Congenital heart disease Flashcards
what is congential heart disease
anatomical malformation of the heart
3 steps to malformation
- cause
- patho mech
- anatomic malformation
what is period of vulnerability
timing is critical
- esp days 15-60
3 main etiologies
- multifactorial/polygenic >70%
- chromo
- teratoggenic
2 main types of chromo
- chromo abnormality
- trisomy 21 - microdeletion
- degeorge syndrome
- williams
2 main classifications of CHD
- isolated/simple
- single defect
- septal defect, patent DA - complex
- multiple defects
- more extensive - truncus arteriosus
most common defect
vent. septal defect
3 main pathophysiologies
- shunts
- obst.
- both
what are shunts
abnormal communication between 2 sides
- patency of fetal structures
- incompletes closrue of septa in dev.
shunt direction depends on pressure of system
4 features of teratology of fallot
cyanotic CHD
- pulm valve stenosis
- vent. septal defect
- over-ridingaorta
- RV hypertrophy
what happens in RtoL shunt
- systemic blood comes back to RV
- portion through pulm valve
- majority goes into LV and back out into body
cons. of RtoL
- blood shunted away from lungs
- blue baby
2 colors of CHD and their patho
- cyanotic
- RtoL shiunt
- low O2 - acyanotic
- LtoR shunts at first
- occurs in most forms of CVS obstruction
2 main causes if RtoL shunt
- fallot
2. transporitoon of great artereies
what happens in transposition
- aorta and pulm. A are switched
- separate systems - death
6 clinical cons. of R to L shunt
- cyanosis
- RV hypertrophy
- systemic hypoxemia
- heart failure
- polycythemia
- spells and sudden death
what is patho of LtoR shunt
blood shunted from the systemic circulation into the pulm. circulation
intially - acyanotic
later - cyanotic - as the lungs get over perfused they become resistant
3 common L to R shunts
- patent ductus arteriosus
- atrial septal defect
- vent. septal defect
what happens to lung over time in LtoR shunt
plexogenic pulm arteriopathy
- structural changes the are irreveresible to deal with the increased pressures
clinical cons. of LtoR shunt
Large - early congesitve heart failure
late - pulm HT - cor pulmonale
2 classes of obstructions that can occur
stenosis - narrowing
atresia - absence of pathway
5 features of hypoplastic L heart syndrome
- Aortic valve atresia/stenosis
- hypoplasia of LV - no blood
- mitral valve atresia/stenosis
- hypoplasia of Asc. aorta
- atrial septal defect or patent DA
what is needed to keep hypoplastic heart alive
shunt - keep DA open
2 main types of coarctation of aorta
- infantile type - preductal
- differential cyanosis - lower body cyanosis, upper body fine - adult type
- differential blod pressure - low after, high above
2 main surgical intervention types and their 2 subtypes
- augment blood flow
- palliative - shiunts, stents
- corrective - dilate or replace valves - restrict flow
- palliative - pulm. rt. banding
- corrective - closing or patching defects
