Congenital Heart 2 Flashcards
genetic association for AV septal defect
downs syndrome
in AV septal defect, ____ fail to fuse resulting in no division of central AV canal
inferior and superior endocardial cushions
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
signs of infantile CHF
tachycardic
tachypnea
inability to feed well
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
why PDA is unhelpful in AV septal defect
would increase flow to lungs (shunting away from systemic(
4 features of Tetralogy of fallot due to anterior deviation of the coronal septum
subpulmonary stenosis
VSD
over riding aorta
RVH
associated genetic syndromes for tetralogy of fallot
DiGeorge
Downs
also Allagiles and CHARGE
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)
5 Cyanotic CHD
Tetralogy of fallot (most common cyanotic)
Truncus arteriosis
Transposition of great arteries
tricuspic atresia
total anamalous pulmoanry venous return
test to verify that CHD exists versus pulmonary cause of cyanosis
Hyperoxia test (should not be responsive to 100% O2)
murmur of TOF
velocity faster from extra volume shunting across VSD and pulmonary stenosis (systolic ejection at LUSB)
managemnet TOF
(side effects of treatment)
PGE to maintain PDA
operative repair
(apnea, fever, HTN, increased secretion, gastric outlet obstruction)
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)
Tet Spells
TOF
RVOT obstuction severe - little pulmonary flow
infant becomes more cyanotic with distress, hyperneic, irrabtable
managemnet, TET
calm child
“sqaut” to increase venous return
increase systemic resistance
oxygen admin
fluid admin
morphine to relax
palliation shunts for TOF
Waterston - ascending arota to right pulmonary
Potts - descending aorta to left pulmonary
operative complete repair TOF
- Patch closure of VSD (direct flow to aorta)
- Relief of RVOT obstruction via RVT patch placement to increase size of area OR placement of RV to Pulmonary artery conduit)
follow up concerns for complete repair TOF
pulmonary regurgitation
Trans pul patch > more regurg, larger RVEDV
patches must be replaced with growth
symptoms of RVR indicating need for re-surgery in TOF repair
Exercise intolerance
dysnea with mild effort,
syncope attributable to arrhythmia
degree of cyanosis in Truncus Arteriosis depends on
ratio of blood blow to lungs compared to body (Qp/Qs ratio)
degree of mixing
blood flow after birth Truncus arteriosis
to lungs - heart failure / pulmonary overcirculation
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
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)
genetic association Truncus arteriosis
Digeorge
aortic valve develops from ___
three sweelings of subendocardial mesenchyme
morphology effects of aortic valve obstruction
aortic root dilatation
leaflet destruction
ventricular hypertrophy
LV dysfunction in aortic stenosis is largely
diastolic (prolonging of systole reduces diastole)
pressure gradient in aortic stenosis
LV systolic pressure greater than aortic pressure (normally no difference)
acute repair, aortic stensosi
balloon valvuloplasty
surgical commissurotomy
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)
post birth aortic stenosis
moderate-mild have normal post-natal
severe dependent on PDA for systemic flow
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
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)
pathophysiology coarction
obstruction increases LV afterload > increased wall stress > compensatory LVH > CHF > Shock, metabolic acidosis, organ failure
IVC vs SVC O2 sat in arortic coarction
IVC is lower sat (LE extracting as much as they can from reduced flow)
epidemeology aortic coarction
4-8% of CHD
59% MAle
Turner XO or Noonan syndrome
each aortic arch is embedded in the
mesenchyme of pharyngeal arches
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)
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
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
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
surgical tx coarction
end to end anastomosis
prostetic patch (aneurysm)
subclavian flap arotoplasty
transcatheter stent
must remain open in hypoplastic left heart
Ductus arteriosis
ffoamen ovale (post birth o2 to body)
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)
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
Glenn and fontan procedures HLHS
detach SVC from heart and to pulmn A.
direct IVC to pulm artieres
(RV pumping blood to body)
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
treatment PDA
closure with indomethacin, suture ligation or placement of obstructing coil
seen more commonly with diabetic mothers
TGA
associated lesions TGA
VSD
LVOT obstruction
Coartion
coronary artery abnml
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
complications of Jatene TGA artery switch
cornary issues
aortic root dilatation
aortic insuffciency
branch pulmonary artery stenosis
crista terminalis, above and below VSD
above = outlet VSD
below = muscular (trabecular VSD)
complciationsVSD
CHF ssecondary to large volume L-R
LV dysfunction
Pulmonary HTN with eventual switch to R-L (eisenmengers syndrome)
bacterial endocarditis
VSD post op complicatoins
endocarditis
aortic regurg
tricuspid regurg
heart block
LV obstruction
