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Questions and Answers
What is the most common type of cardiomyopathy?
What distinguishes primary cardiomyopathy from secondary cardiomyopathy?
Which pathophysiological feature is NOT characteristic of cardiomyopathy?
What is a primary underlying cause of cardiomyopathy?
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Which of the following statements about cardiomyopathy is true?
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What is the classic macroscopic pattern observed in hypertrophic cardiomyopathy?
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What percentage of patients with hypertrophic cardiomyopathy experience dynamic obstruction to the left ventricular outflow?
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Which of the following complications is NOT associated with hypertrophic cardiomyopathy?
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Which genetic factor significantly influences the prognosis of hypertrophic cardiomyopathy?
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What is an effective pharmacologic intervention for patients with hypertrophic cardiomyopathy?
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Which characteristic is associated with endomyocardial fibrosis?
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What is a notable feature of Loeffler endocarditis?
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At what stage of life is endocardial fibroelastosis most commonly observed?
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What is the underlying cause of restricted cardiomyopathy?
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Which of the following is NOT a key feature of restrictive cardiomyopathy?
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What characterizes dilated cardiomyopathy morphologically and functionally?
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Which of the following is a common genetic cause of dilated cardiomyopathy?
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Which statement correctly describes hypertrophic cardiomyopathy?
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What is the typical ejection fraction in end-stage dilated cardiomyopathy?
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What characterizes restrictive cardiomyopathy?
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What are common findings in the pathology of dilated cardiomyopathy under microscopy?
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Which of the following is NOT a systemic disorder associated with restrictive cardiomyopathy?
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Which of the following factors is associated with peripartum cardiomyopathy?
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What distinguishes the pathogenesis of hypertrophic cardiomyopathy?
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What is a common microscopic finding in restrictive cardiomyopathy?
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What is the primary definitive treatment for end-stage dilated cardiomyopathy?
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In restrictive cardiomyopathy, what is often observed regarding the size of the ventricles?
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Which condition is an example of an iatrogenic cause of restrictive cardiomyopathy?
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Study Notes
Cardiomyopathy Overview
- Disease of cardiac myocytes, associated with mechanical and/or electrical dysfunction.
- Can result in inappropriate ventricular hypertrophy or dilatation.
- Often has genetic causes; can be classified as primary or secondary.
Types of Cardiomyopathy
- Dilated Cardiomyopathy: Most common type; characterized by cardiac dilation and systolic dysfunction.
- Hypertrophic Cardiomyopathy: Not simply myocardial hypertrophy; involves left ventricular hypertrophy with impaired diastolic filling.
- Restrictive Cardiomyopathy: Least common; marked by decreased ventricular compliance and impaired filling during diastole.
Pathogenesis
- Identifies genetic mutations leading to dysfunctional myocardial proteins.
- Involves mutations affecting contraction, cell communication, and cytoskeletal integrity.
- Abnormal contraction or relaxation and dysregulated ion transport are common consequences.
Dilated Cardiomyopathy Features
- Morphological Characteristics: Enlarged, heavy heart (~2-3 times normal size) and flabby due to chamber dilation; common mural thrombi.
- Pathological Causes: Genetic mutations (20-50% involve dystrophin), viral myocarditis, toxic exposure (alcohol), and conditions like peripartum cardiomyopathy and iron overload.
- Clinical Significance: Poor prognosis with <25% ejection fraction; high mortality within 2 years without heart transplant.
Hypertrophic Cardiomyopathy Features
- Defined by myocardial hypertrophy and impaired diastolic filling; systolic function usually preserved.
- Genetic Basis: Involves autosomal dominant transmission with more than 400 known mutations in nine genes.
- Clinical Significance: Dynamic obstruction can occur; complications include atrial fibrillation, heart failure, and ventricular arrhythmias. Significant risk for young athletes.
Restrictive Cardiomyopathy Features
- Characterized by impaired ventricular filling with normal or slightly enlarged size; firm, non-compliant myocardium.
- Causes: Systemic disorders (e.g., amyloidosis, sarcoidosis), radiation exposure, multifactorial conditions, and idiopathic cases.
- Specific Entities:
- Endomyocardial Fibrosis: Common in tropical regions, affects young adults.
- Loeffler Endocarditis: Associated with myeloproliferative disorders, presents with eosinophilia.
- Endocardial Fibroelastosis: Fibroelastic thickening of left ventricular endocardium, mainly in infants.
Summary Points
- Cardiomyopathies encompass a variety of diseases leading to myocardial dysfunction with distinct pathophysiological mechanisms.
- Accurate diagnosis and understanding of underlying causes are crucial for effective management and treatment strategies.
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Description
This quiz focuses on the definition and features of cardiomyopathy, including dilated, hypertrophic, and restrictive types. By participating, students will deepen their understanding of the clinical, pathophysiological, and pathological aspects of this condition. Ideal for those studying anatomical pathology.
