Pathology

Question 1

L to R shunts

Answer 1

late cyanosis “ blue kids

Question 2

what is the frequency of L to R shunts?

Answer 2

VSD>ASD>PDA

Question 3

VSD

Answer 3

most common congenital cardiac defect

asymptomatic at birth, may manifest weeks later or remain asymptomatic throughout life

Most self resolve

larger lesions may lead to LV overload and HF

Question 4

ASD

Answer 4

defect in interatrial septum

loud S1

wide, fixed split S2

Ostium secundum defects most common and usually occur as isolated findings

this is different than patent foramen ovale, in that it is not a problem in not fusing but rather the septa is missing tissue

Question 5

PDA

Answer 5

in the fetal period, the shunt is R to L which is normal

in the neonatal period, you have a decrease in lung resistance so the shunt becomes L to R causing progressive RVH and/or LVH and HF

Question 6

wide fixed split S2, loud S1

Answer 6

ASD

Question 7

continuous machine like murmur

Answer 7

PDA

Question 8

how do you keep a PDA open?

Answer 8

maintained by PGE synthesis and low O2 tension

Question 9

how do you close a PDA?

Answer 9

indomethacin

Question 10

Where is a PDA heard best?

Answer 10

left infraclaviular region with max intensity at S2 (inspiratory splitting ofS2)

Question 11

PDA is associated with what maternal condition

Answer 11

Maternal Rubella (maculopapular rash begins in face and spreads down the body)

Question 12

Eisenmenger syndrome

Answer 12

Uncorrected L to R shunt (VSD ASD PDA) –> increase in pulmonary blood flow –> pathologic remodeling of vasculature –> pulmonary arterial hypertension

RVH occurs to compensate, the shunt then becomes right to left

Question 13

What do you see in Eisenmenger syndrome?

Answer 13

late cyanosis, clubbing, and polycythemia

Question 14

COA

Answer 14

aortic narrowing near insertion of ductus arteriosus (“juxtaductal”)

Question 15

COA

Answer 15

aortic narrowing near insertion of ductus arteriosus (“juxtaductal”)

Question 16

COA is associated with

Answer 16

bicuspid aortic valve, other heart defect and Turner syndrome

Question 17

what will you see in COA?

Answer 17

hypertension in upper extremities and weak, delayed pulse in lower extremities (brachial-femoral delay)

with age, collateral arteries erode ribs (notched appearance on CXR)

Question 18

what are 2 consequences of COA

Answer 18

bacterial endocarditis, cerebral hemorrhage

Question 19

what defect will you have from Alcohol exposure in utero (F.A.S)?

Answer 19

VSD, ASD, PDA, TOF

Question 20

what defect will you have from congenital rubella?

Answer 20

septal defects, PDA, Pulmonary artery stenosis

Question 21

what defect will you have from Down Syndrome

Answer 21

AV septal defect (endocardial cushion defect), VSD, ASD

Question 22

what defect will you have from infant of diabetic mother

Answer 22

Transposition of great vessels

Question 23

what congenital defect will you have from Marfan Syndrome

Answer 23

MVP, thoracic aortic aneurysm and dissection, aortic regurgitation

Question 24

what congenital defect will you have from Prenatal lithium exposure

Answer 24

Ebstein anomaly

Question 25

what congenital defect will you have from Turner syndrome

Answer 25

Bicuspid aortic valve, COA

Question 26

what congenital defect will you have from Turner syndrome

Answer 26

Bicuspid aortic valve, COA

Question 27

what congenital defect will you have from Wlliams Syndrome

Answer 27

supravalvular aortic stenosis

Question 28

what congenital defect will you have from 22q11 syndromes

Answer 28

Truncus arteriosus, TOF

Question 29

hypertension

Answer 29

defined as persistent systolic BP > 140 mmHg and/or diastolic BP > 90 mmHg

Question 30

what are the risk factors of hypertension

Answer 30

increase age, obesity, diabetes, physical inactivity, excess salt intake, excess alcohol intake, family history; black> white>asian

Question 31

what are the risk factors of hypertension

Answer 31

increase age, obesity, diabetes, physical inactivity, excess salt intake, excess alcohol intake, family history; black> white>asian

