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Congenital Heart 2 Flashcards

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1
Q

genetic association for AV septal defect

A

downs syndrome

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2
Q

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

A

inferior and superior endocardial cushions

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3
Q

features of all AV septal defects

A

AV valves insert at level of cardiac crux

unwedged anterior displacement of aorta

elongated LVOT

Cleft i AV valve

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4
Q

signs of infantile CHF

A

tachycardic

tachypnea

inability to feed well

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5
Q

qwhy later CHF in AV septal defect?

A

no symptoms until lungs open and body must increase output

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

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6
Q

why PDA is unhelpful in AV septal defect

A

would increase flow to lungs (shunting away from systemic(

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7
Q

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

A

subpulmonary stenosis

VSD

over riding aorta

RVH

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8
Q

associated genetic syndromes for tetralogy of fallot

A

DiGeorge

Downs

also Allagiles and CHARGE

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9
Q

infancy presentation TOF

A

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)

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10
Q

5 Cyanotic CHD

A

Tetralogy of fallot (most common cyanotic)

Truncus arteriosis

Transposition of great arteries

tricuspic atresia

total anamalous pulmoanry venous return

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11
Q

test to verify that CHD exists versus pulmonary cause of cyanosis

A

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

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12
Q

murmur of TOF

A

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

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13
Q

managemnet TOF

(side effects of treatment)

A

PGE to maintain PDA

operative repair

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

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14
Q

Tet spells of TOF recognized via

A

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

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15
Q

Tet Spells

A

TOF

RVOT obstuction severe - little pulmonary flow

infant becomes more cyanotic with distress, hyperneic, irrabtable

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16
Q

managemnet, TET

A

calm child

“sqaut” to increase venous return

increase systemic resistance

oxygen admin

fluid admin

morphine to relax

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17
Q

palliation shunts for TOF

A

Waterston - ascending arota to right pulmonary

Potts - descending aorta to left pulmonary

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18
Q

operative complete repair TOF

A
  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)
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19
Q

follow up concerns for complete repair TOF

A

pulmonary regurgitation

Trans pul patch > more regurg, larger RVEDV

patches must be replaced with growth

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20
Q

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

A

Exercise intolerance

dysnea with mild effort,

syncope attributable to arrhythmia

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21
Q

degree of cyanosis in Truncus Arteriosis depends on

A

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

degree of mixing

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22
Q

blood flow after birth Truncus arteriosis

A

to lungs - heart failure / pulmonary overcirculation

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23
Q

clinical presentation Truncus arteriosis

Study These Flashcards
A

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
Q

Rare clinical presentation Truncus Arteriosis

Study These Flashcards
A

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

Cardiovascular (39 decks)

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