Podcast
Questions and Answers
What is the primary characteristic that differentiates primary systemic amyloidosis from secondary systemic amyloidosis?
What is the primary characteristic that differentiates primary systemic amyloidosis from secondary systemic amyloidosis?
Which type of amyloidosis is most commonly associated with rheumatoid arthritis?
Which type of amyloidosis is most commonly associated with rheumatoid arthritis?
What structural characteristic of amyloid fibrils accounts for their unique staining properties?
What structural characteristic of amyloid fibrils accounts for their unique staining properties?
Which of the following best describes endomyocardial fibrosis?
Which of the following best describes endomyocardial fibrosis?
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Which of the following is NOT a typical symptom of restrictive cardiomyopathies?
Which of the following is NOT a typical symptom of restrictive cardiomyopathies?
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What unique staining property is indicated by the biopsy results when using Congo Red stain?
What unique staining property is indicated by the biopsy results when using Congo Red stain?
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Which condition is most commonly associated with eosinophilic infiltration and myeloproliferative disorders?
Which condition is most commonly associated with eosinophilic infiltration and myeloproliferative disorders?
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Which of the following diagnoses is characterized by the presence of eosinophilic, homogenous material between myocytes?
Which of the following diagnoses is characterized by the presence of eosinophilic, homogenous material between myocytes?
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In the context of restrictive cardiomyopathy, what condition does not typically present the pathological findings associated with the biopsy described?
In the context of restrictive cardiomyopathy, what condition does not typically present the pathological findings associated with the biopsy described?
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Which of the following terms describes the abnormal accumulation of proteins in the heart tissue leading to restrictive cardiomyopathy?
Which of the following terms describes the abnormal accumulation of proteins in the heart tissue leading to restrictive cardiomyopathy?
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Which condition is characterized by impaired ventricular filling during diastole?
Which condition is characterized by impaired ventricular filling during diastole?
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Which of the following symptoms is NOT typically associated with amyloidosis?
Which of the following symptoms is NOT typically associated with amyloidosis?
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What is a common diagnostic method used to confirm amyloidosis?
What is a common diagnostic method used to confirm amyloidosis?
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In cardiac amyloidosis, which of the following is a characteristic finding on an H&E stained tissue section?
In cardiac amyloidosis, which of the following is a characteristic finding on an H&E stained tissue section?
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In wild-type ATTR amyloidosis, which complication is commonly associated with the condition?
In wild-type ATTR amyloidosis, which complication is commonly associated with the condition?
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What is the primary cause of Loeffler endomyocarditis?
What is the primary cause of Loeffler endomyocarditis?
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What is a histological feature of cardiac amyloidosis?
What is a histological feature of cardiac amyloidosis?
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Which symptom is most commonly associated with congestive heart failure due to restrictive cardiomyopathy?
Which symptom is most commonly associated with congestive heart failure due to restrictive cardiomyopathy?
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Which imaging technique typically shows normal-sized heart with signs of pulmonary congestion in restrictive cardiomyopathy?
Which imaging technique typically shows normal-sized heart with signs of pulmonary congestion in restrictive cardiomyopathy?
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Which condition can be considered a tropical form of restrictive cardiomyopathy, often seen in young adults?
Which condition can be considered a tropical form of restrictive cardiomyopathy, often seen in young adults?
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Which of the following is a common cardiovascular finding in restrictive cardiomyopathy?
Which of the following is a common cardiovascular finding in restrictive cardiomyopathy?
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What is a common morphological characteristic of the left ventricle in patients with cardiac amyloidosis?
What is a common morphological characteristic of the left ventricle in patients with cardiac amyloidosis?
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Which type of amyloidosis involves amyloid deposits that commonly cause peripheral neuropathy?
Which type of amyloidosis involves amyloid deposits that commonly cause peripheral neuropathy?
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Which staining method is used to visualize amyloid deposits under polarized light?
Which staining method is used to visualize amyloid deposits under polarized light?
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Study Notes
Restrictive Cardiomyopathies
- A primary reduction in ventricular compliance
- Impaired ventricular filling during diastole
- Normal LV systolic function
- Can be idiopathic or associated with conditions like radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumors, and inborn errors of metabolism.
- Idiopathic restrictive cardiomyopathy:
- Normal or slightly enlarged ventricle, not dilated
- Firm myocardium
- Abnormally rigid, not necessarily thickened ventricle
- Leads to biatrial dilation which can cause arrhythmias
- Microscopy shows patchy or diffuse interstitial fibrosis, ranging from mild to severe
- An endomyocardial biopsy can show disease specific findings in some cases.
