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Clin Med- P > peds: congenital heart defects > Flashcards

peds: congenital heart defects Flashcards

1
Q

_______ prevent the closing of the ductus arteriosus. why is this significant?

A

prostoglandins

*so stay away from prostoglandin-inhibitors late in pregnancy b/c they can cause premature closure

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2
Q

inheritance of CHD: ___% multifactorial inheritance (not related to specific genes)

A

80-85%

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3
Q

what is the cardiac lesion caused by this syndrome? Down Syndrome - Trisomy 21

A

AV canal defect

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4
Q

what is the cardiac lesion caused by this syndrome? Turner ( 46 XO)

A

coarctation

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5
Q

what is the cardiac lesion caused by this syndrome? noonan

A

pulmonary valve stenosis

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6
Q

what is the cardiac lesion caused by this syndrome? Williams

A

supravalvular aortic stenosis

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7
Q

what is the cardiac lesion caused by this syndrome? Trisomy 13 and 18

A

VSD, complex lesions

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8
Q

what is the cardiac lesion caused by this syndrome? DiGeorge

A

Truncus arteriosus, IAA

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9
Q

Implies clinical appreciation of blue color

A

cyanosis

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10
Q

cyanotic birth defects vs non-cyanotic birth defects (which have better outcomes?)

A
  • non-cyanotic: tend to have better early outcomes, not noticed as easily
  • cyanotic: problems early on and need early intervention
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11
Q

6 kinds of cyanotic birth defects

A

Transposition of the great arteries (TGA)
Total anomalous PVR (TAPVR)
*Tetralogy of Fallot (TOF)
Tricuspid atresia (+ other tricuspid valve and right heart lesions)
Truncus arteriosus
Hypoplastic left heart (HLHS)

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12
Q

transposition of the great arteries (TGA) - what is it? what are the usual symptoms?

A

pulmonary artery and aorta are switched (connected to opposite ventricles than normal) - blood can’t get back to body from lungs = cyanosis
Sxs: Generally severe cyanosis, quiet tachypnea

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13
Q

3 txt options for TGA

A

Prostaglandin infusion
Balloon atrial septostomy
Arterial switch procedure

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14
Q

what is the tetralogy of fallot?

A
  1. ventricular septal defect (VSD)
  2. pulmonary valve stenosis
  3. misplaced (“overriding”) aorta that has outflow from both RV and LV, at the VSD
  4. thickened right ventricular wall (right ventricular hypertrophy)
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15
Q

txt for tetralogy of fallot?

A
  • shunting to increase pulmonary blood flow

- closure of VSD, relieve pulmonary stenosis

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16
Q

tetralogy of fallot can have long term problems with what two things?

A

arrythmias + pulmonary regurgitation

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17
Q

truncus arteriosus (rare- kinda weeds)

A

a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal 2 vessels (pulmonary artery and aorta)

18
Q

symptoms from truncus arteriosus (rare- kinda weeds)

A

Develop sx’s of pulmonary overcirculation within 2-4 weeks (tachypnea, sweating, poor feeding)

19
Q

txt of truncus arteriosus (rare- kinda weeds)

A

RV-PA conduit for repair

20
Q

what is tricuspid atresia?

A

no access to right atrium - b/c of septal defect

right acts as a single ventricle

21
Q

txt for tricuspid atresia ?

A

fontan procedure

22
Q

what is the fontan procedure?

A

redirect flow from lower body to lungs:
“Blood from vena cavae is brought passively into the R and L PA. “
1. IV disconnected from heart, routed to PA w/ “conduit” (tube)
2. fenestration between conduit and RA

23
Q

what is the fontan procedure used for? what is the main point of the fontan procedure?

A

for single ventricle lesions (like tricuspid atresia)

-blood high in oxygen and blood low in oxygen are separated. That means more oxygen can get to the body

24
Q

total anomalous PVR (rare- maybe weeds)

A

the 4 pulmonary veins do not connect normally to the left atrium so they drain abnormally to the RA by way of an abnormal (anomalous) connection.
-txt: connect PV to LA

25
Q

hypoplastic left heart

A

left side of the heart can’t effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body.

26
Q

symptoms of hypoplastic left heart? will die if/when what happens?

A

present with shock, poor perfusion

Will die when ductus closes

27
Q

txt for hypoplastic left heart

A

Need PGE1 to survive

- eventual fontan procedure

28
Q

7 types of acyanotic heart defects

A 
Atrial septal defect (ASD)
*Ventricular septal defect (VSD)
Endocardial cushion defect (ECD)
Aortic stenosis (AS)
Pulmonary stenosis (PS)
Coarctation of the aorta (COA)
Patent ductus arteriosus (PDA)
29
Q

most common adult CHD

A

atrial septal defect (most commonly not noticed until adulthood- asymptomatic in kids)

30
Q

atrial septal defect

A

left to right shunt
*increased RV pressures causes atrial and ventricular dilation (with increased atrial arrhythmias), and R–>L embolus movement, resulting in strokes.

31
Q

most common CHD overall?

A

ventrical-septal defect (VSD)

32
Q

VSD

A
  • small ones close on their own, larger ones will be symptomatic.
  • PVR falls, pulmonary blood flow increases (increasing work for right side of heart)
33
Q

symptoms of VSD

A
  • Tachypnea, poor feeding and weight gain

- Heart murmur prominent (loud), seldom missed (but big VSD can actually be missed- not as easy to hear)

34
Q

txt for VSD

A

surgical closure

35
Q

coarctation of the aorta: presentation

A
  • shock in the first 2 weeks of life
  • HTN in children
  • generally no symptoms post neonatal period
36
Q

how to test for coarctation of aorta

A

Coarctation can obstruct orifice of L subclavian

  • giving different BPs in the 2 arms
  • LE BP will be lower, since descending aorta is after the coarctation.
37
Q

what is the most common defect in down syndrome?

A

AV canal (Atrial septal defect)

38
Q

Pediatric cardiologist can differentiate innocent from organic murmurs with _____% accuracy
why is this significant?

A

96-98% accuracy

Echo not needed majority of cases

39
Q

how is fetal circulation different from post-birth?

A

consists of 3 shunts to bypass organs that are not fully formed (liver + lungs)

40
Q

what is the path of fetal circulation

A

O2 blood from mom - placenta- umbilical cord–>

  1. RA - foramen ovale - LA
  2. LA- LV- ascending aorta
  3. ascending aorta- brain & heart, body
  4. SVC- RA- RV
  5. RV- ductus arteriosus (bypasses lungs) - descending aorta
  6. descending aorta- umbilical arteries- placenta

Clin Med- P (124 decks)

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