Cardaic Pathology Pt 2

Question 1

What is the MC structural birth defect?

Answer 1

Congenital heart disease

Question 2

What is congenital heart disease?

Answer 2

Abnormality or defect that affects the heart or great vessel which is present before birth

Question 3

What are some causes of congenital heart disease?

Answer 3

Most commonly due to sporadic genetic mutations

Also environmental factors (ex fetal alcohol syndrome)

Question 4

What are the most common congenital heart disease?

Answer 4

Septal defects 
(VSD #1)

Question 5

How do ASD and VSD affect RV and pulmonary outflow pressure and volume?

Answer 5

They both increase RV and pulmonary outflow volumes and pressure
Whereas PDA increases pulmonary outflow volume and pressure only

Question 6

What is the result of increased pulmonary pressure?

Answer 6

Increases pulmonary vascular resistance which will result in Eisenmenger syndrome

Question 7

Does staph aureus cause acute or subacute endocarditis?

Answer 7

Acute

Question 8

Is early (<2 months) prosthetic valve infection typical with staph aureus infection?

Answer 8

Yes

Question 9

What are risk factors for infective endocarditis caused by staph aureus?

Answer 9

IV drug abuse, very common with prosthetic valves

Question 10

What is the #1 cause of endocarditis?

Answer 10

Staph aureus

Question 11

What are unique features of staph aureus?

Answer 11

Right sided (tricuspid valve) endocarditis associated with IV drug abuse

Question 12

What are causes of subacute infective endocarditis?

Answer 12

Strep viridans, enterococci, staph epidermidis, HACEK group

Question 13

Is early (<2 months) prosthetic valve infection typical with strep viridans, enterococci, staph epidermidis, or the HACEK group?

Answer 13

Only for staph epidermidis

Question 14

What are risk factors for strep viridans?

Answer 14

Dental procedures/poor dentition

Question 15

What are unique features of strep viridans?

Answer 15

1 for native, structurally abnormal

Question 16

What are risk factors and unique features for staph epidermidis?

Answer 16

Very common with prosthetic valves

Unique feature is that its present in normal skin flora

Question 17

What are risk factors for the HACEK group?

Answer 17

Dental procedures/poor dentition

Question 18

What are unique features of the HACEK group?

Answer 18

Rare

<2% of endocarditis cases

Question 19

Which organisms are apart of the HACEK group?

Answer 19

Haemophilus
Aggregatibacter (previously actinobacillus)
Cardiobacterium
Eikenella
Kingella

Question 20

What are the two types of prosthetic valves?

Answer 20

Mechanical and tissue valves

Question 21

What are mechanical valves?

Answer 21

Caged ball, tilting disk, hinged flap (bi-leaflet)

Risk of thromboembolism

Question 22

What are tissue valves (bioprostheses)?

Answer 22

Bovine (cow) or porcine (pig)

Risk of mechanical failure (tearing, incompetence)

Question 23

Both types of prosthetic valves have a risk of what?

Answer 23

Infective endocarditis

Question 24

What are other risks associated with prosthetic valves?

Answer 24

Anti-coagulant related hemorrhage (lifelong anticoagulation with mechanical valves)
Dysfunction (regurgitation or exuberant healing)
Hemolytic anemia

Question 25

Which prosthetic valve lasts longer?

Answer 25

Mechanical valves last a long time (greater than 25 years) whereas tissue valves last 10-15 years

Question 26

What type of dysfunction is seen with dilated cardiomyopathy?

Answer 26

Systolic dysfunction

Question 27

What is the principal cardiac abnormality in dilated cardiomyopathy?

Answer 27

Enlargement of all chambers

Question 28

What are some additional features of dilated cardiomyopathy?

Answer 28

Most common type | Multiple causes

Question 29

What type of dysfunction is seen with hypertrophic cardiomyopathy?

Answer 29

Diastolic dysfunction

Question 30

What is the principal cardiac abnormality seen with hypertrophic cardiomyopathy?

