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Person Peds > Bernstein #2 > Flashcards

Bernstein #2 Flashcards

1
Q

4 things that obstruct systemic flow

A
  • Aortic stenosis
  • Coarctation of aorta
  • Interrupted aortic arch
  • Hypoplastic left heart
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2
Q

What is coarctation?

A

Narrowing of the aorta

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3
Q

3 coarctation tidbits.

A
  • 98% juxtaductal
  • Male to female ration 3:1
  • 7% of CHD
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4
Q

What other heart anomaly is coarctation associated with?

A

Bicuspid aortic valve in 70% cases

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5
Q

What genetic syndrome is coarctation seen in?

A

Turners

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6
Q

Coarctation obstruct what and causes what?

A

LV outflow

-LV hypertrophy

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7
Q

What is the classic signs of coarctation?

A
  • Diminished or absent femoral pulses
  • High BP in upper extremities
  • Systolic HTN of upper extremities
  • Pulse discrepancy between arms
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8
Q

What are clinical signs of coarctation?

A
  • Lower extremity hypoperfusion
  • Acidosis
  • Heart failure
  • Shock
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9
Q

Coarctation reveals what on CXR?

A
  • Cardiomegaly

- Rib notch

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10
Q

in coarctation is essential to maintain ___________ with ________.

A
  • Ductus arteriosus

- Prostaglandin E

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11
Q

4 ductus arteriosus dependent diseases that obstruct systemic flow.

A
  • Aortic Stenosis
  • Coarctation
  • Aortic arch interuption
  • Hypoplastic left heart
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12
Q

What interventions are needed for systemic flow obstructions?

A
  • Surgery within 1st days of life
  • inotropes
  • Mech ventilation
  • diuretics w/ Qp/Qs
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13
Q

3 obstructions to pulmonary flow

A
  • Pulmonary stenosis
  • Tetralogy of fallot
  • pulmonary atresia
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14
Q

T/F obstructions to pulmonary blood flow are ductal dependant and require prostaglandin.

A

True

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15
Q

Name an example of parallel circulation.

A

D-transposition of great arteries

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16
Q

What is parallel circulation?

A
  • RV ejects into aorta

- LV ejects to lungs

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17
Q

During parallel circulation ______ ______ is used enlarge atrial shunt

A

Balloon septoplasty

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18
Q

What are 3 single ventricle lesions?

A
  • tricuspid atresia
  • Double inlet LV
  • Unbalance AV septal defect
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19
Q

What is a double inlet LV?

A

-both atria empty into LV

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20
Q

What does an unbalanced AV septal defect do?

A

-complete mixing of systemic and pulmonary blood

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21
Q

Name 2 intrinsic myocardial disorders.

A
  • Cardiomyopathy

- myocarditis

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22
Q

What determines the severity of a shunt?

A
  • Size of orifice

- Outflow resistance on either side

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23
Q

Who needs endocarditis prophylaxis?

A
  • Prosthetic valve
  • Previous endocarditis
  • Certain CHD
  • Cardiac transplants w/ vavuloplathy
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24
Q

What CHD need endocarditis prophylaxis?

A
  • Unrepaired cyanotic CHD including shunts and conduits
  • prosthetic material for 1st 6 months
  • residual defects
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25
Q

what antibiotics are used for endocarditis?

A

-Amoxicllin 50
-Ancef - 50
Clindamycin - 20

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26
Q

What is Kawasaki disease?

A
  • Mucocutaneous lymphnode syndrome

- Causes vasculitis

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27
Q

In kawasaki disease, coronary artery dilation can cause what?

A
  • Aneurysm

- MI

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28
Q

Trisomy 21 physical abnormalities.

A
  • Small for age
  • Short neck
  • Low ears
  • Large tongue
  • Small teeth and jaw
  • Narrow nsaopharynx
  • Large tonsils
  • Decreased muscle tone
  • C spine disorders
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29
Q

Trisomy 21 anesthesia concerns

A
  • OSA
  • MR
  • Thyroid disease
  • Poor vascular access
30
Q

CV defect in 50% of trisomy 21, what are they?

A
  • AV septal
  • VSD
  • TOF
  • PDA
31
Q

What effect does anesthesia have on trisomy 21?

A
  • Bradycardia

- Pulmonary HTN

32
Q

Turner syndrome abnormalities

A
  • X linked
  • Webbed neck
  • Low ears
  • Small jaw
  • Lymphedema
  • Ovarian failure
  • DM
  • Liver disease
  • Obesity
  • Hypothyroid
33
Q

Turner CV defects

A
  • Aortic coarctation
  • Bicuspid aortic valve
  • HTN
34
Q

What is williams syndrome?

A
  • Chromosome 7
  • Deletion of elastin gene
  • Elfin faces
35
Q

Williams syndrome abnormalities

A
  • Hypercalcemia
  • Hypothyroid
  • MR
  • Growth deficiency
  • Altered neurodevelopment
  • Autism
36
Q

Williams syndrome CV problems

A
  • Aortic stenosis
  • Aortic coarctation
  • Coronary artery stenosis
  • Narrow abd and renal arteries
37
Q

What must be avoided in williams syndrome anesthesia?

