Pathology of the Heart II

Question 1

congenital heart disease

Answer 1

between 3 and 8 weeks gestational age

Question 2

most common cause of congenital defect

Answer 2

trisomy 21

if have this -work up heart

Question 3

most common congenital cardiac malformation

Answer 3

bicuspid aortic valve - 2% of population

second - ventricular septal defect
third - atrial septal defect

Question 4

atrial septal defect

Answer 4

first diagnosed as adults

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

Question 5

ventricular septal defect

Answer 5

picked up in kids

-can close on its own

Question 6

neural crest cells

Answer 6

form aorta and first branches

boundary - coarctation of aorta occurs here

Question 7

atrial septal defect genotype

Answer 7

NKX2-5

sinus venosus type

Question 8

holt-oram syndrome

Answer 8

TBX5 mutation

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

Question 9

digeorge syndrome

Answer 9

deletion 22q11.2 **
TBX1

CATCH-22

cardiac outflow tract defects

Question 10

marfan syndrome

Answer 10

mutation in fibrillin

congenital heart disease
-aortic aneurysm, valve abnormalities

Question 11

CATCH-22

Answer 11

digeorge

cardiac
abnormal facies
thymic aplasia
cleft palate
hypocalcemia
chromosome 22 deletion

Question 12

cardiac defects from neural crest

Answer 12

5th brachial arch

3rd and 4th pharyngeal pouches

Question 13

atresia

Answer 13

complete obstruction

Question 14

left to right shunt

Answer 14

cause pulmonary volume overload

compensation - hypertrophy and vasc resistance

results in right to left shunt - eisenmenger syndrome

Question 15

right to left shunt

Answer 15

hypoxia and tissue cyanosis
-bypass lungs

allow paradoxical emboli

finger and toe clubbing

• hypertrophic osteoarthropathy
• also polycytemia

Question 16

causes of L > R shunts

Answer 16

ASD, VSD, PDA, AVSD
-baby pink usually

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

Question 17

eisenmenger

Answer 17

late cyanotic congenital heart disease

-with reversal of L > R shunt

Question 18

VSD

Answer 18

ventricular septal defect

P hypertrophy RV
V hypertrophy LV

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

Question 19

ASD

Answer 19

atrial septal defect

3 major types

• secundum (majority)
• primum
• sinus venosus

may be asymptomatic
-10% > pulmonary HTN

may have murmur

Question 20

secundum ASD

Answer 20

majority

-involve fossa ovalis

Question 21

primum ASD

Answer 21

adjacent to AV valve

Question 22

sinus venosus ASD

Answer 22

near superior vena cava

Question 23

PDA

Answer 23

patent ductus arterious

communication of aorta and pulmonary artery

usually closes:

• increased O2
• decreased pulmonary resistance
• decreased prostaglandin E2

continuous harsh machine like murmur

Question 24

machine like murmur

Answer 24

patent ductus arteriosus

Question 25

Tx for PDA

Answer 25

NSAID - decreased prostaglandin E2 first - look for other defects -second - fix this

Question 26

AVSD

Answer 26

atrioventricular septal defects complete - all four chambers communicate 1/3 have down syndrome

Question 27

majority of congenital heart disease in children with down syndrome

Answer 27

AVSD 40% then VSD > ASD > PDA > tetralogy of fallot

Question 28

right to left shunt causes

Answer 28

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

Question 29

tetralogy of fallot

Answer 29

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

Question 30

boot shaped heart

Answer 30

tetralogy of fallot

Question 31

overriding aorta

Answer 31

in tetralogy of fallot | -due to ability to look through VSD and see aorta instead of LV wall

Question 32

pink tetralogy of fallot

Answer 32

mild subpulmonic stenosis | -lung perfused

Question 33

transposition of great arteries

Answer 33

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

Question 34

infantile coarctation of aorta

Answer 34

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

Question 35

adult coarctation of aorta

Answer 35

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

Question 36

response to coarctation of aorta

Answer 36

dilated ascending aorta and major branches (proximal to coarctation) collateral ciculations form - intercostal notching also hear murmurs - pansystolic adult form - can get renal HTN

Question 37

pulmonic valvar stenosis

Answer 37

RVH and pulmonary a dilation

Question 38

pulmonic subvalvar stenosis

Answer 38

RVH and NO pulmonary a dilation

Question 39

aortic valvar stenosis

Answer 39

infants only survive with PDA left ventricular underdevelopment -hypoplastic left heart syndrome

Question 40

subaortic stenosis

Answer 40

infective endocarditis, LVH, post-stenotic dilation | -sudden death

Question 41

supravalvar aortic stenosis

Answer 41

aortic dysplasia - thick wall - chromosome 7 deletion - williams beuren syndrome - possible cause

Question 42

IHD

Answer 42

ischemic heart disease imbalance between supply and demand for heart O2 majority - atherosclerotic coronary aa - 90%

Question 43

leading cause of death in US for both M and F

Answer 43

ischemic heart disease higher males premenopausal women - uncommon

Question 44

75% obstruction

Answer 44

symptoms with exercise

Question 45

90% obstruction

Answer 45

ischemia at rest

Question 46

causes of IHD

Answer 46

atherosclerotic coronaries thrombosis over disrupted plaque local platelet aggregation vasospasm

Question 47

acute coronary syndrome

Answer 47

abrupt plaque change followed by thrombosis large core and thin cap (young plaques)

Question 48

plaque rupture

Answer 48

2/3 narrowed less than 50% 85% narrowed less than 70% tend to involve entire RCA and proximal LAD / LCX

Question 49

unstable angina

Answer 49

plaque is changing

Question 50

transmural MI

Answer 50

full wall thickness

Question 51

stable angina

Answer 51

stenosis >75% no change in plaque

Question 52

sudden death

Answer 52

often small platelet aggregates or thrombi and/or thromboemboli