Congential Heart Disease- Leah (3) Flashcards
Two diseases that present at the time of PDA closure (3-4 days old) without cyanosis?
What must be done if these conditions are suspected?
- Aortic coarctation
- Hypoplastic left heart.
Always give these kids PGE1
Four CHDs that are not causes of cyanosis and do not directly correlate with time of PDA closure?
- ASD
- VSD
- PDA
- AVSD
*probably the least symptomatic of all of the congenital defects.
Most common congenital defect?
With what is it commonly associated?
VSD
associated with fetal alcohol poisoning
(Yes, I agre this important!)
Defect Assc with:
- Turners
- Digeorge
- Downs
- Heretotaxy
- Turners: aortic coarctation
- Digeorge: truncus arteriosus
- Downs: AVSD
- Heretotaxy: TAPVR
Most common cyanotic lesion?
Tetralogy of Fallot
How to treat cyanotic spell/ tet spell?
How does it work?
Knee chest position
Decreases systemic venous return; Increases LV resistance
Decreases R –> L shunt
*^ SVR–> ^ resistance in Lt ventricle–> Decreases R–>L shunting (I had to write this to visualize)
Classic murmur Assc with PDA?
Constant machine like murmur
Classic VSD murmur
Holosystolic murmur between T/M regions
Murmurs Assc with ASD?
Basically right sided stenotic murmurs.
- Systolic murmur in pulmonic region
- Diastolic murmur in tricuspid region
- widely fixed and split s2
- this is because you see right sided hypertrophy/ L –> R shunting.
Condition Assc with decreased femoral pulse?
Aortic coarctation.
Blood doesn’t flow through the aorta distal to the subclavian.
Classic murmur Assc with tetralogy
HARSH pulm stenosis murmur radiating into the BACK!
One murmur you can expect to hear in truncus arteriosus? Why?
Condition causes VSD.
Will cause the same murmur + cyanosis.
What murmur does TAPVR mimic?
ASD; causes right sided hypertrophy
* but will also have cyanosis.
Symptoms Assc with transposition of the great arteries?
CHF-like + cyanosis.
Tricuspid atresia Presentation: (3)
Single S2, left PMI, cyanosis.
Five diseases that present with CYANOSIS.
The five T’s.
- Truncus Arteriosus. (1 great artery)
- Transposition of the (2) great arteries
- TRIcuspid atresia
- TETralogy of fallot
- TAPVR (5 letters)
- Most common cause of ASD?
- Physical changes that occur as a result of ASD
- Symptoms
- Heart exam findings (3)
- Usually ostium secundum defect.
- Causes L –> R shunting; R sided hypertrophy; increased pulmonary flow.
- Usually asymptomatic, don’t present at birth/ during PDA closure/ with cyanosis.
- Get RV impulse, split S2, and two right sided murmurs (sounds like pulmonary + tricuspid stenosis)
VSD
- Most common cause
- Physical effects
- Symptoms
- Heart exam findings (1)
- Usually membranous defect
- L–> R shunting; LEFT hypertrophy; increased pulmonary flow.
- Typically asymptomatic; falure to thrive a 1 mos if severe
- Hear holosystolic murmur between T + M
PDA:
- Common cause/ Assc
- Physical effects
- Symptoms
- Classic murmur
- Treatment for preemies
- Assc with maternal rubella infection (first aid)
- Causes aortic –> pulm shunting after birth, leads to left hypertrophy
- Tachypnia/hepatomegly
- Constant machine like murmur
- NO indomethacin for term babies!!!!
Describe the COMPLETE AVSD defect (4).
What is the heart exam like? (2)
How do these patients present?
- -ostium Primum defect
- one AV valve annulus
-no mitral valve
-inlet ventricular septal defect
(Heart = Giant, hollow hole! good) - Hear VSD + ASD like murmurs, very loud S2
- Kiddos have symptoms of CHF but not cyanosis.
Describe the PARTIAL AVSD defect (4).
What is the heart exam like (3)?
How do these patients present?
- -ostium primum defect
- TWO AV valve annuli
- mitral valve present but cleft
- no ventricular septal defect - Presents like an ASD; largely asymptomatic
- pulmonary stenosis like murmur; RV impulse; split S2
- sounds so much like an ASD; I think the only way to differentiate in a test question is if the stem involves a kid with Downs.*
Two conditions Assc with aortic coarctation:
- Turners syndrome
2. 50% have bicuspid aortic valves = aortic stenosis later in life.
Where is the coarctation in aortic coarctation?
Just distal to subclavian
Aortic coarctation classic presentation (3)
- CHF symptoms 3-4 days after birth
- absent femoral pulse (upper extremity hypertension, lower extremity)
- Gallop rhythm.
rib notches on CXR late in life
Hypoplastic left heart syndrome
- Physical cause
- Classic presentation (2)
- Aortic + mitral stenosis —> atresia (severity varies)
- Right heart is dominant; complete mixing of venous/ arterial blood in the right atrium
- Shock at 3-4 days after birth
Tetralogy of Fallot- name the four defects
IHOP Interventricular septal defect Hypertrophy of the right heart Overriding aorta (over-rides the ventricular defect) Pulmonary stenosis
What kind of shunt is seen in tetralogy of Fallot?
Initially it is L –>R, but it progresses from R–> L as pulmonary stenosis increases.
Classic murmur and CXR Assc with Tetralogy?
- Boot shaped heart on CXR
2. Harsh pulmonary stenosis murmur radiating into the back
Truncus Arteriosus
- Physical defect
- Assc findings on heart exam (3)
- Symptoms
- Single great vessel overlying ventricular septal defect (all blood- venous and arterial- mixes in the single vessel)
- Hear VSD murmur + systolic click and diastolic murmur at the truncal valve
- Kiddo will be cyanotic
Note: This disorder is caused by improper neural crest cell migration, as is transposition of the great vessels.
Transposition of the great vessels
- most common form
- Symptoms (2)
- Classic CXR findings
- What do these babies need? What do you give them!?
- D-TGA (aorta is anterior and right; receives deoxygenated blood).
- Babies will present with CHF + cyanosis
- “Egg on string” appearance on CXR
- These babies need a PDA to send any oxygenated blood to the body. Must administer PGE
Tricuspid atresia is Assc with what other physical effects? (3)
Most often kids also have VSD, ASD, and RV hypoplasia
findings are kind of non-specific aside from cyanosis
Describe the TAPVR defect. What is necessary to maintain blood flow?
Pulmonary veins empty into the right atrium.
In order for kids to have any oxygenated blood flow, they need a patient foramen ovale/ ductus arteriosus. Have to give PGE1 if they have PDA + TAPVR
Symptomatic. Presentation of TAPVR (2 scenarios)
Heart exam findings?
Venous return OBSTRUCTED:
- Severe respiratory distress at the time of birth
- Heart exam may not be specific.
Venous return NOT OBSTRUCTED:
- Baby is asymptomatic at birth but fails to thrive
- Presents with cyanosis
- Heart exam findings similar to ASD.
Defect Assc. With maternal diabetes
Transposition of GA