Cardiac Amyloidosis
- Major organ involved in senile amyloidosis
- Amyloid deposits in atria and ventricles or atria alone. If subendocardial, the conduction system may be disturbed
- Isolated atrial amyloidosis is 4x more common in those of African descent over 60 years old due to a mutated gene for transthyretin found in 4% of this population
- Grossly may show no significant findings
- May be mildly enlarged and firm (typical restrictive cardiomyopathy)
- May have a "rubbery," non-compliant texture
- May show waxy semi-translucent nodules, especially on the left atrial endocardium
- Histologically, hyaline eosinophilic deposits of amyloid may be found in the interstitium, conduction tissue, valves, endocardium, pericardium, and small intramural coronary arteries
- Visible as amorphous, eosinophilic deposits on H&E-stained tissues
- Stains salmon-pink with Congo Red stain, demonstrating apple-green birefringence under polarized microscopy
Clinical Presentation of Restrictive Cardiomyopathy
- Signs and symptoms of congestive heart failure are common:
- Decreased cardiac output: Fatigue and decreased exercise tolerance
- Systemic congestion: Often more prominent than pulmonary congestion
- Leads to jugular vein distention (JVD), peripheral edema, ascites, and a large, tender liver
- Pulmonary rales (crackles) are often present
- Chest X-ray: Normal-sized heart with signs of pulmonary congestion
- Electrocardiogram (ECG): Non-specific ST and T wave abnormalities; conduction disturbances such as AV block or bundle branch block
- Restrictive cardiomyopathies share nearly identical symptoms, physical signs, and hemodynamic profiles with constrictive pericarditis
- Distinguish because constrictive pericarditis is often correctable
Miscellaneous Restrictive Cardiomyopathies
Endomyocardial Fibrosis
- Also known as tropical endomyocardial fibrosis
- Primarily seen in children and young adults, especially in Africa and the tropics
- Rare in North America
- Fibrosis of the ventricular endocardium and subendocardium
- Extends from the apex to the atrioventricular (AV) valves
- May involve the AV valves
- Reduces ventricular volume and compliance
- Ventricular mural thrombi can develop
- Unknown etiology and pathogenesis
- Resembles eosinophilic cardiomyopathy, sometimes considered part of the same disease process
- This process includes Loeffler endocarditis (eosinophilic endomyocardial fibrosis)
- Endocardial fibrosis may result from thrombus organization
Loeffler Endomyocarditis
- Rare restrictive cardiomyopathy with endomyocardial fibrosis and typically large mural thrombi
- Not geographically restricted
- Elevated levels of peripheral blood eosinophils and eosinophils in various organs, including the heart
- Abnormal eosinophils may secrete toxic products like major basic protein which damage the endocardium
- Areas of necrosis with eosinophilic infiltrate
- Scarring
- Layering of the endocardium by thrombus which leads to thrombus formation
- Associated with chronic myeloproliferative disorders that have platelet-derived growth factor receptor alpha/beta gene mutations
- Poor prognosis
Endocardial Fibroelastosis
- An uncommon condition with an obscure cause
- Focal or diffuse fibroelastic thickening, usually of the mural left ventricular endocardium
- Typically seen in the first two years of life
- In 1/3 of cases, it occurs alongside aortic valve obstruction or congenital cardiac anomalies
- May be the final morphological outcome of various insults, including viral infections (like intrauterine exposure to mumps) or mutations in the gene for tafazzin, which affects mitochondrial inner membrane integrity.
Comparison of Cardiomyopathies
| Cardiomyopathy | Ventricular Morphology | Symptoms | Physical Exam | Pathophysiology |
|---|---|---|---|---|
| Dilated | Dilated LV, possible myocardial thickening | Fatigue, weakness, dyspnea, orthopnea, paroxysmal nocturnal dyspnea (PND) | Pulmonary rales, S3; if RV failure present, JVD, hepatomegaly, peripheral edema | Impaired systolic contraction |
| Hypertrophic | Marked hypertrophy, often asymmetric | Dyspnea, angina, syncope | S4; if outflow obstruction present, systolic murmur loudest at left sternal border, may be accompanied by mitral regurgitation | Impaired diastolic relaxation; LV systolic function vigorous, often with dynamic obstruction |
| Restrictive | Fibrotic or infiltrated myocardium | Dyspnea, fatigue | Signs of RV failure: JVD, hepatomegaly, peripheral edema | “Stiff” LV with impaired diastolic relaxation, but normal systolic function |
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Description
Explore the key features of restrictive cardiomyopathies and cardiac amyloidosis, including their impact on ventricular filling, compliance, and specific characteristic findings. This quiz will cover the conditions associated with these cardiac issues, such as idiopathic causes and various forms of amyloid deposits. Test your understanding of these important cardiac pathologies.
