Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management
Faizi Jamal1, Michael Rosenzweig2
1Department of Medicine, Division of Cardiology, City of Hope, Duarte, CA, USA.
Current Hematologic Malignancy Reports
|June 9, 2021
Summary
Cardiac amyloidosis diagnosis and management are improving with new imaging tools and treatments for light chain and transthyretin amyloidosis, enhancing patient survival and organ response.
Area of Science:
- Cardiology
- Oncology
- Genetics
Background:
- Amyloidosis involves abnormal protein deposits causing organ dysfunction.
- Cardiac amyloidosis significantly impacts prognosis and treatment strategies.
- Accurate disease characterization is crucial for effective patient management.
Purpose of the Study:
- To review advancements in diagnosing and managing cardiac amyloidosis.
- To highlight the importance of understanding precursor proteins and organ involvement.
- To discuss new therapeutic options for light chain and transthyretin amyloidosis.
Main Methods:
- Review of recent imaging modalities for detecting cardiac amyloid infiltration.
- Analysis of diagnostic algorithms for suspected amyloidosis.
- Evaluation of outcomes from novel treatments for specific amyloidosis types.
Main Results:
- New imaging techniques enhance the identification of cardiac amyloidosis.
- Diagnostic algorithms aid in disease classification and genetic testing.
- Emerging therapies improve survival and organ response in light chain and transthyretin amyloidosis.
Conclusions:
- Advanced imaging and diagnostic tools improve cardiac amyloidosis detection and management.
- Novel treatments offer improved outcomes for patients with specific amyloidosis types.
- Continued research is vital for addressing the morbidity and mortality associated with systemic amyloidosis.
Related Concept Videos
Cardiomyopathy IV: Restrictive Cardiomyopathy
142
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
142
Cardiomyopathy III: Hypertrophic Cardiomyopathy
109
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
109
Cardiomyopathy V: Interprofessional Care
93
Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
93
Cardiomyopathy I: Introduction and Classification
169
Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
169
Myocarditis II: Clinical Features and Diagnostic Tests
70
Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
70
Cardiomyopathy II: Dilated Cardiomyopathy
111
Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
111


