Table of ContentsView AllTable of ContentsProtein BuildupTypesWarning SignsOther Signs and SymptomsIs It Life-Threatening?DiagnosisTreatmentComplicationsTreatment Success
Table of ContentsView All
View All
Table of Contents
Protein Buildup
Types
Warning Signs
Other Signs and Symptoms
Is It Life-Threatening?
Diagnosis
Treatment
Complications
Treatment Success
Cardiac amyloidosisoccurs when abnormal proteins accumulate in the heart muscle, leading to potential arrhythmias and heart failure. Because heart failure has many causes, it can take years to diagnose. The complications of cardiac amyloidosis can be life-threatening, and close follow-up and treatment with a cardiologist (medical doctor specializing in conditions affecting the heart) is important to manage the condition.
This article will discuss the causes and types of cardiac amyloidosis and its symptoms, complications, and management.
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Protein Buildup in Cardiac Amyloidosis
Overall risk factors for cardiac amyloidosis include amyloidosis affecting family members, history of thyroid disease, and being over 60 years old.
Types of Cardiac Amyloidosis
Cardiac amyloidosis is classified into two main types based on the type of misfolded protein: Light chain amyloidosis andtransthyretinamyloidosis.
Light Chain Amyloidosis
While light chains are essential to the function of the immune system, problems arise when plasma cells go unchecked and create too many light chains or abnormal light chains. These can deposit in the heart and other organs, leading to specific symptoms and organ dysfunction.
Risk factors for AL amyloidosis include a history of plasma cell disorders, such asmultiple myelomaandmonoclonal gammopathy of undetermined significance(MGUS).
Transthyretin Amyloidosis
There are two subtypes of ATTR: hereditary and wild-type.Inhereditary ATTR, a genetic mutation makes the protein more prone to fold and, thus, deposit in tissues, typically the heart and nerves. It’s hereditary as the genetic mutation is passed down in the genes.
In wild-type ATTR (previously called senile amyloidosis), on the other hand, there is no genetic mutation. Instead, the protein deposits in the heart over a period of decades. Wild type ATTR is generally seen in people older than 70 years.
Signs and Symptoms of hATTR Amyloidosis
Warning Signs of Cardiac Amyloidosis
Cardiac amyloidosis is often diagnosed after several years of experiencing heart failure symptoms, including:
It’s important to note that these symptoms are not specific to cardiac amyloidosis; other types of heart failure can cause them. This is why diagnosis can be delayed. Acardiologistcan investigate these symptoms and help determine the underlying cause. In addition, people with cardiac amyloidosis may be intolerant of common heart failure therapies and experience blood pressure drops with symptoms when taking these medications.
Types of Amyloidosis Symptoms
Other Associated Signs and Symptoms
Having these signs in combination with heart failure should raise suspicion of cardiac amyloidosis.
Is Cardiac Amyloidosis Life-Threatening?
Amyloidosis that involves the heart is serious and may be life-threatening due toheart failureandarrhythmiasleading to sudden cardiac death.
Untreated AL cardiac amyloidosis is associated with six-month mortality.In ATTR amyloidosis, median survival after diagnosis ranged from 2.5 to 3.6 years.However, newer techniques to diagnose amyloidosis, as well as an increased appreciation of amyloidosis as a cause of heart failure can lead to an earlier diagnosis. This may ultimately help to improve outcomes as newer treatments can help slow the accumulation of misfolded proteins.
When to Seek Emergency CareCardiac amyloidosis can lead to life-threatening complications, and it’s important to know when to seek help. Notify your healthcare provider for any new or worsening symptoms. From there, they can advise you on next steps. Seek immediate evaluation and treatment for concerning symptoms such as sudden shortness of breath, chest pain, or loss of consciousness.
When to Seek Emergency Care
Cardiac amyloidosis can lead to life-threatening complications, and it’s important to know when to seek help. Notify your healthcare provider for any new or worsening symptoms. From there, they can advise you on next steps. Seek immediate evaluation and treatment for concerning symptoms such as sudden shortness of breath, chest pain, or loss of consciousness.
Diagnosis of Cardiac Amyloidosis
The following tests can help your cardiologist arrive at a diagnosis of cardiac amyloidosis:
Staging Cardiac AmyloidosisVarious staging systems exist for AL amyloidosis that rely on cardiac biomarkers (NT-proBNP and troponin levels) and free light chain measurements. Elevated cardiac biomarkers indicate greater heart involvement, and diagnosis at later stages is associated with worse prognosis.
Staging Cardiac Amyloidosis
Various staging systems exist for AL amyloidosis that rely on cardiac biomarkers (NT-proBNP and troponin levels) and free light chain measurements. Elevated cardiac biomarkers indicate greater heart involvement, and diagnosis at later stages is associated with worse prognosis.
Cardiac Amyloidosis Treatment
Treatment of cardiac amyloidosis includes general management of heart failure and arrhythmias, as well as treatments to target the underlying cause.
Heart failure management includesdiureticsand dietary salt restriction to treat fluid overload. Some common heart failure medications such as beta-blockers, angiotensin converting enzyme–inhibitors (ACE inhibitors), angiotensin receptor blockers (ARBs), and aldosterone antagonists are often not tolerated well in people with amyloidosis and do not show the same benefit as in other types of heart failure.
Arrhythmia management may include beta-blockers and antiarrhythmic medications.Pacemakerimplantation may be necessary for slow heart rhythms.Cardiologistsmay also determine whether placement of animplantable cardioverter defibrillator (ICD)is indicated.
