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'''For the WikiDoc page for this topic, click [[Cardiac amyloidosis|here]]'''
'''For the WikiDoc page for this topic, click [[Cardiac amyloidosis|here]]'''


{{Cardiac amyloidosis (patient information)}}
{{Cardiac amyloidosis (patient information)}}
 
{{CMG}}; {{AE}} Jinhui Wu, MD; [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]
{{CMG}}; '''Associate Editors-In-Chief''': Jinhui Wu, MD. [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]


==Overview==
==Overview==
Cardiac amyloidosis is a disorder caused by deposits of an abnormal protein (amyloid) in the heart tissue, which make it hard for the heart to work properly.
Cardiac amyloidosis is a disorder caused by deposits of an abnormal protein ([[amyloid]]) in the heart tissue, which makes it hard for the heart to work properly. Involvement of the heart can result in loss of consciousness, abnormal heart rhythms, shortness of breath, fatigue, swelling of the legs, weight loss, nausea, dizziness, and disrupted sleep.


==What are the symptoms of Cardiac amyloidosis?==
==What are the Symptoms of Cardiac Amyloidosis?==
*Most patients with cardiac amyloidosis may experience symptoms [[restrictive cardiomyopathy]]. While other may show no symptoms.
*Most patients with cardiac amyloidosis may experience symptoms of [[restrictive cardiomyopathy]], while others may show no symptoms. Common symptoms include:
:*[[Palpitation]]s
:*[[Skipped heart beats]] or [[extra heart beats]] also known as [[Palpitation]]s
:*[[Shortness of breath]], [[orthopnea]]
:*[[Shortness of breath]], or [[difficulty breathing while lying flat]] also known as [[orthopnea]]
:*[[Fatigue]]
:*[[Fatigue]]
:*Increased [[nocturia]]
:*[[Increased urination at night]] also known as [[nocturia]]
:*[[Swelling]] of legs, ankles, or other part of the body
:*[[Swelling of the ankles]], the legs, or other part of the body


*Diseases with similar symptoms
*Diseases with similar symptoms:
:*[[Coronary artery disease]]
:*[[Coronary artery disease]]
:*[[Constrictive pericarditis]]
:*[[Constrictive pericarditis]]
Line 24: Line 24:
:*[[Sarcoidosis cordis]]
:*[[Sarcoidosis cordis]]


==What causes Cardiac amyloidosis?==
==What Causes Cardiac Amyloidosis?==
Amyloidosis refers to a family of diseases in which there is a buildup of clumps of proteins called [[amyloid]]s in body tissues and organs. These proteins slowly replace normal tissue, leading to failure of the involved organ. There are many forms of amyloidosis.
 
Cardiac amyloidosis usually occurs during [[primary amyloidosis]] (AL type amyloidosis). Primary amyloidosis is often seen in people with [[multiple myeloma]],cancer.
 
Cardiac amyloidosis ("stiff heart syndrome") occurs when amyloid deposits take the place of normal heart muscle. It is the most typical type of [[restrictive cardiomyopathy]]. Cardiac amyloidosis may affect the way electrical signals move through the heart (conduction system). This can lead to [[arrhythmias]] and conduction disturbances (heart block).


[[Secondary amyloidosis]] (AA type) rarely affects the heart. However, a form of secondary amyloidosis called senile amyloidosis involves the heart and blood vessels. Senile amyloidosis is caused by overproduction of a different protein. The condition is becoming more common as the average age of the population increases.
* Amyloidosis refers to a family of diseases in which there is a buildup of clumps of proteins called [[amyloid]]s in body tissues and organs. These proteins slowly replace normal tissue, leading to failure of the involved organ. There are many forms of amyloidosis that may involve specific organs like the heart ([[cardiac amlyoidosis]]), the neurologic system with a [[peripheral neuropathy]] or the kidney.
* Cardiac amyloidosis usually occurs as part of [[primary amyloidosis]] ([[AL type amyloidosis]]). Primary amyloidosis is often seen in people with [[multiple myeloma]] and cancer.
* Cardiac amyloidosis ("[[stiff heart syndrome]]") occurs when [[amyloid]] deposits take the place of normal heart muscle. It is the most typical form of [[restrictive cardiomyopathy]]. Cardiac amyloidosis may affect the way electrical impulses move through the heart (electrical conduction system). This can lead to [[irregular heart beating]] also known as [[arrhythmias]] and [[failure of the electrical impulses to conduct through the heart]] ([[heart block]]).
* [[Amyloidosis pathophysiology#Secondary Amyloidosis|Secondary amyloidosis]] ([[AA type amyloidosis]]) rarely affects the [[heart]]. However, one form of secondary amyloidosis called [[senile amyloidosis]] may involve the heart and blood vessels. Senile amyloidosis is caused by overproduction of a different protein. The condition is becoming more common as the average age of the population increases.


