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Flashcards in Congenital Heart 2 Deck (56):
1

genetic association for AV septal defect 

downs syndrome

2

in AV septal defect, ____ fail to fuse resulting in no division of central AV canal

inferior and superior endocardial cushions

3

features of all AV septal defects

AV valves insert at level of cardiac crux

unwedged anterior displacement of aorta

elongated LVOT

Cleft i AV valve

4

signs of infantile CHF

tachycardic

tachypnea

inability to feed well

5

qwhy later CHF in AV septal defect?

no symptoms until lungs open and body must increase output

 

as lungs open up, receive increasing flow as body tries to compensate

6

why PDA is unhelpful in AV septal defect

would increase flow to lungs (shunting away from systemic(

7

4 features of Tetralogy of fallot due to anterior deviation of the coronal septum

subpulmonary stenosis

VSD

over riding aorta

RVH

8

associated genetic syndromes for tetralogy of fallot

DiGeorge 

Downs

also Allagiles and CHARGE

9

infancy presentation TOF

Cyanotic (or pink if RVOT obstruction is minimal)

systolic ejection murmur at LUSB

Tachypnea (esp when RVOT obstruction minimal > pulmonary overcirculation from shunting across VSD)

10

5 Cyanotic CHD

Tetralogy of fallot (most common cyanotic)

Truncus arteriosis

Transposition of great arteries

tricuspic atresia

total anamalous pulmoanry venous return

11

test to verify that CHD exists versus pulmonary cause of cyanosis

Hyperoxia test (should not be responsive to 100% O2)

12

murmur of TOF

velocity faster from extra volume shunting across VSD and pulmonary stenosis (systolic ejection at LUSB)

13

managemnet TOF

(side effects of treatment)

PGE to maintain PDA

operative repair

(apnea, fever, HTN, increased secretion, gastric outlet obstruction)

14

Tet spells of TOF recognized via

disappearance of systolic ejection murmur (RVOT obstruction so makred that little flow to pulmonary system, leading to L-R shunt)

15

Tet Spells

TOF

RVOT obstuction severe - little pulmonary flow

infant becomes more cyanotic with distress, hyperneic, irrabtable

16

managemnet, TET

calm child

"sqaut" to increase venous return

increase systemic resistance

oxygen admin

fluid admin

morphine to relax

 

17

palliation shunts for TOF

Waterston - ascending arota to right pulmonary

Potts - descending aorta  to left pulmonary 

18

operative complete repair TOF

1. Patch closure of VSD (direct flow to aorta)

2. Relief of RVOT obstruction via RVT patch placement to increase size of area OR placement of RV to Pulmonary artery conduit)

19

follow up concerns for complete repair TOF

pulmonary regurgitation 

Trans pul patch > more regurg, larger RVEDV

patches must be replaced with growth

20

symptoms of RVR indicating need for re-surgery in TOF repair

Exercise intolerance

dysnea with mild effort,

syncope attributable to arrhythmia

21

degree of cyanosis in Truncus Arteriosis depends on

ratio of blood blow to lungs compared to body (Qp/Qs ratio)

 

degree of mixing

22

blood flow after birth Truncus arteriosis

to lungs - heart failure / pulmonary overcirculation

23

clinical presentation Truncus arteriosis

cyanosis

widened pulse press (lungs stealing blood)

hyperdynamic precordium

normal S1 with ejection click, single S2

Loud pansystolic murmur at LLSB

Systolic murmur at USB from truncal stenosis (diastolic if insuf)

tachypnea, hepatomegaly, poor feeding, diaphoresis

24

Rare clinical presentation Truncus Arteriosis

decreased Pulm flow and no regurg

(due to PA stenosis or pulmnonary vascular disease)

moderate to severe canosis

normal pulses and pulse pressure

Loud S2

Systolic murmur (stenotic PAs)

25

genetic association Truncus arteriosis

Digeorge

26

aortic valve develops from ___

three sweelings of subendocardial mesenchyme

27

morphology effects of aortic valve obstruction

aortic root dilatation

leaflet destruction

ventricular hypertrophy

28

LV dysfunction in aortic stenosis is largely

diastolic (prolonging of systole reduces diastole)

29

pressure gradient in aortic stenosis

LV systolic pressure greater than aortic pressure (normally no difference)

