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Unit 4: Path - The Heart > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

What is cardiomyopathy?

A

Primary disease of the myocardium excluding damage by extrinsic factors

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2
Q

What are the subtypes of cardiomyopathy?

A
  • Dilated cardiomyopathy (DCM)
  • Hypertrophic cardiomyopathy (HCM)
  • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
  • Restrictive Cardiomyopathy (RCM)
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3
Q

What is the most common type of cardiomyopathy? What are the subtypes?

A

Dilated cardiomyopathy

  1. Secondary
  2. Primary
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4
Q

What type of genetic transmission is most common in DCM?

A

Autosomal dominant

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5
Q
  1. What are mutated genes that encode for cytoskeletal proteins that result in DCM?
  2. What are the mutated genes that encode for anchoring the cytoskeleton and the sarcolemma to the ECM?
  3. What is the hypothesis of the genetic mutations that cause DCM?
A
  1. Lammin A/C, Desmin, Metavinculin
  2. Delta-sarcoglycan, dystrophin
  3. Defects in force transmission lead to development of a dilated poorly contracting heart
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6
Q

Besides genetic factors, what other factors can lead to DCM?

A

Viral myocarditis and immunologic abnormalities

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7
Q

What is your Dx? What are the macroscopic findings? Microscopic findings?

A

Idiopathic Dilated Cardiomyopathy; Heart weight tripled, flabby/pale myocardium, conspicuous left/right ventricular dilation; Cardiacmyocytes show degeneration (Myocytolysis), perivascular fibrosis

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8
Q

Pathogenesis of DCM?

A
  1. Compensatory ventricular hypertrophy
  2. Asymptomatic left ventricular dilation
  3. Decrease exercise tolerance
  4. Congestive HF
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9
Q

What is the single most identifiable cause of DCM in the US?

A

Ethanol

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10
Q

How can catecholamines cause DCM?

A

High concentrations of catechols cause focal myocyte necrosis (contraction band necrosis). Major mechanism is enhanced calcium influx into myocytes

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11
Q

What types of medications are notorious for causing cardiomyopathy?

A
  • Anthracyclines (ie doxorubicin) - mjr clinical effect - cardiac myocyte degeneration and decreased contractility
  • Cyclophosphamide
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12
Q

Describe the cardiac complications of pregnancy. Describe the molecular pathogenesis

A

In the last trimester or first 6 months following delivery, cardiomyopathy can occur.

  1. Increased levels of biologically active prolactin
  2. Results in increased blood volume, decreased blood pressure and diminshed renal excretion of water, sodium, and potassium
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13
Q

What are the two genes associated with 80% of patients with HCM?

A

Beta-myosin and myosin binding protein C

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14
Q

What is the proposed mechanism leading to HCM?

A

Mutant proteins are incorporated into sarcomeres where it acts as a dominant-negative fashion to cause loss of sarcomeric function. Therefore, there is altered force generation leading to compensatory hypertrophy

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15
Q

What is you Dx? What macroscopic characteristics point toward this Dx?

A

Hypertrophic Cardiomyopathy; Asymmetric hypertrophy of the left ventricular wall (especially the septum), Septum bulges into left ventricle obstructing ventricular systole

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16
Q

What are these histologic findings characteristic of? What’s going on here? Where are these changes most commonly seen?

A

Hypertrophic cardiomyopathy; Myofiber disarray - oblique and perpendicular myocytes; Interventricular septum

17
Q

What Syx are observed in HCM? What are patients with HCM at risk of? What are the complications of HCM in the heart?

A

Rarely any!; Sudden cardiac death; Ejection fraction and SV are high, muscle compliance is low (diastolic dysfn.)

18
Q

What are the Tx for HCM?

A
  1. Beta Blockers
  2. Calcium Blockers
  3. Surgical removal of portion of the heart to unblock systolic obstruction
19
Q

The heart of a young Mediterranean who experienced a sudden death is shown below. What is your Dx? What is the molecular pathogenesis of this condition?

A

Arrhythmogenic Right Ventricular Cardiomyopathy; Mutations in genes encoding proteins in desmosomes are the main culprit in the pathogenesis

20
Q

What is restrictive cardiomyopathy? Describe how it causes pathology

A

Group of diseases in which myocardial or endocardial abnormalities limit diastolic filling while contractile function remains normal.

21
Q

What are the four major causes of restrictive cardiomyopathy?

A
  1. Amyloid/Metastatic Carcinoma/Sarcoid Granules
  2. Endomyocardial disease characterized by fibrotic thickening
  3. Genetic storage disease (ie hemochromatosis)
  4. Marked increase in interstitial fibrous tissue
22
Q

What are the anatomical changes found in restrictive cardiomyopathy?

A
  1. Increased preload
  2. Defective diastolic compliance
  3. Restricted ventricular filling
  4. Atrial Dilation
  5. Venous Congestion
23
Q

What is the relationship between amyloidosis and the dimensions of the heart?

A

Amyloid infiltration results in cardiac enlargement without ventricular dilation

24
Q

What are the main Syx in cardiac amyloidosis?

A

Right sided heart failure

25
Q

What protein is deposited in senile cardiac amyloidosis? What are the Syx?

A

Transthyretin (prealbumin); Usually no Syx

26
Q

What are the two subtypes of endomyocardial disease?

A
  1. Endomyocardial fibrosis - Africa
  2. Eosinophilic Endomyocardial Disease (Loeffler endocarditis) - Temperate regions - hypereosinophilia
27
Q

What is the believed mechanism of myocardial injury?

A

Myocardial injury caused by eosinophils and possibly cardiotoxic granule components

28
Q

What is the pathogenesis of EMD?

A
  1. Necrotic Stage - First Few Months - Eosinophilic infiltrate with vascular injury and myocyte necrosis
  2. Thrombotic Stage - One year - Attached mural thrombi
  3. Fibrotic stage - Chronic - Conspicuous fibrotic thickening of the endocardium
29
Q

Describe the effects of EMD on the valves of the heart

A

Adherence of the posterior mitral valve leaflet to endocardium - mitral regurg

Adherence of tricuspid valve leaflet - tricuspid regurg

30
Q

What are 4 storage diseases that have restrictive cardiomyopathic changes?

A
  1. Glycogen storage disease
  2. Mucopolysaccharidoses
  3. Sphingolipidoses
  4. Hematochromatosis
31
Q

What glycogen storage diseases affect the heart? Which is the most severe?

A
  1. II (Pompe Disease)
  2. III (Cori Disease)
  3. IV (Andersen Disease)

Pompe is the worst

32
Q

What two Sphingolipidoses commonly cause cardiac complications?

A
  • Fabry Disease - Glycosphingolipid accum. w/i heart
  • Gaucher Disease - rarely involves the heart
33
Q

A patient expires and on autopsy, the heart is dilated and the ventricular walls are thickened. The myocardium is notably browned. The patient appears to have died of congestive heart disease. What is the Dx? What is the best predictor of disease progression?

A

Hemochromatosis; Severity of myocardial dysfn. is proportional to quantity of deposited iron.

34
Q

A patient has a heart block and dies of arrhythmia. A biopsy of the interventricular septum shows noncaseating granulomas with prominent giant cells. What is the Dx? What part of the heart does this disease have a tropism for?

A

Cardiac sarcoidosis

Unit 4: Path - The Heart (14 decks)

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