Congenital Heart Defects (Exam 1)

Question 1

-bypasses the liver
-shunts oxygenated blood from the placental vein to the IVC and then the RA

Answer 1

ductus venosus

Question 2

-bypasses the lungs
-shunts oxygenated blood from the RA to the LA

Answer 2

foramen ovale

Question 3

-bypasses the lungs
-shunts oxygenated blood from the pulmonary artery to the aorta

Answer 3

ductus arteriosus

Question 4

What are the 3 fetal shunts?

Answer 4

1) ductus venosus
2) foramen ovale
3) ductus arteriosus

Question 5

The 3 fetal shunts all close shortly after birth due to

Answer 5

pressure changes

Question 6

50% of children will have innocent (functional or physiologic) heart murmurs. What are the red flags?

Answer 6

murmur+ cyanosis or decreased perfusion

Question 7

What are the 3 types of congenital heart defects?

Answer 7

1) non-cyanotic/ acyanotic
2) cyanotic
3) obstructive

Question 8

Which congenital heart defect type is this?
-left to right shunt

Answer 8

non-cyanotic defect

Question 9

What are the 3 non-cyanotic defects?

Answer 9

1) patent ductus arteriosus (PDA)
2) atrial septal defect (ASD)
3) ventricular septal defect (VSD)

Question 10

What is the most common non-cyanotic defect?

Answer 10

ventricular septal defect (VSD)

Question 11

Which congenital heart defect type is this?
-right to left shunt

Answer 11

cyanotic defect

Question 12

What are the 5 cyanotic defects?

Answer 12

1) tetralogy of fallot
2) transposition of the great vessels
3) hypoplastic left heart
4) pulmonary atresia
5) truncus arteriosus

Question 13

What is the 2 obstructive defects?

Answer 13

1) coarctation of the aorta
2) subclavian steel syndrome

Question 14

What does TORCH stand for?

Answer 14

T= toxoplasmosis (parasite found in undercooked meat and cat feces)
O= other (syphilis, listeria, varicella, parocovirus B19/5th disease)
R= rubella/german measles
C= CMV
H= herpes simplex

Question 15

-autosomal dominant disorder
-generally tall and thin with an arm span that exceeds height
-cardiovascular defects like mitral valve prolapse, tricuspid prolapse or atrial septal defect are common

Answer 15

marfan’s syndrome

Question 16

-trisomy 21
-congenital heart defects like valvular malformations, atrial septal defect, or ventricular septal defect

Answer 16

down syndrome

Question 17

Ductus arteriosis in utero provides a connection between the ________________ and the ____________. This allows fetal circulation to bypass the lungs since the blood is not oxygenated in this way

Answer 17

pulmonary artery, aorta

Question 18

Closure of the ductus arteriosus creates the

Answer 18

ligamentum arteriosum

Question 19

When does ductus arteriosus close?

Answer 19

by day 4 after birth

Question 20

Patency of ductus arteriosus in utero is maintained by ___________________ secreted by the placenta

Answer 20

prostaglandins

note: if closure does not occur, NSAIDs like indomethacin will be given to decrease prostaglandins and promote closure, or if patency is needed due to other defects, then misoprotol is given to prevent closure

Question 21

After birth, a _______ will result in blood shunting from the aorta (higher pressure to the pulmonary artery (lower pressure), returning the blood to the pulmonary circulation

Answer 21

patent ductus arteriosus (PDA)

Question 22

A continuous machine like murmur will be heard at the left 2nd intercostal space. This murmur is continuous due to the blood flow through the ductus is constant and not related to the cardiac cycle

Answer 22

patent ductus arteriosus (PDA)

Question 23

Uncorrected ___________ will result in increased pulmonary blood flow, increased pulmonary arterial pressure (pulmonary HTN), increased workload on right side of heart (high afterload), and right sided heart failure over time. Increased workload on the left heart pump will lead to left sided heart failure over time

Answer 23

patent ductus arteriosus (PDA)

Question 24

Chronic pulmonary hypertension will cause a shunt reversal and cyanosis will occur called

Answer 24

Eisenmenger’s syndrome

Question 25

When a non-cyanotic defect becomes cyanotic AFTER adaptation leads to reversal with pressure gradient is called

Answer 25

Eisenmenger's syndrome

Question 26

-a hole in the interatrial septum causing blood to shunt from the LA to RA -if theres a large defect, there will be overloads of pulmonary circulation which overworks the right side of heart and causes RV hypertrophy -when pulmonary HTN occurs it can lead to shunt reversal and cyanosis= Eisenmenger's syndrome -murmur is due to volume overload on the right side of heart which causes an increased amount of blood flow over the valves (turbulent like a bruit)

Answer 26

atrial septal defect (ASD)

Question 27

-a hole in the interventricular septum results in the shunting of blood from LV to RV -the L to R shunt will increase blood volume to the pulmonary system and result in pulmonary HTN and RV hypertrophy -over time if it is not repaired then pressure changes will cause shunt to reverse causing deoxygenated blood to enter systemic circulation and result in cyanosis= Eisenmenger's syndrome -systolic murmur

Answer 27

ventricular septal defect (VSD)

Question 28

T/F: normally, the LV is a higher pressure system compared to RV

Answer 28

true

Question 29

What is another name for tetralogy of fallot?