Cardiac amyloidosis is more common in men than in women. The disease is rare in people under age 40
There is no difference in the incidence of cardiac amyloidosis between men and women as it affects both genders equally. The disease is rare in people under age 40.


==Who is at highest risk?==
==Who Is at the Highest Risk?==
[[Amyloidosis]] refers to a buildup of clumps of amyloids in body tissues and organs. These proteins called amyloids slowly replace normal tissue and resuly in organ function failure. Amyloidosis often occurs on patients with the following characteristics:  
[[Amyloidosis]] refers to abnormal deposition of amyloid protein in the body tissues and organs. These proteins gradually replace the normal tissue resulting in organ dysfunction. Amyloidosis often occurs in patients with the following diseases or characteristics:  
*[[Multiple myeloma]]: Cardiac amyloidosis usually occurs in patients with multiple myeloma.
*[[Multiple myeloma]]: Cardiac amyloidosis usually occurs in patients with [[multiple myeloma]].
*Male gender: Cardiac amyloidosis is more common in men than in women.  
*Age over 40: The disease is rare in people under age 40.
*Age over 40: The disease is rare in people under age 40.


==When to seek urgent medical care?==
==When to Seek Urgent Medical Care?==
Call your health care provider if symptoms of cardiac amyloidosis develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:  
Call your health care provider if symptoms of cardiac amyloidosis develop. If you experience any of the following symptoms, seek urgent medical care as soon as possible:  
*[[Palpitation]]s
:*[[Skipped heart beats]] or [[extra heart beats]] also known as [[palpitation]]s
*Severen [[shortness of breath]]
:* Severe [[shortness of breath]], or [[difficulty breathing while lying flat]] also known as [[orthopnea]]
*[[Orthopnea]]


==Diagnosis==
==Diagnosis==
Your physician may run the following test to confirm the diagnosis:
* [[EKG]]: This is a basic test for cardiovascular diseases that records the heart's electrical activity. During this test, you are asked to lie down. The health care provider will clean several areas on your arms, legs, and chest, and attach electrodes to these areas. Then, the electrical activities of your heart will be recorded. ECG may help your doctor identify the type of [[irregular heart beats]] also known as [[arrhythmia]] such as [[extra beats from the larger chambers of the heart]] also known as [[ventricular premature beats]], [[extra beats from the smaller chamber of the heart]] also known as [[atrial fibrillation]] or [[abnormal conduction of the impulses through the heart]] also known as [[heart block]].
* [[Echocardiogram]]: This is also a painless test for patients which can be used to assess both the structure and function of the heart. Patients with cardiac amyloidosis may have a [[thickening of the heart muscle]] a condition known as [[left ventricular hypertrophy]] and [[stiffness of the heart]], areas that light up showing amyloid deposition in the heart called an echogenic focus, and decreased heart pumping also known as a reduced [[ejection fraction]].
* [[Chest x-ray]]: On chest x-ray, patients with cardiac amyloidosis may show an [[enlarged heart]] and [[fluid in the lungs]] also known as [[pulmonary congestion]]. 
* Thoraco-abdominal [[CT]] scan
* [[Magnetic resonance imaging]] ([[MRI]])
* [[Nuclear heart scan]]s
* [[Coronary angiography]]
* [[Cardiac biopsy]]


Your physician may run the following test to confirm the diagnosis
==Treatment Options==
 
There is no definitive treatment available for cardiac amyloidosis. The purpose of treatment is to decrease the synthesis and deposition of the abnormal protein and to protect the heart's function.
*[[ECG]]: This is a basic test for cardiovascular diseases that records the heart's electrical activity. During this test, you are asked to lie down. The health care provider will clean several areas on your arms, legs, and chest, and attach electrodes to these areas. Then, the electrical activities of your heart will be recorded. ECG may help your doctor identify the type of your [[arrhythmia]], such as [[st-segment depression]], [[ventricualr premature]], [[tachycardia]] or [[heart block]].
 
