Path: Cardiomyopathies

Question 1

Most common form of cardiomyopathy.

Answer 1

Dilated

Question 2

Pathology of dilated cardiomyopathy.

Answer 2

Progression of dilation of heart chambers with mild hypertrophy of myocardium.

Systolic Dysfunction

Question 3

Most common cause of dilated cardiomyopathy.

Answer 3

Toxin-Induced (EtOH)

-chemotherapy agents (doxorubicin) can also cause it but are not as common

Question 4

Cause of dilated cardiomyopathy in children.

Answer 4

Mitochondrial Disease

Question 5

Most common X-linked cause of cardiomyopathy.

Answer 5

Dystrophin Gene Mutation

Question 6

Main two culprits that cause myocarditis that can lead to dilated cardiomyopathy.

Answer 6

1. Coxsackie B

2. Enterovirus

Question 7

Describe Arrhythmogenic RV Cardiomyopathy for real.

Answer 7

Inherited disease disease of the cardiac muscle that affects the RV leading to V-tach or fib and possibly sudden cardiac death.

Autosomal Dominant and very thin RV wall that accumulates fat tissue.

Question 8

Pathology of Hypertrophic Cardiomyopathy.

Answer 8

Genetic mutation of genes encoding sarcomere proteins. Leads to a loss of compliance.
-diastolic dysfunction

Question 9

Gross characteristic seen on pathological exam of a Hypertrophic Cardiomyopathy heart and region of the heart where most hypertrophy occurs.

Answer 9

Banana-like configuration. Ventricular septum hypertrophies the most and leads to a smaller left ventricular chamber size.

Question 10

How do cardiac myocytes appear on biopsy in a heart with Hypertrophic Cardiomyopathy?

Answer 10

completely disorganized thick muscle fibers

Question 11

Major cause of Restrictive Cardiomyopathy.

Answer 11

Amyloidosis: a variety of conditions where normally soluble proteins become insoluble and are deposited in the extracellular space of various organs or tissues, disrupting normal function, in this case the heart. Often associated with Diabetics.

Leads to a loss of compliance.
-diastolic dysfunction

Question 12

Method to diagnose amyloidosis on biopsy.

Answer 12

1. Congo Red dye stains the amyloid.

2. shine UV light to reveal apple green birefringence

Question 13

Name two conditions that lead to cardiomyopathy due to catecholamine overload.

Answer 13

1. Pheochromocytoma

2. Broken Heart Syndrome (Takotsubo)

Question 14

Why are sickle cell patients susceptible to cardiomyopathy?

Answer 14

They receive many blood transfusions leading hemosiderosis (high iron in the blood). The iron disrupts metal-dependent enzyme systems leading to dilated systolic dysfunction and oxygen free-radical damage.

Question 15

How can Cardiomyopathy caused by iron overload be diagnosed?

Answer 15

Prussian blue stain on biopsy

Question 16

Main two microbes that cause general myocarditis.

Answer 16

1. Coxsackie B virus

2. Adenovirus

Question 17

When looking at a biopsy of heart with myocarditis, what is the diagnosis if the biopsy shows:

1. many lymphocytes
2. Eosinophils
3. fused neutrophils
4. Protozoans

Answer 17

1. Viral
2. Hypersensitivity rxn
3. Giant Cell myocarditis
4. Chagas caused by Trypanosomi cruzi

Question 18

What is the diagnosis for a myocarditis that shows fused neutrophils within granulomas on biopsy?

Answer 18

Sarcoid Myocarditis
-Sarcoidosis is a disease with the formation of many non-caseating granulomas (granulomas with no necrosis in the center) in multiple organs. The cause is unknown but thought to be related to immune reactions to environmental, infectious or medical interaction.

Question 19

What is the treatment for any dilated cardiomyopathy?

Answer 19

Transplant

Question 20

Most common tumors in the heart.

Answer 20

Metastatic (secondary) Tumors

Question 21

Most common primary tumor in adult hearts.

Answer 21

Atrial Myxoma: may cause ball valve obstruction or embolism

Question 22

What is Carney Syndrome?

Answer 22

Autosomal Dominant inherited Myxoma disorder. Includes:

1. Multiple Myxomas
2. Skin Lesions - blue nevi, lentigines (small pigmented spot on skin)
3. Endocrine disorders - cushing, acromegaly

Question 23

Most common complication of Lipomas that form in the heart.

Answer 23

Ball Valve obstruction, must be removed or patient will die.

arrhythmias can also occur

Question 24

Site of Papillary Fibroelastomas and major complication.

Answer 24

Valve tissue (mainly papillary muscle)
Can embolize

Question 25

Most common primary tumor in children.

Answer 25

Rhabdomyoma

Question 26

Condition associated with rhabdomyoma formation and characteristic cells seen on biopsy.

Answer 26

Tuberous Sclerosis
-mutation in genes that code proteins that act as tumor growth suppressors. Tumors may form in many different organs.

Spider Cell seen on biopsy

Question 27

Two most common malignant neoplasms that are associated with the heart.

Answer 27

Angiosarcoma - forms in blood vessels around the heart.

Mesothelioma - forms in the membrane surrounding the heart and lungs

Question 28

Describe Carcinoid Heart Disease.

Answer 28

Paraneoplastic Syndrome involving a serotonin-secreting tumor. Mostly affects valves or involves plaque formation in the RIGHT SIDE OF THE HEART.

Rarely affects the left side.

Question 29

Why does Carcinoid Heart disease rarely affects the left side.

Answer 29

The carcinoid tumors are located elsewhere in the body, so the vasoactive amines released by the tumor arrive at the heart through the blood. They enter the right side first where the damage occurs and rarely make it past the lungs to affect the left side.