Pathology of the Heart II Flashcards

1
Q

congenital heart disease

A

between 3 and 8 weeks gestational age

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2
Q

most common cause of congenital defect

A

trisomy 21

if have this -work up heart

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3
Q

most common congenital cardiac malformation

A

bicuspid aortic valve - 2% of population

second - ventricular septal defect
third - atrial septal defect

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4
Q

atrial septal defect

A

first diagnosed as adults

  • no problems in childhood
  • pulmonary HTN in adults shows symptoms
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5
Q

ventricular septal defect

A

picked up in kids

-can close on its own

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6
Q

neural crest cells

A

form aorta and first branches

boundary - coarctation of aorta occurs here

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7
Q

atrial septal defect genotype

A

NKX2-5

sinus venosus type

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8
Q

holt-oram syndrome

A

TBX5 mutation

  • ASD, VSD, conduction defects
  • also limb and hand problems
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9
Q

digeorge syndrome

A

deletion 22q11.2 **
TBX1

CATCH-22

cardiac outflow tract defects

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10
Q

marfan syndrome

A

mutation in fibrillin

congenital heart disease
-aortic aneurysm, valve abnormalities

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11
Q

CATCH-22

A

digeorge

cardiac
abnormal facies
thymic aplasia
cleft palate
hypocalcemia
chromosome 22 deletion
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12
Q

cardiac defects from neural crest

A

5th brachial arch

3rd and 4th pharyngeal pouches

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13
Q

atresia

A

complete obstruction

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14
Q

left to right shunt

A

cause pulmonary volume overload

compensation - hypertrophy and vasc resistance

results in right to left shunt - eisenmenger syndrome

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15
Q

right to left shunt

A

hypoxia and tissue cyanosis
-bypass lungs

allow paradoxical emboli

finger and toe clubbing

  • hypertrophic osteoarthropathy
  • also polycytemia
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16
Q

causes of L > R shunts

A

ASD, VSD, PDA, AVSD
-baby pink usually

get pulmonary HTN - reversal (eisenmenger)
-reversal - needs transplant

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17
Q

eisenmenger

A

late cyanotic congenital heart disease

-with reversal of L > R shunt

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18
Q

VSD

A

ventricular septal defect

P hypertrophy RV
V hypertrophy LV

majority - membranous septum (upper)
minority - muscular septum (lower)

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19
Q

ASD

A

atrial septal defect

3 major types

  • secundum (majority)
  • primum
  • sinus venosus

may be asymptomatic
-10% > pulmonary HTN

may have murmur

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20
Q

secundum ASD

A

majority

-involve fossa ovalis

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21
Q

primum ASD

A

adjacent to AV valve

22
Q

sinus venosus ASD

A

near superior vena cava

23
Q

PDA

A

patent ductus arterious

communication of aorta and pulmonary artery

usually closes:

  • increased O2
  • decreased pulmonary resistance
  • decreased prostaglandin E2

continuous harsh machine like murmur

24
Q

machine like murmur

A

patent ductus arteriosus

25
Q

Tx for PDA

A

NSAID - decreased prostaglandin E2

first - look for other defects
-second - fix this

26
Q

AVSD

A

atrioventricular septal defects

complete - all four chambers communicate

1/3 have down syndrome

27
Q

majority of congenital heart disease in children with down syndrome

A

AVSD

40%

then VSD > ASD > PDA > tetralogy of fallot

28
Q

right to left shunt causes

A

cyanotic congenital heat disease

get clubbing of tips of fingers and toes - hypertrophic osteoarthropathy
-polycythemia due to hypoxia
also can have paradoxical emboli

BLUE BABY

29
Q

tetralogy of fallot

A

1 - VSD
2 - subpulmonic valve stenosis with obstruction of right ventricular outflow tract
3 - overridding aorta
4 - right ventricular hypertrophy

boot shaped heart

degree of stenosis gives degree of blue baby

30
Q

boot shaped heart

A

tetralogy of fallot

31
Q

overriding aorta

A

in tetralogy of fallot

-due to ability to look through VSD and see aorta instead of LV wall

32
Q

pink tetralogy of fallot

A

mild subpulmonic stenosis

-lung perfused

33
Q

transposition of great arteries

A

aorta and pulmonary artery switched
-separate circulations

only survive with VSD, PDA, or patent foramen ovale

to stop closure - infuse prostaglandin E and perform atrial septostomy

Tx - surgery

34
Q

infantile coarctation of aorta

A
preductal
with PDA
-infant - blue bottom, pink top
-deox blood from pulmonary a to aorta
-pressure difference
35
Q

adult coarctation of aorta

A

postductal
junction where ligamentum arteriosum is
-no color difference
-do see pressure difference

36
Q

response to coarctation of aorta

A

dilated ascending aorta and major branches (proximal to coarctation)

collateral ciculations form - intercostal notching

also hear murmurs - pansystolic

adult form - can get renal HTN

37
Q

pulmonic valvar stenosis

A

RVH and pulmonary a dilation

38
Q

pulmonic subvalvar stenosis

A

RVH and NO pulmonary a dilation

39
Q

aortic valvar stenosis

A

infants only survive with PDA

left ventricular underdevelopment
-hypoplastic left heart syndrome

40
Q

subaortic stenosis

A

infective endocarditis, LVH, post-stenotic dilation

-sudden death

41
Q

supravalvar aortic stenosis

A

aortic dysplasia - thick wall

  • chromosome 7 deletion - williams beuren syndrome
  • possible cause
42
Q

IHD

A

ischemic heart disease

imbalance between supply and demand for heart O2

majority - atherosclerotic coronary aa - 90%

43
Q

leading cause of death in US for both M and F

A

ischemic heart disease

higher males

premenopausal women - uncommon

44
Q

75% obstruction

A

symptoms with exercise

45
Q

90% obstruction

A

ischemia at rest

46
Q

causes of IHD

A

atherosclerotic coronaries
thrombosis over disrupted plaque
local platelet aggregation
vasospasm

47
Q

acute coronary syndrome

A

abrupt plaque change followed by thrombosis

large core and thin cap (young plaques)

48
Q

plaque rupture

A

2/3 narrowed less than 50%

85% narrowed less than 70%

tend to involve entire RCA and proximal LAD / LCX

49
Q

unstable angina

A

plaque is changing

50
Q

transmural MI

A

full wall thickness

51
Q

stable angina

A

stenosis >75%

no change in plaque

52
Q

sudden death

A

often small platelet aggregates or thrombi and/or thromboemboli