*[[Echocardiogram]]: This is also a painless and no risk test for patients. Cardiac echo can demonstrate both structure and function of the heart. Patients with cardiac amyloidosis may appear signs of ventricular wall [[hypertrophy]] and [[stiffness]], several cardiac echogenic focus, and decreased [[ejection fraction]].
 
*[[Chest x-ray]]: On chest x-ray, patients with cardiac amyloidsis may demonstrate images of enlarged heart and pulmonary congestion. 
 
*Thoracoabdominal [[CT]] scan
 
*[[Magnetic resonance imaging]] ([[MRI]])
 
*[[Nuclear heart scan]]s
 
*Coronary [[angiography]]
 
*Cardiac [[biopsy]]
 
==Treatment options==
Treatment effect of cardiac amyloidosis is not good. The purpose of treatment is to decrease the synthesis and deposition of the abnormal protein and protect heart function.


*Diet: A diet of salt and fluid restrictions is recommended to patients with cardiac amyloidosis.
* Diet: Salt and fluid restriction is recommended to patients with cardiac amyloidosis.
*[[Medication]]s
* [[Medication]]s
:*[[Chemotherapy]]: Drugs such as [[cyclophosphamide]], [[carmustine]] and [[doxorubicin]], can be used to inhibit the over-immunoreaction and decrease the synthesis of abnormal protein.
:* [[Chemotherapy]]: Drugs such as [[cyclophosphamide]], [[carmustine]] and [[doxorubicin]], can be used to inhibit the over-immunoreaction and decrease the synthesis of abnormal protein.
:*[[Prednisone]]: Prednisone, an anti-inflammatory medicine, may promote the decomposition of the abnormal protein and relieve patients' [[side effect]]s. Prednisone is usually used with chemotherapy drugs.
:* [[Prednisone]]: Prednisone, an anti-inflammatory medicine, may promote the dissolution of the abnormal protein and relieve patients' symptoms. Prednisone is usually used along with other chemotherapy drugs.
:*[[Diuretics]]: Diuretics may help your body remove excess fluid and relieve swelling.
:* [[Diuretics]]: Diuretics may help your body remove excess fluid and relieve swelling.
:*[[Digoxin]], [[calcium channel blocker]]s, and [[beta blocker]]s: These three types of drugs can be used to control heart rate when [[atrial fibrillation]] happens. Usual drugs include [[dilthiazem]], [[metoprolol]], [[bisoprolol]], and [[carvedilol]]. These drugs should be monitored by the doctors.  
:* [[Digoxin]], [[calcium channel blocker]]s, and [[beta blocker]]s: These three types of drugs can be used to control heart rate when [[atrial fibrillation]] happens. Digoxin and calcium channel blockers are contraindicated in cardiac amyloidosis due to a potential for toxicity. Usual drugs include [[metoprolol]], [[bisoprolol]], and [[carvedilol]]. These drugs should be monitored carefully by the doctors.


==Where to find medical care for Cardiac amyloidosis?==
==Where to Find Medical Care for Cardiac Amyloidosis?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|cardiac amyloidosis}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating cardiac amyloidosis]
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|cardiac amyloidosis}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating cardiac amyloidosis]


==What to expect (Outlook/Prognosis)?==
==What to Expect (Outlook/Prognosis)?==
Cardiac amyloidosis is a long-term (chronic) condition that slowly gets worse. On average, persons with cardiac amyloidosis live less than 1 year.
Cardiac amyloidosis is a long-term (chronic) condition that slowly worsens. On average, persons with cardiac amyloidosis live less than 1 year.


