosis associated with cardiac involvement:
- Light chain (AL)
- Familial or senile (ATTR)
- Secondary amyloidosis (AA) 3. Any cardiovascular structure can be affected by amyloid infiltration, including the valves 4.
Effects on the Heart
Cardiac amyloidosis often results in heart failure with preserved left ventricular ejection fraction, and may display echocardiographic features of restrictive cardiomyopathy associated with left ventricular hypertrophy or mimic hypertrophic obstructive cardiomyopathy 3. Amyloid accumulation within the cardiac valves may lead to their structural and functional impairment, with a profound impact on patients' prognosis and quality of life 4.
Diagnosis and Treatment
Imaging studies, blood and urine analysis, and sometimes a tissue biopsy are usually the main tools for diagnosis 3. Treatment will be carried out on two fronts: treatment of the symptoms and complications that the disease already caused and prevention of additional amyloid deposits while managing the concomitant complications 3. Recent advances in diagnosis and treatment have emphasized the importance of early recognition and changed management of this condition 5.