In AL amyloidosis, treatment may involvechemotherapyor astem cell transplantto address the underlying plasma cell disorder.Newer treatments targeting abnormal transthyretin protein in ATTR are being developed. In 2019, the Food and Drug Administration (FDA) approved a medication called tafamidis for treatment of ATTR. This medication works to stabilize transthyretin to prevent further accumulation in the heart.
Complications of Cardiac Amyloidosis
Cardiac amyloidosis has an overall poor prognosis due to the cardiac complications that may arise, including:
If it spreads to other organ systems, it can lead to:
Unfortunately, cardiac amyloidosis is often not diagnosed until later stages.However, the understanding of cardiac amyloidosis continues to advance, and new treatments are developing. Treatment success depends on the type of amyloidosis and early diagnosis so that appropriate treatments may be started promptly.
Summary
9 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Garcia-Pavia P, Rapezzi C, Adler Y, et al.Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC working group on myocardial and pericardial diseases.Eur Heart J. 2021;42(16):1554-1568. doi:10.1093/eurheartj/ehab072Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 18(2):27-35. doi:10.14797/mdcvj.1070MedlinePlus.Transthyretin amyloidosis.American College of Cardiology.Cardiac amyloidosis.Teng C, Li P, Bae JY, Pan S, Dixon RAF, Liu Q.Diagnosis and treatment of transthyretin-related amyloidosis cardiomyopathy.Clin Cardiol. 2020;43(11):1223-1231. doi:10.1002/clc.23434Kittleson MM, Maurer MS, Ambardekar AV, et al.Cardiac amyloidosis: Evolving diagnosis and management: A scientific statement from the American Heart Association.Circulation. 2020;142(1):e7-e22. doi:10.1161/CIR.0000000000000792Grogan M, Dispenzieri A, Gertz MA.Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.Heart. 2017;103(14):1065-1072. doi:10.1136/heartjnl-2016-310704Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 2022;18(2):27-35. doi:10.14797/mdcvj.1070American College of Cardiology.FDA approves first treatment for ATTR-CM.
9 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Garcia-Pavia P, Rapezzi C, Adler Y, et al.Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC working group on myocardial and pericardial diseases.Eur Heart J. 2021;42(16):1554-1568. doi:10.1093/eurheartj/ehab072Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 18(2):27-35. doi:10.14797/mdcvj.1070MedlinePlus.Transthyretin amyloidosis.American College of Cardiology.Cardiac amyloidosis.Teng C, Li P, Bae JY, Pan S, Dixon RAF, Liu Q.Diagnosis and treatment of transthyretin-related amyloidosis cardiomyopathy.Clin Cardiol. 2020;43(11):1223-1231. doi:10.1002/clc.23434Kittleson MM, Maurer MS, Ambardekar AV, et al.Cardiac amyloidosis: Evolving diagnosis and management: A scientific statement from the American Heart Association.Circulation. 2020;142(1):e7-e22. doi:10.1161/CIR.0000000000000792Grogan M, Dispenzieri A, Gertz MA.Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.Heart. 2017;103(14):1065-1072. doi:10.1136/heartjnl-2016-310704Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 2022;18(2):27-35. doi:10.14797/mdcvj.1070American College of Cardiology.FDA approves first treatment for ATTR-CM.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Garcia-Pavia P, Rapezzi C, Adler Y, et al.Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC working group on myocardial and pericardial diseases.Eur Heart J. 2021;42(16):1554-1568. doi:10.1093/eurheartj/ehab072Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 18(2):27-35. doi:10.14797/mdcvj.1070MedlinePlus.Transthyretin amyloidosis.American College of Cardiology.Cardiac amyloidosis.Teng C, Li P, Bae JY, Pan S, Dixon RAF, Liu Q.Diagnosis and treatment of transthyretin-related amyloidosis cardiomyopathy.Clin Cardiol. 2020;43(11):1223-1231. doi:10.1002/clc.23434Kittleson MM, Maurer MS, Ambardekar AV, et al.Cardiac amyloidosis: Evolving diagnosis and management: A scientific statement from the American Heart Association.Circulation. 2020;142(1):e7-e22. doi:10.1161/CIR.0000000000000792Grogan M, Dispenzieri A, Gertz MA.Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.Heart. 2017;103(14):1065-1072. doi:10.1136/heartjnl-2016-310704Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 2022;18(2):27-35. doi:10.14797/mdcvj.1070American College of Cardiology.FDA approves first treatment for ATTR-CM.
Garcia-Pavia P, Rapezzi C, Adler Y, et al.Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC working group on myocardial and pericardial diseases.Eur Heart J. 2021;42(16):1554-1568. doi:10.1093/eurheartj/ehab072
Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 18(2):27-35. doi:10.14797/mdcvj.1070
MedlinePlus.Transthyretin amyloidosis.
American College of Cardiology.Cardiac amyloidosis.
Teng C, Li P, Bae JY, Pan S, Dixon RAF, Liu Q.Diagnosis and treatment of transthyretin-related amyloidosis cardiomyopathy.Clin Cardiol. 2020;43(11):1223-1231. doi:10.1002/clc.23434
Kittleson MM, Maurer MS, Ambardekar AV, et al.Cardiac amyloidosis: Evolving diagnosis and management: A scientific statement from the American Heart Association.Circulation. 2020;142(1):e7-e22. doi:10.1161/CIR.0000000000000792
Grogan M, Dispenzieri A, Gertz MA.Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.Heart. 2017;103(14):1065-1072. doi:10.1136/heartjnl-2016-310704
Baker KR.Light chain amyloidosis: Epidemiology, staging, and prognostication.Methodist Debakey Cardiovasc J. 2022;18(2):27-35. doi:10.14797/mdcvj.1070
American College of Cardiology.FDA approves first treatment for ATTR-CM.
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