Cardiac amyloidosis differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]; Aarti Narayan, M.B.B.S [3]; Lakshmi Gopalakrishnan, M.B.B.S. [4]

Overview

Cardiomyopathy with congestive heart failure is the most common presentation of cardiac amyloidosis. Other common causes of a cardiomyopathy should be excluded, and cardiac amyloidosis should be considered in the absence of a history of myocaridal ischemia, myocardial infarction or presence of coronary artery disease risk factors. Cardiac amyloidosis should be included in the differential diagnoses in patients with unexplained congestive heart failure who have no history of Valvular heart disease, long-standing hypertension or myocardial ischemia.

Differentiating Cardiac Amyloidosis from Other Diseases

Cardiac amyloidosis should be differentiated from the following:

• Hypertension
• Anderson-Fabry disease
• Hypertrophic cardiomyopathy
• Cardiac sarcoidosis
• Cardiac lymphoma

Cardiac Amyloidosis is Differentiated from the Above Disorders by the Presence of the Following:

• Presence of low voltage on the EKG. Other causes of a low QRS voltage are shown here.
• Echo features such as:
  • Diffuse increased echogenicity
  • Valve thickening
  • Thickened interatrial septum
• Global late gadolinium enhancement (LGE) on MRI

Cardiac amyloidosis (AL and TTRwt) should be differentiated from other causes of heart failure:

Differential Diagnosis	History and Symptoms	Physical Examination	Laboratory Findings	Imaging Findings
Cardiac amyloidosis	Fatigue Dyspnea Dizziness Orthopnea Peripheral edema Weight loss due to cardiac cachexia Ascites Syncope on exertion Transthyretin (TTR) associated more common in African-Americans during sixth to seventh decade of life	Elevated jugular pressure Periorbital purpura: Often occurs with sneezing, coughing or with minor trauma. Indicates capillary involvement of AL type amyloidosis. Macroglossia Abnormal phonation Hepatomegaly Ascites may be present in the setting of heart failure Valvular involvement murmurs of mitral and tricuspid regurgitation (systolic).	Normocytic mormochromic anemia Serum free-light-chain assay positive Increased BNP, ANP and β2 microglobulin Voltage-to-mass ratio is more sensitive than EKG, 2D Echo and nuclear scanning alone	Granular or sparkling appearance of the left ventricular (LV) myocardium Increased left and right atrial volumes and reduced atrial function on cardiac MRI Atrial septal thickening Dynamic LV flow Mildly or moderately increased wall thickness in the early asymptomatic phase and severe thickening and hypokinesia of the left ventricular posterior wall and interventricular septum
ST Segment Elevation Myocardial Infarction	Chest pain with possible radiation to left arm and lower jaw Squeezing, crushing chest pain Sweating Nausea and vomiting	Anxious patient in pain with diaphoresis Signs of heart failure may be present Arrhythmia	ST elevation, new left bundle branch block, and Q wave on EKG Elevated cardiac biomarkers	Either complete or subtotal occlusion of an epicardial coronary artery on coronary angiography Confluent hyperenhancement extending from the endocardium
Non ST Elevation Myocardial Infarction	Crushing, left-sided substernal chest pain or pressure that radiates to the neck or left arm	Same as ST-elevation MI	ST-segment depression or T-wave inversion on EKG Elevated cardiac biomarkers
Pericarditis	Chest pain relieved by sitting up and leaning forward and worsened by lying down Fever, anxiety, difficulty breathing	Pericardial friction rub Signs of cardiac tamponade may be present	PR segment depression and electrical alternans on EKG	A flask-shaped, enlarged cardiac silhouette on CXR Pericardial thickness of more than 4 mm on MRI Pericardial effusion and cardiac chamber indentation or collapse on echo when cardiac tamponade is present
Alcoholic Cardiomyopathy	History of alcohol abuse Fatigue, weakness, anorexia, palpitations, and shortness of breath on activity Leg swelling and pedal edema	Signs of heart failure such as presence of S3 and S4 heart sounds, pedal edema, and jugular venous distension Signs of alcoholic liver disease may be present	Elevated MCV and MCHC on CBC Elevated LDH, AST, ALT, creatine kinase, gammaglutamyl transpeptidase, malic dehydrogenase, and alpha-hydroxybutyric dehydrogenase Q waves and non specific ST and T wave changes on EKG	Cardiomegaly, pulmonary congestion, and pleural effusions on CXR Left ventricular dilatation on echo