Congenital heart disease

Question 1

what is congential heart disease

Answer 1

anatomical malformation of the heart

Question 2

3 steps to malformation

Answer 2

1. cause
2. patho mech
3. anatomic malformation

Question 3

what is period of vulnerability

Answer 3

timing is critical

- esp days 15-60

Question 4

3 main etiologies

Answer 4

1. multifactorial/polygenic >70%
2. chromo
3. teratoggenic

Question 5

2 main types of chromo

Answer 5

1. chromo abnormality
   - trisomy 21
2. microdeletion
   - degeorge syndrome
   - williams

Question 6

2 main classifications of CHD

Answer 6

1. isolated/simple
   - single defect
   - septal defect, patent DA
2. complex
   - multiple defects
   - more extensive - truncus arteriosus

Question 7

most common defect

Answer 7

vent. septal defect

Question 8

3 main pathophysiologies

Answer 8

1. shunts
2. obst.
3. both

Question 9

what are shunts

Answer 9

abnormal communication between 2 sides
- patency of fetal structures
- incompletes closrue of septa in dev.
shunt direction depends on pressure of system

Question 10

4 features of teratology of fallot

Answer 10

cyanotic CHD

1. pulm valve stenosis
2. vent. septal defect
3. over-ridingaorta
4. RV hypertrophy

Question 11

what happens in RtoL shunt

Answer 11

• systemic blood comes back to RV
• portion through pulm valve
• majority goes into LV and back out into body

Question 12

cons. of RtoL

Answer 12

• blood shunted away from lungs

- blue baby

Question 13

2 colors of CHD and their patho

Answer 13

1. cyanotic
   - RtoL shiunt
   - low O2
2. acyanotic
   - LtoR shunts at first
   - occurs in most forms of CVS obstruction

Question 14

2 main causes if RtoL shunt

Answer 14

1. fallot

2. transporitoon of great artereies

Question 15

what happens in transposition

Answer 15

• aorta and pulm. A are switched

- separate systems - death

Question 16

6 clinical cons. of R to L shunt

Answer 16

1. cyanosis
2. RV hypertrophy
3. systemic hypoxemia
4. heart failure
5. polycythemia
6. spells and sudden death

Question 17

what is patho of LtoR shunt

Answer 17

blood shunted from the systemic circulation into the pulm. circulation
intially - acyanotic
later - cyanotic - as the lungs get over perfused they become resistant

Question 18

3 common L to R shunts

Answer 18

1. patent ductus arteriosus
2. atrial septal defect
3. vent. septal defect

Question 19

what happens to lung over time in LtoR shunt

Answer 19

plexogenic pulm arteriopathy

- structural changes the are irreveresible to deal with the increased pressures

Question 20

clinical cons. of LtoR shunt

Answer 20

Large - early congesitve heart failure

late - pulm HT - cor pulmonale

Question 21

2 classes of obstructions that can occur

Answer 21

stenosis - narrowing

atresia - absence of pathway

Question 22

5 features of hypoplastic L heart syndrome

Answer 22

1. Aortic valve atresia/stenosis
2. hypoplasia of LV - no blood
3. mitral valve atresia/stenosis
4. hypoplasia of Asc. aorta
5. atrial septal defect or patent DA

Question 23

what is needed to keep hypoplastic heart alive

Answer 23

shunt - keep DA open

Question 24

2 main types of coarctation of aorta

Answer 24

1. infantile type - preductal
   - differential cyanosis - lower body cyanosis, upper body fine
2. adult type
   - differential blod pressure - low after, high above

Question 25

2 main surgical intervention types and their 2 subtypes

Answer 25

1. augment blood flow
   - palliative - shiunts, stents
   - corrective - dilate or replace valves
2. restrict flow
   - palliative - pulm. rt. banding
   - corrective - closing or patching defects