A

-Hypotension

38
Q

What is the leading cause of cardiac arrest in the perioperative cardiac arrest registry?

A

William’s syndrome

39
Q

What is DiGeorge / Velocardiofacial Syndrome

A

-22q11.2 deletion syndrome

40
Q

What does DiGeorge cause?

A
  • Cardiac deficits
  • Thymic hypoplasia
  • Cleft plaate
  • hypocalcemia
41
Q

What cardiac abnormalities are seen in DiGeorge?

A
  • Conotruncal abnormalities

- Outflow tract

42
Q

What type of blood do DiGeorge patients need and why?

A
  • Irradiated

- Thymic hypoplasia

43
Q

What type of CHD does noonan syndrome have?

A
  • Pulmonary valve dysplasia

- Pulmonary stenosis

44
Q

Why does Noonan need coags?

A

-Bleeding diathesis

45
Q

What is marfans syndrome?

A
  • Mutation of fibrillin gene

- Connective tissue protein

46
Q

What is unique about marfans?

A
  • Aortic dissection can occur at anytime?
  • Arrhythmias
  • Beta Blockers
  • BP control
  • Ventricular dilation
47
Q

What type of pulmonary deformities presents with marfans?

A
  • Chest wall deformities
  • Scoliosis
  • Restrictive lung disease
48
Q

What is VACTERL association

A
  • Group of non random abnormalities
  • Vertebral
  • Anal
  • CV
  • Trachesophegal
  • Renal
  • Limbs
49
Q

An appropriate blood pressure for a neonate should be:

A

65/40

50
Q

What is CHARGE association?

A
  • Coloboma (eye)
  • Heart defects
  • Choanal atresia (blocked nose)
  • Retardation of growth
  • GU problems
  • Ear abnormalities
51
Q

How do peds compensate for chronic hypoxia?

A
  • ↑ erythropesis
  • ↑ volume
  • ↑ 2,3 DPG
52
Q

Why should cyanotic children not be NPO any longer than guidelines?

A
  • Dehydration leads to ↑ viscosity

- ↑ viscosity leads to thrombi formation

53
Q

HCT over ______ will impair microvascular perfusion.

A

65%

54
Q

What 2 things increase a Right to Left shunt?

A
  • ↓ SVR
  • ↑ PVR
  • Will lead to desaturation
55
Q

What 2 things will increase a left to right shunt?

A
  • ↑ SVR

- ↓ PVR

56
Q

Left to right shunt does what?

A
  • Blood is hunted to lungs
  • Hypotension
  • Acidosis
  • ↓ coronary perfusion
57
Q

CHF signs in PEDS

A
  • Failure to thrive
  • Difficult feeding
  • Breathlessness
  • Chest infection
  • Tachycardia
  • Murmur
  • Hepatomegaly
  • Cardiamegaly
  • Pulm plethora
  • Wheeze
58
Q

Does the premature infant’s heart exhibit greater or lesser sensitivity to catecholamines?

A
  • Less sensitive

- Already near max

59
Q

What 2 structures does the PDA connect?

A

-Descending aorta to main pulmonary artery

60
Q

When is PDA common?

A

-Preterm infants

61
Q

Things needed preop for PDA repair.

A
  • Cross match blood
  • ABX
  • Vit K
62
Q

3 problems w/ PDA repair

A
  • Difficult ventilation
  • Hemorrhage
  • ligation of aorta/pulm artery
63
Q

Where are pulse ox monitors placed in PDA repair and why?

A
  • R hand
  • Lower limb
  • If lower limb is lost, indication of aorta clamp
64
Q

What is the most common cyanotic CHD defect?

A

Tetralogy of fallot

65
Q

4 common things in tetraolgy of fallot?

A
  • VSD
  • Overriding aorta
  • RV outflow tract obs
  • RV hypertophy
66
Q

What is a TET spell?

A

Dynamic narrowing that leads to a hypercyanotic episode.

-Increases R to L shunt

67
Q

In tetralogy of fallot, the R to L shunt is dependent on what?

A
  • R ventricular outflow tract obstruction

- VSD

68
Q

In tetralogy of fallot, hypoxemia depends on what?

A

relationship between RVOTO and SVR

69
Q

tetralogy of fallot is associated with what?

A

DiGeorge

Trisomy 21

70
Q

In tetralogy of fallot, what causes the hypercyanotic flow?

A
  • Crying
  • Feeding
  • Metabolic acidosis
  • ↑ PaCo2
  • Cathecholamine
  • Surgical stimulation
71
Q

What interventions are needed for a TET spell?

A
  • Phenylepherine .5-1 mcg/kg
  • 100% O2
  • Fluid Bolus
  • Sedation (fent, morphine)
  • Bicarb
  • Beta blocker
72
Q

Why beta blockers for Tet spell?

A
  • Relaxes infundiblar spasm

- propanolol

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