Congenital Heart Defects Flashcards
hyperplastic left heart syndrome
aortc atresia with intact VS
leads to hypoplastic left sided heart
depend on PFA to supply systemic system (RV flow)
clinical presentation postductal aortic coarction
Bicuspid AV in 50%
Surgically treatable HTN upper > Lower
reib notching on CXR
Complete AV septal defect
AVSD and common AV valve
sequelae isolated PV stensosi
RV dilatation and hypetrophy
post-stenotic injury to Pulmonary artery
may be asymptomatic until adulthood
sequelae aortic stensosi
LV hypertrophy and LA dilatation
systolic murmur
direction of VSD shunt
L to R, until R sided changes due to increasing Pul HTN shift to R>L
blood flood pattern PDA
at birth, from aorta > through PA > into RV
after right overload > hypertrophy,
RV > PA > Aorta
clinical presentation preductal (infantile) aortic coarction
bicuspid AV in 50%
lower body cyanosis (normal UE)
surgery in neonatal period
Partial AV septal defect
primum ASD and cleft Mitral Valve with MR
etiology Truncus arteriosus
failure of separation oaf embrylogic truncus into aorta and Pulmonary Artery
clinical sequelae truncus arteriosis
mixing of blood
increased pulmonary bloodflow > Pul HTN
cyanosis
Muscular VSD
small defect
closure spontaneous fibrous adhesion in 60% by 1 year
(multiple = swiss cheese)
perimemranous VSD
usually large
requires surgical closure
spontaneous closure by septal TV leaflet possible
AV septal defect associated ith
MV and TV anamoiles
most common form of cyanotic congenital HD
tertralogy of fallot
truncus arteriosis =
origin of aorta an dpulmonary artery from truncal arteral,
most have large VSD (needed for survival)
blood leaving the right ventricle is shunted to _____ via ____
to aorta via Ductus arteriorsis
features of PDA
machinery like murmur
usually seen in isolation
needed for survival in AV atresia, PV atresia
shunts that present cyanotic
R-L shunts
most common location of VSD
90% at membranous septum
in classic tetralogy of Fallot, ____ protects the lungs from overload due to RVH
sub-pulmonic stenosis
symptoms of R-L shunt
cyanosis
digital clubbing
polycythemia
chronic effects of ASD
RVH and dilation
RA and LA dilation
Anomalies causing L-R shunt
ASD
VSD
PDA
AVSD
physical features Tricuspid atresia
RV hypoplasia,
mitral valve enlarged (unequal division of common AV valve)
sequelae Transposition of great arteries
aorta anterior and right of pulmonary
pulmonary+systemic circulation seperate
RVH
Pulmonary HTN (unilis pulmonary stenosis present)
associated with digeorge
truncus arteriosis
in right to left shunts, paradoxical emboli occur when
clots or gas bubbles are not filtered by lugns and pass directly into systemic
two types of aortic coarction
preductal - infantile = tubular hypoplasia with PDA
post ductal-adult = ridgelike infolding at ligament without PDA
L-R shunts result in
increased pulmonary blood flow
pul HTN, RV hypertrophy
eventually shifts to R-L shunt
pulmonary vasculature tolerates increase in ___ well but not increases in ____
increased in flow, not increases in pressure
most abnormalities arise during
weeks 3-8 of embryogenesis
clinical features ASD
often asymptomatic
paradoxical embolism
tetralogy of fallot
anterior superior displacement of infundibular septum leads to
- VSD
subpulmonary stenosis
- overriding aorta
- RV hypertrophy
complete AVS association,
treatment
downs syndrome,
early surgical correction
tricuspid atresis blood pattern
no flow into RV, must flow through ASD or PFO to reach left ventricle,
need VSD to reach RV, oxygenate blood in lungs
blood in the IVC is shunted to ____ via ____
left atrium via oval foramen
etiology total anamalous pulmonary venous return
pulmonary vein fails to develop or regress
shunts that present with late cyanotic
L-R shunts
normal ductus arteriorus closure timelien
fxl closure at 12h
structural at 3 months
oxygen rich blood from the placenta is shunted around the liver via
ductus venosus
late effects of CHD
endocarditis
hyperviscosity
Pulm HTN and shunt reversal (eisenmeiger complex)
Childbearing risk
residual post-surgical pathology
plexogenic pulmonary HTN
medial hypertrohpy
intimal proliferation
plexiform lesions
(VSD > PDA >> ASD)
sequelae of Ebstein anomaly of tricuspid
RV and RA dilatations
arrhythmieas (WPW due to conduction disrupt by stretching)
locations of ADD
Secoundum (at dossa ovalis) (90%)
Primum, adjacent to AV valves
Sinus venosus, near SVC entrance
ebstein anomaly of tricuspid vavle
inferioal displaced and adherent septal and posterior leaflets.
reduntant anterir leaftlet,dilataed annulus with Tricuspid regurgitaion
sequelae PV atresia with intact VS
hypoplastic RV and TV
PDA needed to get blood to lungs
Pulm artery dilatation, RVH and RA dilatation
total anamalous pulmonary venous return =
pulmonary veins not to LA > LA hypoplasia
conenct via left innominate vein or coronary sinus
ASD/PFO allows oxygenated blood to enter systemic circulation
exam finding tetralogy of fallot
boot shaped heart
most common congenital heart abnormality
membranous VSD
anamolies with R-L shunts
Tetralogy of Fallot
Transposition of great arteries
tricuspid atresia
Total aomalous pulmonary venous connection