==Possible complications==
==Possible Complications==
*[[Atrial fibrillation]] or ventricular arrhythmias
* [[Irregular heart beating in the small chamber of the heart]] also known as [[atrial fibrillation]] or [[irregular heart beating in the large chamber of the heart]] also known as  [[ventricular arrhythmia]]s
*[[Congestive heart failure]]
* [[Failure of the heart to push blood forward to the body]] also known as [[congestive heart failure]]
*Fluid buildup in the abdomen [[ascites]]
* [[Fluid buildup in the abdomen]] also known as [[ascites]]
*[[Sick sinus syndrome]]
* Alternating slow and fast heart beating also known as [[Sick sinus syndrome]]


==Sources==
==Sources==
[http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm Medline]
http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm


{{WH}}
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[[CME Category::Cardiology]]


[[Category:Disease]]
[[Category:Disease]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]
[[Category:Rheumatology patient information]]
[[Category:Patient information]]
[[Category:Patient information]]
[[Category:Overview complete]]
[[Category:Cardiology]]

Latest revision as of 04:21, 6 November 2019

For the WikiDoc page for this topic, click here

Cardiac amyloidosis

Overview

What are the symptoms?

What are the causes?

Who is at highest risk?

When to seek urgent medical care?

Diagnosis

Treatment options

Where to find medical care for Cardiac amyloidosis?

What to expect (Outlook/Prognosis)?

Possible complications

Cardiac amyloidosis On the Web

Ongoing Trials at Clinical Trials.gov

Images of Cardiac amyloidosis

Videos on Cardiac amyloidosis

FDA on Cardiac amyloidosis

CDC on Cardiac amyloidosis

Cardiac amyloidosis in the news

Blogs on Cardiac amyloidosis

Directions to Hospitals Treating Cardiac amyloidosis

Risk calculators and risk factors for Cardiac amyloidosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jinhui Wu, MD; Ujjwal Rastogi, MBBS [2]

Overview

Cardiac amyloidosis is a disorder caused by deposits of an abnormal protein (amyloid) in the heart tissue, which makes it hard for the heart to work properly. Involvement of the heart can result in loss of consciousness, abnormal heart rhythms, shortness of breath, fatigue, swelling of the legs, weight loss, nausea, dizziness, and disrupted sleep.

What are the Symptoms of Cardiac Amyloidosis?

  • Most patients with cardiac amyloidosis may experience symptoms of restrictive cardiomyopathy, while others may show no symptoms. Common symptoms include:
  • Diseases with similar symptoms:

What Causes Cardiac Amyloidosis?

There is no difference in the incidence of cardiac amyloidosis between men and women as it affects both genders equally. The disease is rare in people under age 40.

Who Is at the Highest Risk?

Amyloidosis refers to abnormal deposition of amyloid protein in the body tissues and organs. These proteins gradually replace the normal tissue resulting in organ dysfunction. Amyloidosis often occurs in patients with the following diseases or characteristics:

When to Seek Urgent Medical Care?

Call your health care provider if symptoms of cardiac amyloidosis develop. If you experience any of the following symptoms, seek urgent medical care as soon as possible:

Diagnosis

Your physician may run the following test to confirm the diagnosis:

Treatment Options

There is no definitive treatment available for cardiac amyloidosis. The purpose of treatment is to decrease the synthesis and deposition of the abnormal protein and to protect the heart's function.

  • Diet: Salt and fluid restriction is recommended to patients with cardiac amyloidosis.
  • Medications
  • Chemotherapy: Drugs such as cyclophosphamide, carmustine and doxorubicin, can be used to inhibit the over-immunoreaction and decrease the synthesis of abnormal protein.
  • Prednisone: Prednisone, an anti-inflammatory medicine, may promote the dissolution of the abnormal protein and relieve patients' symptoms. Prednisone is usually used along with other chemotherapy drugs.
  • Diuretics: Diuretics may help your body remove excess fluid and relieve swelling.
  • Digoxin, calcium channel blockers, and beta blockers: These three types of drugs can be used to control heart rate when atrial fibrillation happens. Digoxin and calcium channel blockers are contraindicated in cardiac amyloidosis due to a potential for toxicity. Usual drugs include metoprolol, bisoprolol, and carvedilol. These drugs should be monitored carefully by the doctors.

Where to Find Medical Care for Cardiac Amyloidosis?

Directions to Hospitals Treating cardiac amyloidosis

What to Expect (Outlook/Prognosis)?

Cardiac amyloidosis is a long-term (chronic) condition that slowly worsens. On average, persons with cardiac amyloidosis live less than 1 year.

Possible Complications

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm

Template:WH Template:WS CME Category::Cardiology