Cardiomyopathies & Cardiac Tumors

Question 11

What are the three types of cardiomyopathy?

Answer 11

Dilated
Hypertrophic
Restrictive

Question 12

What are the causes of dilated cardiomyopathy?

Answer 12

Genetics, idiopathic, alcoholism (Happy holiday syndrome)

Question 13

What type of cardiomyopathy can ‘Happy holiday’ syndrome cause?

Answer 13

Dilated cardiomyopathy

Question 14

What are the clinical features of dilated cardiomyopathy?

Answer 14

• Myocardium becomes flabby, weak and pale
• compensatory ventricular hypertrophy & dilation
• Exercise intolerance progresses to congestive heart failure
• 75% die within 5 years of onset without transplant, due to ventricular arrythmias (50%)

Question 15

What is hypertrophic cardiomyopathy?

Answer 15

• altered sarcomere function due to thick wall and small cavity of LV
• papillary mm and trabeculae carnae are prominent and encroach on lumen
• often asymmetric hypertrophy of interventricular septum

Question 16

A 16 year old star soccer player collapses on the field in the fourth quarter and expires from a massive cardiac failure of his left ventricle. What cardiomyopathy does this pattern indicate?

Answer 16

Hypertrophic cardiomyopathy

Question 17

A hypertrophic cardiomyopathy is an example of what type of aortic stenosis?

Answer 17

Subvalvular

Question 18

What is restrictive cardiomyopathy?

Answer 18

A group of diseases in which myocardial or endocardial abnormalities limit diastolic filling while contractile function is normal

Question 19

What pathologies can cause restrictive cardiomyopathy?

Answer 19

• Systemic amyloidosis
• hemachromatosis (^iron) (genetic & storage diseases)

Question 20

What type of cardiomyopathy can be caused by hemachromatosis and systemic amyloidosis?

Answer 20

Restrictive cardiomyopathy

Question 21

When does a myxoma present in life?

Answer 21

Adulthood

Question 22

What is the cell of origin for a myxoma?

Answer 22

Connective tissue (fibroblast)

Question 23

What is the preferential location for a myxoma?

Answer 23

Left atrium

Question 24

49 year old patient presents with a heart tumor in his left atrium composed of fibroblasts. What is the name given to this gelatinous mass?

Answer 24

Myxoma

Question 25

What age does a rhabdomyoma of the heart onset?

Answer 25

Pediatric

Question 26

What is the most common pediatric heart tumor?

Answer 26

Rhabdomyoma

Question 27

What is the cell of origin for a rhabdomyoma?

Answer 27

Striated cardiac myocytes

Question 28

Where does a rhabdomyoma preferentially exist?

Answer 28

Walls of ventricles

Question 29

8 year old male patient presents with a tumor in the right ventricle of his heart composed of striated cardiac myocytes. What type of tumor is described?

Answer 29

Rhabdomyoma

Question 30

What is a hamartoma?

Answer 30

benign tumor composed of the primary tissue of an organ

Question 31

What cardiomyopathy can be caused by holiday heart syndrome or Saturday night syndrome? Why?

Answer 31

Dilative cardiomyopathy due to a B1 deficiency

Question 32

In a restrictive cardiomyopathy, the heart has trouble in (Systole/Diastole)?

Answer 32

Diastole

Question 33

What is the most common etiology of sudden cardiac death in people under 35 years of age?

Answer 33

Hypertrophic cardiomyopathy

Question 34

What is the most common etiology of sudden cardiac death in people over 35 years of age?

Answer 34

Coronary heart disease

Question 35

What is the most common cardiac primary tumor in adults?

Answer 35

Myxoma

Question 36

36 year old female patient is diagnosed with a mass in her heart. The mass is gelatinous upon biopsy measuring 5 cm. The mass appears to be a mix of connective tissue and is situated in the left atrium. What is the diagnosis?

Answer 36

Myxoma

Question 37

A rhabdomyoma is a (Benign/Malignant) neoplasm of (Smooth/Striated) muscle cells?

Answer 37

Benign, striated

Question 38

9 year old male patient is diagnosed with a mass in the left ventricle of their heart. The mass appears to be benign. What is the most common tumor based on this patients age and location?

Answer 38

Rhabdomyoma

Question 39

Metastasis to the heart is common (TRUE/FALSE)?

Answer 39

FALSE

Question 40

What cancers most commonly metastasize to the heart?

Answer 40

Lung, breast, and GI tract

Question 41

How does dilated cardiomyopathy result in reduced stroke volume and cardiac output?

Answer 41

poorly contracting heart (weak) causes ^stretch & compliance -> valves become regurgitant -> less blood pumped out

Question 42

What is dilated cardiomyopathy?

Answer 42

Genetic defects in cytoskeletal proteins lead to hypothesis that **defects in force transmission** lead to development of a **dilated, poorly contracting heart**

Question 43

What is the most notable histologic feature of hypertrophic cardiomyopathy?

Answer 43

myofiber disarray

Question 44

What population is hypertrophic cardiomyopathy seen in?

Answer 44

young athletes, but can occur at any age

Question 45

What are the symptoms of hypertrophic cardiomyopathy?

Answer 45

- asymptomatic, although at risk for sudden death - angina, dyspnea, syncope which may progress to heart failure

Question 46

What is the result of restrictive cardiomyopathy on blood flow?

Answer 46

decreased compliance -> decreased filling (diastole) -> decreased SV & Q