8.3 Congenital Defects Flashcards
What heart defect to suspect:
-fetal alcohol syndrome
VSD
What heart defect to suspect:
-Bicuspid aortic valve
Coarctation of the Aorta, adult form
Transposition of the great vessels
- what is it
- Tx
- aorta and pulm artery switch places. Lethal immediately after birth
- Treat by surgery–switching places of vessels.
- To keep infant alive before definitive surgery, do 2 things:
1. PGE to keep PDA for blood flow
2. surgical creation of septal shunts in heart
Truncus arteriosus
- trunk of aorta/pulmonary artery fails to fully divide
- causes cyanosis
What congential disorder is Transposition of the Great Vessels associated with?
-maternal diabetes
what congential disorder is ASD associated with?
Down’s (with less common ostium primum type of ASD)
What heart defect to suspect:
-Down syndrome
ASD (ostium primum type, not the more common ostium secondum)
Tx of PDA
- Indomethacin, to decrease PGE
- PDA is maintained by PGE (‘pgE kEEps PDA open’)
Coarctation of the Aorta
-describe both types
Aorta is narrowed.
- Infantile
- coarctation occurs between PDA and aortic arch. Because of low pressure distal to coarctation, there is R-L shunt through the PDA, resulting in lower extremity cyanosis - Adult
- no PDA. HTN of upper extremities, hypotension of lower extremities (weak pulses). Blood will try to bypass coarctation through intercostal arteries, causing ‘notching of ribs’ by engorging those arteries.
Eisenmenger’s syndrome
-what results (4)
- cyanosis (reversal to R-L shunt)
- RV hypertrophy (from increase in pulm resistance)
- polycythemia (low pO2 increases EPO, increasing hematocrit)
- Clubbing (caused by cyanosis)
Clinical progression, symptoms of PDA
- asymptomatic at birth
- eventual Eisenmenger’s syndrome, results in cyanosis of lower extremities b/c PDA is located distal to aortic arch
What congential disorder is PDA associated with?
Congenital rubella (german measles)
what does PDA murmur sound like?
-constant, machine-like murmur
What heart feature is Coarctation of the Aorta (adult form) associated with?
bicuspid aortic valve
Tetralogy of Fallot
- what do patients learn to do
- characteristic X Ray finding
- pts learn to squat to increase arterial resistance, pushing back the shunted blood towards pulmonic circulation
- Boot shaped heart
What congential disorder is Coarctation of the Aorta (infant form) associated with?
Turner’s
baby with lower extremity cyanosis:
-what to suspect?
Think R-L shunt through PDA (distal to aortic arch)
- Coarctation of the Aorta (infantile)
- PDA with Eisenmenger’s syndrome
Suspect what? Adult on physical exam:
-you find strong pulses in upper extremities but weak pulses in lower extremities
Coarctation of the Aorta (adult)
Tetralogy of Fallot
- those 4 things
- which one determines severity of disorder
- RV stenosis
- VSD
- Aorta attaches to VSD
- RV hypertrophy
- the RV stenosis level determines severity by determining how much blood is shunted to the VSD aorta (causing cyanosis).
What heart defect to suspect:
-congenital rubella (german measles)
PDA
tricuspid atresia
- failure of formation of tricuspid valve (blood can not enter RV)
- often associated with a ASD (where else will the blood go) , so you get R-L shunt, early cyanosis
What weeks in embryogenesis do cardiac defects usually occur?
weeks 3-8
what is most common congenital heart defect?
VSD
ASD
- types
- clinical finding on physical exam
- complications
- Ostium secundum (90%)
- Ostium primum (associated with Down’s)
- fixed splitting of S2, independent of inspiration. Pulmonic valve closes later, from extra blood.
- can eventually cause Eisenmenger’s syndrome
- also possible paradoxical emboli
