MHD - Lec #9 - Valvular Heart Disease

Question 1

The haphazard pattern of myocyte organization and interstitial fibrosis is a hallmark of _____

Answer 1

Hypertrophic Cardiomyopathy

Question 2

What is the inheritance pattern of hypertrophic cardiomyopathy

Answer 2

Autosomal dominant

Question 3

Peripartum abnormalitites due to prolactin levels are often associated with what cardiomyopathy?

Answer 3

Dilated Cardiomyopathy

• genetic
• idiopathic
• peripartum
• toxin

Question 4

What proteins are affected by hypertrophic cardiomyopathy. (3)

Does this lead to a gain or loss of function?

Answer 4

1. β-myosin heavy chain i
2. myosin-binding protein C
3. troponin I/T.

Gain of function resulting in a hyper contractile state.

Mutations in these three genes account for 70% to 80% of all cases of HCM.
The diverse mutations underlying HCM have one unifying feature: they all affect sarcomeric proteins and increase myofilament activation.

Question 5

Which of the following (dilated or hypertrophic cardiomyopathy) is due to 100% genetic causes?

Answer 5

HYPERTROPHIC

Question 6

What type of murmur is associated with hypertrophic cardiomyopathy?

Answer 6

Harsh systolic ejection murmur

Symptoms:
Diastolic heart failure
Exertional dyspnea
Anginal pain
Intractable heart failure
Arrhythmias

Question 7

What is an amyloid?

Which cardiomyopathy is it associated with?

Answer 7

Misfolded protein –> BETA -PLEADED SHEET –> FIBRILS!

• Deposits in extracellular space
• Causes tissue damage

RESTRICTIVE CARDIOMYOPATHY

• LV normal but STIFF
• Left Atrium dilates due to stiffness of LV
• diastolic heart failure due to decreased filling

Question 8

What are the 3 most common features of amyloid deposits, regardless of protein?
(Hint: when is it red, when is it green)

Answer 8

1. B-pleated sheet” configuration
2. Congo red staining in tissue
3. “apple-green” under polarized light

Question 9

Infections with ______ are the most common etiology of myocarditis in the US

Answer 9

coxsackie A & B

-Cytomegalovirus
Human immunodeficiency virus
are 2nd

Question 10

What are some non-infectious causes of myocarditis?

Answer 10

1. Hypersensitivity reactions
2. Rheumatic fever
3. Giant cell myocarditis
4. Sarcoidosis

Question 11

What can myocarditis progress into?

Answer 11

Dilated cardiomyopathy

• arrhythmia
• acute congestive heart failure

viral infection today, but no presentation for years
as the virus resolves there may be smoldering inflammation that goes on for years = DILATED CARDIOMYOPATHY
how do we know if coxsackie? (can find enteroviral particles in myocardium)

Question 12

Tuberculosis*
Malignancy*

result in what type of pericarditis?

Answer 12

HEMORRHAGIC

TB also results in caseous pericarditis