Cardiac amyloidosis: Difference between revisions
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{{Infobox medical condition | |||
| name = Cardiac amyloidosis | |||
| image = [[File:Heart_anterior_exterior_view.jpg|250px]] | |||
| caption = Anterior view of the heart | |||
| synonyms = Stiff heart syndrome | |||
| specialty = [[Cardiology]] | |||
| symptoms = [[Heart failure]], [[arrhythmia]], [[syncope]], [[peripheral edema]] | |||
| complications = [[Heart failure]], [[sudden cardiac death]] | |||
| onset = Typically in [[adulthood]] | |||
| duration = [[Chronic (medicine)|Chronic]] | |||
| causes = Deposition of [[amyloid]] proteins in the heart | |||
| risks = [[Family history]], [[multiple myeloma]], [[chronic inflammatory conditions]] | |||
| diagnosis = [[Echocardiography]], [[cardiac MRI]], [[endomyocardial biopsy]] | |||
| differential = [[Hypertrophic cardiomyopathy]], [[restrictive cardiomyopathy]] | |||
| treatment = [[Chemotherapy]], [[heart transplant]], [[supportive care]] | |||
| medication = [[Tafamidis]], [[doxycycline]], [[diflunisal]] | |||
| prognosis = Variable, depends on type and stage | |||
| frequency = Rare | |||
}} | |||
[[File:Heart anterior exterior view.jpg|Heart anterior exterior view|thumb|left]] | |||
[[File:Amyloidosis with severe cardiac involvement E00109 (CardioNetworks ECHOpedia).webm|Amyloidosis with severe cardiac involvement|thumb|left]] | |||
[[File:Cardiac amyloidosis high mag he.jpg|Cardiac amyloidosis high magnification|thumb]] | |||
'''Cardiac Amyloidosis''' is a medical condition characterized by the abnormal deposition of [[amyloid]] proteins in the heart tissue, leading to progressive heart failure. It is a form of [[systemic amyloidosis]] and is classified into several types based on the protein involved. | '''Cardiac Amyloidosis''' is a medical condition characterized by the abnormal deposition of [[amyloid]] proteins in the heart tissue, leading to progressive heart failure. It is a form of [[systemic amyloidosis]] and is classified into several types based on the protein involved. | ||
==Etiology== | ==Etiology== | ||
Cardiac amyloidosis is caused by the accumulation of insoluble amyloid fibrils in the extracellular space of the myocardium. These fibrils are derived from different precursor proteins, which determine the type of cardiac amyloidosis. The most common types are: | Cardiac amyloidosis is caused by the accumulation of insoluble amyloid fibrils in the extracellular space of the myocardium. These fibrils are derived from different precursor proteins, which determine the type of cardiac amyloidosis. The most common types are: | ||
* '''[[AL amyloidosis]]''': Also known as primary amyloidosis, it is caused by light chain proteins produced by abnormal plasma cells. | * '''[[AL amyloidosis]]''': Also known as primary amyloidosis, it is caused by light chain proteins produced by abnormal plasma cells. | ||
* '''[[ATTR amyloidosis]]''': This type can be hereditary (ATTRm) or non-hereditary (ATTRwt). It is caused by transthyretin, a protein produced by the liver. | * '''[[ATTR amyloidosis]]''': This type can be hereditary (ATTRm) or non-hereditary (ATTRwt). It is caused by transthyretin, a protein produced by the liver. | ||
==Clinical Presentation== | ==Clinical Presentation== | ||
Patients with cardiac amyloidosis may present with symptoms of heart failure, such as [[dyspnea]], fatigue, and edema. Other symptoms can include [[arrhythmia]]s and [[syncope]]. The disease often goes undiagnosed due to its non-specific symptoms and the rarity of the condition. | Patients with cardiac amyloidosis may present with symptoms of heart failure, such as [[dyspnea]], fatigue, and edema. Other symptoms can include [[arrhythmia]]s and [[syncope]]. The disease often goes undiagnosed due to its non-specific symptoms and the rarity of the condition. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of cardiac amyloidosis involves a combination of clinical assessment, imaging studies, and laboratory tests. [[Echocardiography]] and [[cardiac MRI]] are commonly used imaging modalities. Definitive diagnosis requires a biopsy of the affected tissue, demonstrating amyloid deposits. | Diagnosis of cardiac amyloidosis involves a combination of clinical assessment, imaging studies, and laboratory tests. [[Echocardiography]] and [[cardiac MRI]] are commonly used imaging modalities. Definitive diagnosis requires a biopsy of the affected tissue, demonstrating amyloid deposits. | ||
==Treatment== | ==Treatment== | ||