30

acute repair, aortic stensosi

balloon valvuloplasty

surgical commissurotomy

31

fetal circulation impact of aortic stenosis

moderate no effect

severe - high LVEDP reduces flow from umbilical vein itno LV and into the AAO, 

high O2 blood from umbilicus directed through RV, mixing with SVC (low O) > ductus arterioussis then into Descending Aorta and retrograde into AA (results lower O2 supply to brain)

32

post birth aortic stenosis

moderate-mild have normal post-natal 

 

severe dependent on PDA for systemic flow

33

formation of atrial septum

septum primum meets endocardial cushions below

secundum grows down, overlap is pressure sensitive

(high pressure pushes through to R>L shunt)

As lugns open, RA pressure drops, LA pressure pushes shunt close

34

failure of atrial secundum into adulthood closure results in

Left > right shunting

R sided dilatation

CHF
no hypertrophy 

fixed splitting of S2 (always have increased preload)

35

pathophysiology coarction

obstruction increases LV afterload > increased wall stress > compensatory LVH > CHF > Shock, metabolic acidosis, organ failure

36

IVC vs SVC O2 sat in arortic coarction

IVC is lower sat (LE extracting as much as they can from reduced flow)

37

epidemeology aortic coarction

4-8% of CHD

59% MAle

Turner XO or Noonan syndrome

38

each aortic arch is embedded in the 

mesenchyme of pharyngeal arches

39

fates of aortic arches

1 - maxillary artery

2- stapedial and hyoid artery

3 - common cortid, part of  internal coratid, external coratid

4 - R = subclavian, L = arch between LCC and L SubClav

6 Right= pulmonaryA, Left = PDA

(dorsal arch = R side regresses, L = descending aorta)

40

clincial presentation coarction

infant with CHF - LV systolic dysfunction and CHF (low SV, high LVEDP, high LA pressure, acidosis, reduced contractility)

3 child/teen with arterial systolic HTN

4 child with murmur

41

physical exam coarction

pale, iratable, resp distresss, diferential cyanosis if R-L shunt

LE pulses delayed with reduced amplitude

heaving LV at apex, sustolic thrill pos.

constant systollic ejection click (if bicuspid aorta)

SEM at LUSB, collaterals giving constitent murmurs

gallow rhythm if CHF

42

CXR coarction

infant with CHF - Pul vascular congestion

Older child:

prominent aortic nob

3 sign indent at left border desc. aorta

reverse 3 sign on esophogus (barium swallow)

rib notching

43

surgical tx coarction

end to end anastomosis

prostetic patch (aneurysm)

subclavian  flap arotoplasty

transcatheter stent 

44

must remain open in hypoplastic left heart

Ductus arteriosis 

ffoamen ovale (post birth o2 to body)

45

presentation HLHS

abnormal ultrasound

soft SEM at LUSB,

often single, prominent heart sounds

mild hypoxia (SaO2 very low if atrial septum intact)

OR Cardiogenic shock (if PDA fully closed)

(pain with feed, poor perfusion, decreased urine, lethargy)

46

norwood procedure HLHS

attach pulmonary arteries to heart

patch to direct blood from RV to aorta

Shunt RV to pulmonary arteries

cut out atrial septum to allow blood to leave pulmonary system from LA

47

Glenn and fontan procedures HLHS

detach SVC from heart and to pulmn A. 

 

direct IVC to pulm artieres 

 

(RV pumping blood to body)

48

pathophysiology / process PDA

pulmonary resistance drops at birth> blood flows from aorta to pulmonary through PDA > oxygenated blood to lungs instead of body > heart failure and poor growth 

49

treatment PDA

closure with indomethacin, suture ligation or placement of obstructing coil

50

seen more commonly with diabetic mothers

TGA

51

associated lesions TGA

VSD

LVOT obstruction

Coartion

coronary artery abnml

52

presentation TGA

cyanosis

murmur absent

loud single S2 (due to anterior aorta) 

hyperoxia test (no change with 100% O2)

egg in a string CXR, mild cardiomegaly

53

complications of Jatene TGA artery switch

cornary issues

aortic root dilatation

aortic insuffciency

branch pulmonary artery stenosis

54

crista terminalis, above and below VSD

above = outlet VSD

below = muscular (trabecular VSD)

55

complciationsVSD

CHF ssecondary to large volume L-R

LV dysfunction

Pulmonary HTN with eventual switch to R-L (eisenmengers syndrome)

bacterial endocarditis

56

VSD post op complicatoins

endocarditis

aortic regurg

tricuspid regurg

heart block

LV obstruction