Answer 30

Thickened left ventricular wall (septal)

Question 31

What are additional features associated with hypertrophic cardiomyopathy?

Answer 31

Risk of sudden death in young athletes | Beta myosin heavy chain mutation (common mutation)

Question 32

What type of dysfunction is seen with restrictive cardiomyopathy?

Answer 32

Diastolic dysfunction

Question 33

What is the principal cardiac abnormality seen with restrictive cardiomyopathy?

Answer 33

Rigid ventricular walls

Question 34

What are additional features associated with restrictive cardiomyopathy?

Answer 34

``` Least common type Multiple causes (especially amyloidosis) ```

Question 35

What is iron overload cardiomyopathy?

Answer 35

Hereditary hemochromatosis caused by mutation in HFE gene

Question 36

How does hereditary hemochromatosis affect different systems of the body?

Answer 36

Skin (pigmentation), pancreas (diabetes) = bronze diabetes Heart (dilated cardiomyopathy and arrhythmia) Liver (hepatosplenomegaly), bone (arthritis), testicles (hypogonadism)

Question 37

What are other types of restrictive cardiomyopathy?

Answer 37

Endomyocardial fibrosis Loeffler endocarditis Endocardial fibroelastosis

Question 38

What is endomyocardial fibrosis?

Answer 38

Children and young adults in tropical and subtropical regions (Africa) Fibrosis of endocardium and subendocardium Sx: Dyspnea with exertion, fatigue, paroxysmal nocturnal dyspnea, orthpnea

Question 39

What is Loeffler endocarditis?

Answer 39

Part of spectrum of endomyocardial fibrosis Shows eosinophilic infiltration Associated with myeloproliferative disorders (leukemia/lymphoma) Seen in subtropical areas Sx: Fever, cough, rash

Question 40

What is endocardial fibroelastosis?

Answer 40

Fibroelastic thickening of left ventricular endocardium Presents in the first 2 years of life Associated with congenital heart defects (aortic stenosis, atresia, etc) S/S: Infant with feeding difficulty, breathlessness, failure to thrive and wheezing

Question 41

What is the most common functional pattern?

Answer 41

Dilated

Question 42

What is the least common functional pattern?

Answer 42

Restrictive

Question 43

What is the mechanism of heart feature in dilated functional pattern?

Answer 43

Impairment of contractility (systolic dysfunction)

Question 44

What are the causes of the phenotype associated with dilated functional pattern?

Answer 44

``` Genetic Alcohol Peripartum Hemochromatosis Doxorubicin toxicity ```

Question 45

What is the mechanism of heart failure associated with hypertrophic functional pattern?

Answer 45

Impairment of compliance (diastolic dysfunction)

Question 46

What are the causes of the phenotype associated with hypertrophic functional pattern?

Answer 46

Genetic (hypertrophic cardiomyopathy)

Question 47

What is the mechanism of heart failure for restrictive functional pattern?

Answer 47

Impairment of compliance (diastolic dysfunction)

Question 48

What are the causes of the phenotype associated with restrictive functional pattern?

Answer 48

Amyloidosis | Radiation induced fibrosis

Question 49

What is the MC form of myocarditis?

Answer 49

Lymphocytic myocarditis | Seen both in viral/post viral infection, autoimmune or idiopathic

Question 50

What is eosinophilic myocarditis?

Answer 50

Characterized by marked increased eosinophils Hypersensitivity myocarditis due to underlying allergy (hypersensitivity) reaction (typically drug reaction) Can be idiopathic Must exclude parasitic infection

Question 51

What are the types of myocarditis with giant cells?

Answer 51

Idiopathic giant cell myocarditis | Myocardial sarcoidosis

Question 52

What is idiopathic giant cell myocarditis?

Answer 52

Aggressive, poor prognosis, survival less than 3 months from onset Giant cells admired with variable mixed inflammation (typically increased eosinophils)

Question 53

What is myocardial sarcoidosis?

Answer 53

Idiopathic process, rare cause of myocarditis, variable presentation Giant cells with non-necrotizing granulomas