Answer 29

blue baby syndrome

Question 30

What is the most common cause of cyanosis after infancy?

Answer 30

tetralogy of fallot

Question 31

Which congenital heart defect is associated with delections on chromosome 22 (DiGeorge's syndrome) and down's syndrome?

Answer 31

tetralogy of fallot

Question 32

What are the 4 pathologies with tetralogy of fallot? memory cue: you must "PROV" it

Answer 32

P= pulmonary stenosis R= RV hypertrophy O= overriding aorta (aorta lies between the R/L ventricles over the ventricular septal defect, so systemic circulation is a mix of oxygenated and deoxygenated blood) V= ventricular septal defect

Question 33

What congenital defect is known to have chronic cyanosis, tet spells, fingernail clubbing, and a boot shaped heart on xray?

Answer 33

tetralogy of fallot

Question 34

What are tet spells?

Answer 34

-crying, eating, exertion, etc increases the demand for systemic blood flow-> vasodilation, decreased peripheral vascular resistance and decreased LV pressure-> increased shunting of blood from the R to L = cyanosis -the child will innately adapt to the cyanotic episode by squatting to increase blood return, increase peripheral vascular resistance in the arterial system and increase LV pressure, which reverses shunt L to R and the cyanosis will result

Question 35

-the aorta comes off the RV and the pulmonary artery comes off the LV -aorta delivers oxygen poor blood to the body from the right side of the heart -pulmonary artery recycles oxygen rich blood to the lungs from the left side of the heart -results in 2 parallel closed circuits that do not communicate -incompatible with post natal life unless ASD, VSD, or PDA is present (PDA is maintained with prostaglandins, allows a connection between the aorta and pulmonary artery)

Answer 35

transposition of the great vessels

Question 36

What congenital heart defect is known to have nail clubbing, heaving of the chest wall, and an egg shaped heart with narrow mediastinum (egg on string appearance)?

Answer 36

transposition of the great vessels

Question 37

-arteries exiting the heart fail to separate into the aorta and pulmonary artery -only one artery exits the heart to send blood to the lungs, heart muscle, and systemic circulation -results in too much blood going into the lungs as well as an increased workload on the heart -a ventricular septal defect is always present and allows for mixing of oxygen rich and oxygen poor blood -bounding heart

Answer 37

truncus arteriosus

Question 38

atresia=

Answer 38

absence of or abnormal development

Question 39

-no blood flow from RV to the lungs = no oxygenation of blood -severe right sided heart failure -medical emergency

Answer 39

pulmonary atresia

Question 40

What is the aka for hypoplastic left heart syndrome?

Answer 40

too small left heart

Question 41

-multiple structures on the left side of heart are underdeveloped or not formed: mitral valve, LV, aortic valve, ascending aorta -an ASD is common with this -in the first few days, PDA and ASD allow for adaptation

Answer 41

hypoplastic left heart syndrome

Question 42

Congenital narrowing of the aorta is called

Answer 42

coarctation of aorta

Question 43

Congenital narrowing of aorta causes a high afterload resulting in: -high LV and systolic volume -decreased cardiac output (pulmonary congestion) -hypotension -LV hypertrophy -left sided heart failure -decreased perfusion of the abdominal organs and lower limbs -leg claudication -systolic murmur when the blood is being pushed through narrowing -upper limb BP is > lower limb BP -cold lower extremities -rib notching appearance on xray

Answer 43

coarctation of aorta

Question 44

What are the 2 types of coarctation of aorta?

Answer 44

1) pre ductal 2) post ductal

Question 45

Which coarctation of aorta type is this? -narrowing occurs proximal to the ductus arteriosus -R to L shunt (pulmonary artery to aorta) -results in cyanosis of the lower half of body

Answer 45

pre ductal

Question 46

Which coarctation of aorta type is this? -narrowing occurs distal to the ductus arteriosus -L to R shunt (aorta to pulmonary artery) -results in pulmonary HTN and congestive heart failure

Answer 46

post ductal

Question 47

-stenosis of the subclavian artery proximal to the origin of the vertebral artery -results in retrograde blood flow in the vertebral artery on the affected side -arm claudication -vertebrobasilar insufficiency -causes: congenital cervical rib, Takayasu's arteritis, atherosclerosis

Answer 47

subclavian steel syndrome