Question 32

primary htn (essential) is related to an increase in

Answer 32

CO or TPR

Question 33

when is secondary HTN seen

Answer 33

renal/renovascular disease (fibromuscular dysplasia, usually found in younger women) and primary hyperaldosteronism

Question 34

hypertensive urgency

Answer 34

severe hypertension without acute end organ damage

Question 35

hypertensive urgency BP

Answer 35

>180/>120

Question 36

hypertensive emergency

Answer 36

severe ht. with end organ damage

Question 37

what are some examples of hypertensive emergency

Answer 37

stroke, encephalopathy, papilledema, MI, HF, aortic dissection, kidney injury, microangiopathic hemolytic anemia, eclampsia, retinal hemorrhages and exudates

Question 38

hypertension predisposes to

Answer 38

CAD, LVH, HF, afib, aortic aneurysm, stoke, chronic kidney disease, retinopathy

Question 39

isolated systolic hypertension

Answer 39

systolic pressure is increased while diastolic is not; after age 50, caused by age related decrease in the compliance of aorta and its proximal major branches (ex:aortic stiffening)

Question 40

isolated systolic hypertension

Answer 40

systolic pressure is increased while diastolic is not; after age 50, caused by age related decrease in the compliance of aorta and its proximal major branches (ex:aortic stiffening)

Question 41

what are signs of hyperlipidemia

Answer 41

xanthomas, tendinous xanthoma, corneal arcus

Question 42

xanthomas

Answer 42

plaques or nodules composed of lipid-laden histiocytes in skin

Question 43

where do you normally find xanthomas, and what is a name for it?

Answer 43

on the eyelids, xanthelasma

Question 44

tendinous xanthoma

Answer 44

lipid deposit in tendon

Question 45

where do you normally find a tendinous xanthoma

Answer 45

achilles

Question 46

corneal arcus

Answer 46

lipid deposits in cornea

Question 47

corneal acrus is most common in

Answer 47

common in elderly! (arcus senilis), but appears earlier in life in hypercholesterolemia

Question 48

arteriosclerosis

Answer 48

hardening of the arteries, with arterial wall thickening and loss of elasticity

Question 49

arteriolosclerosis

Answer 49

affects the small arteries and arterioles

Question 50

what are the two types of arteriolosclerosis

Answer 50

hyaline (thickening of vessel walls in essential hypertension or diabetes mellitus) hyperplastic (onion skinning in severe hypertension with proliferation of smooth muscle cells)

Question 51

hyaline arteriolosclerosis

Answer 51

(thickening of vessel walls in essential hypertension or diabetes mellitus)

Question 52

hyperplastic arteriolosclerosis

Answer 52

(onion skinning in severe hypertension with proliferation of smooth muscle cells)

Question 53

hyperplastic arteriolosclerosis

Answer 53

(onion skinning in severe hypertension with proliferation of smooth muscle cells)

Question 54

Monckeberg (medial calcific sclerosis)

Answer 54

affects medium sized arteries calcification of elastic lamina of arteries --> vascular stiffening without obstruction

Question 55

what do you see on an X-ray of someone with Monckeberg

Answer 55

pipestem appearance

Question 56

which is common, arteriolosclerosis or Monckeberg

Answer 56

arteriolosclerosis

Question 57

do you see an obstruction in Monckeberg?

Answer 57

no! does not obstruct blood flow, and the intimate is not involved

Question 58

Atherosclerosis

Answer 58

disease of elastic arteries (large) and large and medium sized muscular arteries caused by buildup of cholesterol plaques

Question 59

What are risk factors for atherosclerosis

Answer 59

modifiable: smoking, hypertension, hyperlipidemia, diabetes nonmodifiable: age, sex (increase in men and postmenopausal women), family hx

Question 60

what is important in the pathogenesis of atherosclerosis

Answer 60

inflammation

Question 61

what is the pathogenesis of atherosclerosis?

Answer 61

endothelial cell dysfunction --> macrophage and LDL accumulation --> foam cell formation -->fatty streaks --> smooth muscle cell migration (involves PDGF and FGF) -->proliferation, and extracellular matrix deposition --> fibrous plaque --> complex atheromas