Congenital Heart Disease

Question 1

list the R to L shunt CHDzs

Answer 1

persistent truncus arteriosus
transposition of the great vessels
tricuspid atresia
tetralogy of fallot
Total anomalous pulmonary venous return

Question 2

what genetic dz predisposes to persistent truncus arteriosus?

Answer 2

22q11

Question 3

what dz predisposes to transposition of the great vessels

Answer 3

diabetes in mom

Question 4

what dz predisposes to tetraology of fallot

Answer 4

fetal alcohol syndrome

22q11

Question 5

22q11 congential heart defects?

Answer 5

persistent truncus arteriosus

tetralogy of fallot

Question 6

what dz predisposes to VSD

Answer 6

cri du chat
fetal OH syndrome
Downs

Question 7

what dz predisposes to ASD

Answer 7

fetal OH syndrome

downs

Question 8

what dz predisposes to PDA

Answer 8

congenital rubella
prematurity
fetal alcohol syndrome

Question 9

what dz predisposes to coarcation of the aorta and bicuspid aortic valve?

Answer 9

turneres

Question 10

describe persistent truncus arteriosus

Answer 10

truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticaopulmonary septum formation
most have a VSD accompanying it

Question 11

describe transposition fo the great vessels

Answer 11

due to failure of the aorticopulmonary septum to spiral =
aorta leaves RV anteriorally and pulmonary trunk leaves LV posteriorly. atria are normal
= systemic and pulmonary circulations are separate
requires a shunt - VSD, PDA or patent foramen ovale to be compatible with life

Question 12

describe tricuspid atresia

Answer 12

absence of tricuspid valve – hypoplastic RV

needs BOTH ASD and VSD to be viable

Question 13

Describe tetraology of fallot

Answer 13

caused by anterosuperior displacement of the infundibular septum
MOST COMMON CAUSE OF EARLY CHILDHOOD CYANOSIS
1) pulmonary infundibular stenosis - prognosis determinant
2) right ventricular hypertrophy - BOOT SHAPED heart
3) overriding aorta
4) VSD (persistent truncus arteriosus also has this commonly)

Question 14

boot shaped heart on CXR

Answer 14

tetraology of fallot

Question 15

what improves the cyanosis in Tetralogy of Fallot

Answer 15

squatting - increases systemic venous return - decreases right to left shunt??? what the heck?

Question 16

describe the SaO2 throughout the chambers of the heart in tetralogy of fallot

Answer 16

decreased SaO2 in the left ventricle and aorta

Question 17

describe total anomalous pulmonary venous return

Answer 17

pulmonary veins drain into right heart circulation
associated with an ASD (persistent truncus arteriosus and tetralogy of fallot have VSD often) and sometimes a PDA t0 maintain r to l shunt to maintain CO (transposition needs a VSD, ASD or PDA and tricuspid atresia needs a VSD and ASD)

Question 18

describe the frequency relationships between the congenital heart defects that cause left to right shunts

Answer 18

VSD>ASD>PDA

Question 19

describe the SaO2 in the chambers of the heart in a VSD compared to the regular heart

Answer 19

increased in RV and PA

Question 20

VSD occur where?

Answer 20

required in transposition of great vessels and with ASD in tricuspid atresia
commonly in persistent truncus arteriosus and tetralogy of fallot
cri du chat
fetal OH syndrome
down syndrome (membranous)

Question 21

what sort of MI can cause a VSD

Answer 21

anterior wall LDA

Question 22

what do you hear with an ASD

Answer 22

loud S1 wide fixed split S2
ostium secundum defects most common.
ostium primum more rare but usually occur with other heart defects
NOT patent foarmen ovalve - unfused, ASD 0 missing

Question 23

what conditions are associated with ASD?

Answer 23

down - primum
fetal alcohol syndrome
often with total anomalous pulmonary venous return
needed for surivival if no vsd, pda in transposition of great vessels

Question 24

describe SaO2 changes in ASD

Answer 24

increased SaO2 in RA, RV, PA

Question 25

what can come of a PDA

Answer 25

differential cyanosis when lower limbs are cyanotic

Question 26

what closes and opens a PDA

Answer 26

low O2 and PGs keep open

indomethacin and high O2 close

Question 27

what conditions are associated with PDA?

Answer 27

may require in transposition of great vessels
congenital rubella (mom - rash, lymphadenopathy, arthtritis; baby - maybe blueberry muffin rash, PDA/pul artery hypoplasia, cataracts, deafness)
respiratory distress syndrometetralogy of fallot

Question 28

what is Eisenmenger syndrome?

Answer 28

due to an uncorrected left to right shunt. – increased pulmonary blood flow – pathologic remodeling of vasculature – pulmonary arterial hypertension – RVH to compensate – shunt becomes right to left

Question 29

CXPX of eisnemengers syndrome

Answer 29

a) late cyanosis
b( clubbing
c( polycythemia

Question 30

what is coarctation fo the aorta associated with?

Answer 30

bicuspid aortic valve,

turner

Question 31

presentation of coarctation of the aorta

Answer 31

hypertension in upper extremities
weak delaye dpulse in lower extremities (brachial-femoral delay)
collateral arteris erode ribs

Question 32

OH exposure in utero

Answer 32

VSD
PDA
ASD
tetraology of fallot

Question 33

congenital rubella

Answer 33

septal defects
PDA
pulmonary artery stenosis

Question 34

down syndrome

Answer 34

Av septal defect - endocardial cushin defect
VSD
ASD

Question 35

diabetic mother

Answer 35

transposition of great vessels

Question 36

margan syndrome

Answer 36

MVP
thoracic aortic aneurysm and dissection
aortic regurgitation

Question 37

prenatal lithium exposure

Answer 37

ebstein anomaly

Question 38

turner syndrome

Answer 38

bicuspid aortic valve

coarctation of the aorta

Question 39

Williams syndrome

Answer 39

supravlvular aortic stenosis

Question 40

22q11

Answer 40

truncus arteriosus

tetralogy of fallot