Congenital Heart Disease Flashcards
list the R to L shunt CHDzs
persistent truncus arteriosus transposition of the great vessels tricuspid atresia tetralogy of fallot Total anomalous pulmonary venous return
what genetic dz predisposes to persistent truncus arteriosus?
22q11
what dz predisposes to transposition of the great vessels
diabetes in mom
what dz predisposes to tetraology of fallot
fetal alcohol syndrome
22q11
22q11 congential heart defects?
persistent truncus arteriosus
tetralogy of fallot
what dz predisposes to VSD
cri du chat
fetal OH syndrome
Downs
what dz predisposes to ASD
fetal OH syndrome
downs
what dz predisposes to PDA
congenital rubella
prematurity
fetal alcohol syndrome
what dz predisposes to coarcation of the aorta and bicuspid aortic valve?
turneres
describe persistent truncus arteriosus
truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticaopulmonary septum formation
most have a VSD accompanying it
describe transposition fo the great vessels
due to failure of the aorticopulmonary septum to spiral =
aorta leaves RV anteriorally and pulmonary trunk leaves LV posteriorly. atria are normal
= systemic and pulmonary circulations are separate
requires a shunt - VSD, PDA or patent foramen ovale to be compatible with life
describe tricuspid atresia
absence of tricuspid valve – hypoplastic RV
needs BOTH ASD and VSD to be viable
Describe tetraology of fallot
caused by anterosuperior displacement of the infundibular septum
MOST COMMON CAUSE OF EARLY CHILDHOOD CYANOSIS
1) pulmonary infundibular stenosis - prognosis determinant
2) right ventricular hypertrophy - BOOT SHAPED heart
3) overriding aorta
4) VSD (persistent truncus arteriosus also has this commonly)
boot shaped heart on CXR
tetraology of fallot
what improves the cyanosis in Tetralogy of Fallot
squatting - increases systemic venous return - decreases right to left shunt??? what the heck?
describe the SaO2 throughout the chambers of the heart in tetralogy of fallot
decreased SaO2 in the left ventricle and aorta
describe total anomalous pulmonary venous return
pulmonary veins drain into right heart circulation
associated with an ASD (persistent truncus arteriosus and tetralogy of fallot have VSD often) and sometimes a PDA t0 maintain r to l shunt to maintain CO (transposition needs a VSD, ASD or PDA and tricuspid atresia needs a VSD and ASD)
describe the frequency relationships between the congenital heart defects that cause left to right shunts
VSD>ASD>PDA
describe the SaO2 in the chambers of the heart in a VSD compared to the regular heart
increased in RV and PA
VSD occur where?
required in transposition of great vessels and with ASD in tricuspid atresia
commonly in persistent truncus arteriosus and tetralogy of fallot
cri du chat
fetal OH syndrome
down syndrome (membranous)
what sort of MI can cause a VSD
anterior wall LDA
what do you hear with an ASD
loud S1 wide fixed split S2
ostium secundum defects most common.
ostium primum more rare but usually occur with other heart defects
NOT patent foarmen ovalve - unfused, ASD 0 missing
what conditions are associated with ASD?
down - primum
fetal alcohol syndrome
often with total anomalous pulmonary venous return
needed for surivival if no vsd, pda in transposition of great vessels
describe SaO2 changes in ASD
increased SaO2 in RA, RV, PA
what can come of a PDA
differential cyanosis when lower limbs are cyanotic
what closes and opens a PDA
low O2 and PGs keep open
indomethacin and high O2 close
what conditions are associated with PDA?
may require in transposition of great vessels
congenital rubella (mom - rash, lymphadenopathy, arthtritis; baby - maybe blueberry muffin rash, PDA/pul artery hypoplasia, cataracts, deafness)
respiratory distress syndrometetralogy of fallot
what is Eisenmenger syndrome?
due to an uncorrected left to right shunt. – increased pulmonary blood flow – pathologic remodeling of vasculature – pulmonary arterial hypertension – RVH to compensate – shunt becomes right to left
CXPX of eisnemengers syndrome
a) late cyanosis
b( clubbing
c( polycythemia
what is coarctation fo the aorta associated with?
bicuspid aortic valve,
turner
presentation of coarctation of the aorta
hypertension in upper extremities
weak delaye dpulse in lower extremities (brachial-femoral delay)
collateral arteris erode ribs
OH exposure in utero
VSD
PDA
ASD
tetraology of fallot
congenital rubella
septal defects
PDA
pulmonary artery stenosis
down syndrome
Av septal defect - endocardial cushin defect
VSD
ASD
diabetic mother
transposition of great vessels
margan syndrome
MVP
thoracic aortic aneurysm and dissection
aortic regurgitation
prenatal lithium exposure
ebstein anomaly
turner syndrome
bicuspid aortic valve
coarctation of the aorta
Williams syndrome
supravlvular aortic stenosis
22q11
truncus arteriosus
tetralogy of fallot
