Pathology of Cardiomyopathies, Hypertension, & Myocarditis

Question 1

Mutations of what protein are most commonly associated with dilated cardiomyopathy?

Answer 1

Titin (truncation of the TTN gene)

Question 2

What is the mechanism of heart failure due to hypertrophic cardiomyopathy?

Answer 2

Impairment of compliance (diastolic dysfunction)

Question 3

What is myocarditis?

Answer 3

Myocardial inflammation with necrosis

Question 4

What is the classic histologic finding of malignant hypertension?

Answer 4

Hyperplastic necrotizing arteriolitis

Question 5

This is a type of left ventricular contractile dysfunction caused by extreme psychological stress.

Answer 5

Takotsubo cardiomyopathy (broken heart syndrome)

Question 6

Cardiac hypertrophy leads to an increase in mycoyte size. What other histologic findings may be present?

Answer 6

Multiple and enlarged nuclei due to DNA replication without cell division

Question 7

What is a classic histological finding of hypertrophic cardiomyopathy?

Answer 7

Myocyte disarray (top is normal)

Question 8

Mural thrombi are most associated with what type of cardiomyopathy?

Answer 8

Dilated cardiomyopathy

Question 9

Hemochromatosis may present with what type of cardiomyopathy?

Answer 9

Features of both dilated and restrictive cardiomyopathy (systolic and diastolic impairment)

Question 10

What are the different types of cardiomyopathy?

Answer 10

Dilated, hypertrophic, restrictive

Question 11

What cells are present in cases of hypersensitivity myocarditis?

Answer 11

Eosinophils, lymphocytes, macrophages

Question 12

What characterizes sarcoidosis restrictive cardiomyopathy?

Answer 12

Non-necrotizing granulomas with prominent giant cells

Question 13

What is peripartum cardiomyopathy?

Answer 13

A type of dilated cardiomyopathy that occurs late in pregnancy or up to 6 months postpartum

Question 14

In what age group is myocarditis most common?

Answer 14

Children 1-10yo

Question 15

This patient has a type of myocarditis. What is it?

Answer 15

Giant cell myocarditis - presence of multinucleated giant cells

Question 16

True/False. Deficits of mental function may present in cases of right-sided heart failure.

Answer 16

True - due to hypoxia of the CNS. This is most common in older patients

Question 17

This type of cardiomyopathy is characterized by massive fatty infiltration and focal fibrosis.

Answer 17

Arrhythmogenic cardiomyopathy (type of dilated cardiomyopathy)

Question 18

What cells may be found in the lungs in cases of left-sided heart failure?

Answer 18

Hemosiderin-laden macrophages (heart failure cells) - accumulated iron from phagocytized RBCs

Question 19

What toxins are most associated with dilated cardiomyopathy?

Answer 19

Alcohol, cobalt, chemotherapeutic drugs

Question 20

This cause of restrictive cardiomyopathy is linked to nutritional deficiencies and parasitic infections.

Answer 20

Endomyocardial fibrosis

Question 21

What is the most common cause of sudden cardiac death in young athletes?

Answer 21

Hypertrophic cardiomyopathy

Question 22

Volume overload causes assembly of new sarcomeres in (series/parallel).

Answer 22

Series - eccentric hypertrophy

Question 23

Restrictive cardiomyopathy due to this cause presents in the first 2 years of life.

Answer 23

Endocardial fibroelastosis

Question 24

What histological findings are present with dilated cardiomyopathy?

Answer 24

Enlarged, bizarre, hyperchromatic nuclei (“ninja stars”

Question 25

Chronic alcohol use is associated with what cardiomyopathy?

Answer 25

Dilated cardiomyopathy

Question 26

In cases of right heart failure, hepatic and portal vessels become congested and cause hepatosplenomegaly. What is the characteristic term to describe the liver’s appearance in these cases?

Answer 26

Nutmeg liver

Question 27

Nephrosclerosis occurs during congestive heart failure due to hemodynamic changes. What gross and histologic changes are present?

Answer 27

Thickening, hyalinization of arteries, decreased kidney mass, nodular developement

Question 28

What is senile cardiac amyloidosis?

Answer 28

A type of restrictive cardiomyopathy due to deposits of amyloid with transthyretin

Question 29

This type of restrictive cardiomyopathy is associated with extensive eosinophilic infiltrate.

Answer 29

Loeffler endomyocarditis

Question 30

What are the most common gene mutations associated with hypertrophic cardiomyopathy?

Answer 30

Myosin-binding protein C & B-myosin heavy chain

Question 31

True/False. Expression of fetal genes occurs during cardiac hypertrophy.

Answer 31

True - fetal forms of myosin, natriuretic peptides, and collagen are present

Question 32

What virus is most associated with myocarditis in the USA?

Answer 32

Coxsackie viruses A & B

Question 33

What is the presentation of malignant hypertension?

Answer 33

Rapid rise in blood pressure, renal failure, retinal hemorrhages, papilledema

Question 34

Pressure overload causes assembly of new sarcomeres in (series/parallel).

Answer 34

Parallel - concentric hypertrophy

Question 35

What is the mechanism of heart failure due to dilated cardiomyopathy?

Answer 35

Impairment of contractility (systolic dysfunction)

Question 36

What non-viral pathogen is associated with myocarditis?

Answer 36

Trypanosoma cruzi (agent of Chagas disease)

Question 37

What is Naxos Syndrome?

Answer 37

Arrhythmogenic right ventricular cardiomyopathy with woolly hair and hyperkeratosis