Treatment of cardiac amyloidosis is aimed at reducing the production of the amyloid protein and managing the symptoms of heart failure. This can involve chemotherapy for AL amyloidosis or liver transplantation for ATTRm amyloidosis. Supportive care, including diuretics and antiarrhythmics, is also important. | Treatment of cardiac amyloidosis is aimed at reducing the production of the amyloid protein and managing the symptoms of heart failure. This can involve chemotherapy for AL amyloidosis or liver transplantation for ATTRm amyloidosis. Supportive care, including diuretics and antiarrhythmics, is also important. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis of cardiac amyloidosis is generally poor, with a median survival of less than a year for untreated AL amyloidosis. However, early detection and treatment can improve survival and quality of life. | The prognosis of cardiac amyloidosis is generally poor, with a median survival of less than a year for untreated AL amyloidosis. However, early detection and treatment can improve survival and quality of life. | ||
==See Also== | ==See Also== | ||
* [[Amyloidosis]] | * [[Amyloidosis]] | ||
* [[Heart failure]] | * [[Heart failure]] | ||
* [[Cardiomyopathy]] | * [[Cardiomyopathy]] | ||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
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{{Medicine-stub}} | {{Medicine-stub}} | ||
{{Cardiology-stub}} | {{Cardiology-stub}} | ||
Latest revision as of 21:18, 4 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
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| Cardiac amyloidosis | |
|---|---|
| |
| Synonyms | Stiff heart syndrome |
| Pronounce | N/A |
| Specialty | Cardiology |
| Symptoms | Heart failure, arrhythmia, syncope, peripheral edema |
| Complications | Heart failure, sudden cardiac death |
| Onset | Typically in adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Deposition of amyloid proteins in the heart |
| Risks | Family history, multiple myeloma, chronic inflammatory conditions |
| Diagnosis | Echocardiography, cardiac MRI, endomyocardial biopsy |
| Differential diagnosis | Hypertrophic cardiomyopathy, restrictive cardiomyopathy |
| Prevention | N/A |
| Treatment | Chemotherapy, heart transplant, supportive care |
| Medication | Tafamidis, doxycycline, diflunisal |
| Prognosis | Variable, depends on type and stage |
| Frequency | Rare |
| Deaths | N/A |
File:Amyloidosis with severe cardiac involvement E00109 (CardioNetworks ECHOpedia).webm
Cardiac Amyloidosis is a medical condition characterized by the abnormal deposition of amyloid proteins in the heart tissue, leading to progressive heart failure. It is a form of systemic amyloidosis and is classified into several types based on the protein involved.
Etiology[edit]
Cardiac amyloidosis is caused by the accumulation of insoluble amyloid fibrils in the extracellular space of the myocardium. These fibrils are derived from different precursor proteins, which determine the type of cardiac amyloidosis. The most common types are:
- AL amyloidosis: Also known as primary amyloidosis, it is caused by light chain proteins produced by abnormal plasma cells.
- ATTR amyloidosis: This type can be hereditary (ATTRm) or non-hereditary (ATTRwt). It is caused by transthyretin, a protein produced by the liver.
Clinical Presentation[edit]
Patients with cardiac amyloidosis may present with symptoms of heart failure, such as dyspnea, fatigue, and edema. Other symptoms can include arrhythmias and syncope. The disease often goes undiagnosed due to its non-specific symptoms and the rarity of the condition.
Diagnosis[edit]
Diagnosis of cardiac amyloidosis involves a combination of clinical assessment, imaging studies, and laboratory tests. Echocardiography and cardiac MRI are commonly used imaging modalities. Definitive diagnosis requires a biopsy of the affected tissue, demonstrating amyloid deposits.
Treatment[edit]
Treatment of cardiac amyloidosis is aimed at reducing the production of the amyloid protein and managing the symptoms of heart failure. This can involve chemotherapy for AL amyloidosis or liver transplantation for ATTRm amyloidosis. Supportive care, including diuretics and antiarrhythmics, is also important.
Prognosis[edit]
The prognosis of cardiac amyloidosis is generally poor, with a median survival of less than a year for untreated AL amyloidosis. However, early detection and treatment can improve survival and quality of life.
See Also[edit]
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