Pathology: Cardiomyopathy

Question 1

What causes Cardiomyopathies?

Answer 1

Question 2

• Three clinical, functional, and pathologic patterns are recognized.
  
  • What are they?
  • Which one is most common, which is the least common?
  • Which are associated with diastolic dysfunction and which are associated with systolic dysfunction?

Answer 2

Question 3

For this type of Cardiomyopathy

• Describe the LC ejection fraction
• Mechanism of heart failure
• General Causes
• Secondary Myocardial Dysfunction

Dilated

Answer 3

Question 4

For this type of Cardiomyopathy

• Describe the LC ejection fraction
• Mechanism of heart failure
• General Causes
• Secondary Myocardial Dysfunction

Hypertrophic

Answer 4

Question 5

For this type of Cardiomyopathy

• Describe the LC ejection fraction
• Mechanism of heart failure
• General Causes
• Secondary Myocardial Dysfunction

Hypertrophic

Answer 5

Question 6

Dilated Cardiomyopathy

• What are the two main characteristics?
• What is it usually concurrent with?
• Why is early dx important for this disease?

Answer 6

Question 7

• Genetic and epidemiologic studies suggest that at least five pathways can lead to end-stage Dilated Cardiomyopathy
  
  • List them

Answer 7

Question 8

Genetic Causes of Dilated Cardiomyopathy

• What percentage of cases are genetic?
• What kind of inheritance is usually the case?
• What mutations are involved?
• What protein is associated with the X-chromosome?

Answer 8

Question 9

Mutations in what protein accounts for approximately 20% of all dilated
cardiomyopathy?

Answer 9

Question 10

Pathogenesis of Dilated Cardiomyopathy

• Infection with what virus is most common in late-stage DCM?
• What condition is a common precursor to DCM?

Answer 10

Question 11

Pathogenesis of Dilated Cardiomyopathy: Toxic exposure

• What are some of the toxic agents that can cause DCM?
  
  • Which agent is a chemotherapy drug?
• What is heart disease is associated with chronic alcohol abuse and its related consequences?

Answer 11

Question 12

Peripartum Cardiomyopathy

• When does this occur in pregnancy?
• What can cause this condition? (5)
• What is the primary defect in this condition, and what does it lead to?

Answer 12

Question 13

• How does Iron Overload lead to DCM?
• What genetic condition can lead to iron overload?
• What other type of cardiomyopathy can it cause?

Answer 13

Question 14

Morphology of Dilated Cardiomyopathy

• What are the general changes that occur to the heart?
• What type of thrombi are often present?

Answer 14

Question 15

• In DCM
  
  • What happens to most myocytes?
  • What areas of the heart show fibrosis?
• In DCM secondary to iron overload,
  
  • What accumulates in cells

Answer 15

Question 16

Clinical Features of Dilated Cardiomyopathy

• What age do most patients get dx with this?
• What condition is usually associated with this?
  
  • What symptoms do PTs present with?
• What is the prognosis for patients?
  
  • How do they usually die?
• What is the only definitive treatment for DCM?

Answer 16

Question 17

Arrhythmogenic Right Ventricular Cardiomyopathy

• What kind of genetic order is this?
• What cardiac manifestation accompanies this condition?
• How prevalent is this condition?
• What specific population has 10% of its sudden deaths attributed to it?
• What happens to the right ventricle wall, and why?

Answer 17

Question 18

How can you tell that this heart and histological stain are from a patient who had Arrhythmogenic Right Ventricular Cardiomyopathy?

Answer 18

Question 19

What proteins are implicated in Arrhythmogenic Right Ventricular Cardiomyopathy?

What puts stress on these proteins that eventually make them detach?

Answer 19

Question 20

Hypertrophic Cardiomyopathy

• What are the 3 main characteristics of this condition?
• How does the shape of the heart change?
• What happens to systolic function?

Answer 20

Question 21

What two disorders can hypertrophic cardiomyopathy be mistaken for?

Answer 21

Question 22

Hypertrophic Cardiomyopathy

• What type of mutations are usually the cause of this condition?
  
  • What type of genes are usually mutated?
  • What kind of inheritance pattern is usually the case?
• What is the result of most of the mutations?

Answer 22

Question 23

In hypertrophic cardiomyopathy,

• What is the most frequently involved sarcomeric protein?
  
  • What 2 other proteins are commonly involved? (These make up the majority of all cases of HCM)

Answer 23

Question 24

Morphology of Hypertrophic Cardiomyopathy

• What happens to the ventricles and myocardium?
  
  • What occurs in about 10% of cases?
• What shape does this ventricle start to adopt?
• What happens to the mitral valve, particularly the anterior leaflet, during ventricular systole?
  
  • What does this produce?

Answer 24

Question 25

Histologic features in HCM * What happens to the myocytes? * What kind of fibrosis is seen, if any?

Answer 25

Question 26

Clinical Features of Hypertrophic Cardiomyopathy * When does this usually manifest in patients? * What happens to the left ventricle and stroke volume? Why? * What happens in about 25% of patients that is related to this? * **A harsh systolic ejection murmur** is often heard. What is this a sign of?

Answer 26

Question 27

Clinical Features of Hypertrophic Cardiomyopathy * What type of death is often associated with HCM? * In HCM, Myocardial ischemia (with angina) is often observed? What is the cause of this? * What are the major clinical problems that result from HCM? * What type of therapies usually helps patients?

Answer 27

Question 28

Restrictive Cardiomyopathy * What happens to the ventricles, what does this do to diastole? * What are the common causes of systemic diseases that cause this disorder? * What are the 3 main forms of Restrictive Cardiomyopathy?

Answer 28

Question 29

* What form of Restrictive Cardiomyopathy is associated with insoluble β-pleated sheets? * What kind of systemic condition can this occur in? * Can this form of Restrictive Cardiomyopathy be predominantly restricted to the heart? * What racial group has a 4% risk of developing this?

Answer 29

Question 30

Restrictive Cardiomyopathy: Endomyocardial Fibrosis * What population does this mainly affect? * What is it characterized by? What valves are affected? * What conditions has it been linked to?

Answer 30

Question 31

Loeffler Endomyocarditis * What area of the heart does this effect, and how? * What is characterized by? '

Answer 31

Question 32

Morphology of Restrictive Cardiomyopathy * What is notable about the myocardium? * What is notable about the atria? Why?

Answer 32

Question 33

Morphology of Restrictive Cardiomyopathy * What does microscopic examination usually reveal?

Answer 33

Interstitial fibrosis

Question 34

This person has a special type of restrictive CM. * What kind is it, and how can you tell?

Answer 34