Pathology: Cardiomyopathy Flashcards
What causes Cardiomyopathies?
- 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?
For this type of Cardiomyopathy
- Describe the LC ejection fraction
- Mechanism of heart failure
- General Causes
- Secondary Myocardial Dysfunction
Dilated
For this type of Cardiomyopathy
- Describe the LC ejection fraction
- Mechanism of heart failure
- General Causes
- Secondary Myocardial Dysfunction
Hypertrophic
For this type of Cardiomyopathy
- Describe the LC ejection fraction
- Mechanism of heart failure
- General Causes
- Secondary Myocardial Dysfunction
Hypertrophic
Dilated Cardiomyopathy
- What are the two main characteristics?
- What is it usually concurrent with?
- Why is early dx important for this disease?
- Genetic and epidemiologic studies suggest that at least five pathways can lead to end-stage Dilated Cardiomyopathy
- List them
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?
Mutations in what protein accounts for approximately 20% of all dilated
cardiomyopathy?
Pathogenesis of Dilated Cardiomyopathy
- Infection with what virus is most common in late-stage DCM?
- What condition is a common precursor to DCM?
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?
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?
- How does Iron Overload lead to DCM?
- What genetic condition can lead to iron overload?
- What other type of cardiomyopathy can it cause?
Morphology of Dilated Cardiomyopathy
- What are the general changes that occur to the heart?
- What type of thrombi are often present?
- In DCM
- What happens to most myocytes?
- What areas of the heart show fibrosis?
- In DCM secondary to iron overload,
- What accumulates in cells
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?
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?
How can you tell that this heart and histological stain are from a patient who had Arrhythmogenic Right Ventricular Cardiomyopathy?
What proteins are implicated in Arrhythmogenic Right Ventricular Cardiomyopathy?
What puts stress on these proteins that eventually make them detach?
Hypertrophic Cardiomyopathy
- What are the 3 main characteristics of this condition?
- How does the shape of the heart change?
- What happens to systolic function?
What two disorders can hypertrophic cardiomyopathy be mistaken for?
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?
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)
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?
Histologic features in HCM
- What happens to the myocytes?
- What kind of fibrosis is seen, if any?
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?
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?
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?
- 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?
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?
Loeffler Endomyocarditis
- What area of the heart does this effect, and how?
- What is characterized by?
’
Morphology of Restrictive Cardiomyopathy
- What is notable about the myocardium?
- What is notable about the atria? Why?
Morphology of Restrictive Cardiomyopathy
- What does microscopic examination usually reveal?
Interstitial fibrosis
This person has a special type of restrictive CM.
- What kind is it, and how can you tell?
