1
Q
4 things that obstruct systemic flow
A
- Aortic stenosis
- Coarctation of aorta
- Interrupted aortic arch
- Hypoplastic left heart
2
Q
What is coarctation?
A
Narrowing of the aorta
3
Q
3 coarctation tidbits.
A
- 98% juxtaductal
- Male to female ration 3:1
- 7% of CHD
4
Q
What other heart anomaly is coarctation associated with?
A
Bicuspid aortic valve in 70% cases
5
Q
What genetic syndrome is coarctation seen in?
A
Turners
6
Q
Coarctation obstruct what and causes what?
A
LV outflow
-LV hypertrophy
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
8
Q
What are clinical signs of coarctation?
A
- Lower extremity hypoperfusion
- Acidosis
- Heart failure
- Shock
9
Q
Coarctation reveals what on CXR?
A
- Cardiomegaly
- Rib notch
10
Q
in coarctation is essential to maintain ___________ with ________.
A
- Ductus arteriosus
- Prostaglandin E
11
Q
4 ductus arteriosus dependent diseases that obstruct systemic flow.
A
- Aortic Stenosis
- Coarctation
- Aortic arch interuption
- Hypoplastic left heart
12
Q
What interventions are needed for systemic flow obstructions?
A
- Surgery within 1st days of life
- inotropes
- Mech ventilation
- diuretics w/ Qp/Qs
13
Q
3 obstructions to pulmonary flow
A
- Pulmonary stenosis
- Tetralogy of fallot
- pulmonary atresia
14
Q
T/F obstructions to pulmonary blood flow are ductal dependant and require prostaglandin.
A
True
15
Q
Name an example of parallel circulation.
A
D-transposition of great arteries
16
Q
What is parallel circulation?
A
- RV ejects into aorta
- LV ejects to lungs
17
Q
During parallel circulation ______ ______ is used enlarge atrial shunt
A
Balloon septoplasty
18
Q
What are 3 single ventricle lesions?
A
- tricuspid atresia
- Double inlet LV
- Unbalance AV septal defect
19
Q
What is a double inlet LV?
A
-both atria empty into LV
20
Q
What does an unbalanced AV septal defect do?
A
-complete mixing of systemic and pulmonary blood
21
Q
Name 2 intrinsic myocardial disorders.
A
- Cardiomyopathy
- myocarditis
22
Q
What determines the severity of a shunt?
A
- Size of orifice
- Outflow resistance on either side
23
Q
Who needs endocarditis prophylaxis?
A
- Prosthetic valve
- Previous endocarditis
- Certain CHD
- Cardiac transplants w/ vavuloplathy
24
Q
What CHD need endocarditis prophylaxis?
A
- Unrepaired cyanotic CHD including shunts and conduits
- prosthetic material for 1st 6 months
- residual defects
25
what antibiotics are used for endocarditis?
-Amoxicllin 50
-Ancef - 50
Clindamycin - 20
26
What is Kawasaki disease?
- Mucocutaneous lymphnode syndrome
| - Causes vasculitis
27
In kawasaki disease, coronary artery dilation can cause what?
- Aneurysm
| - MI
28
Trisomy 21 physical abnormalities.
- Small for age
- Short neck
- Low ears
- Large tongue
- Small teeth and jaw
- Narrow nsaopharynx
- Large tonsils
- Decreased muscle tone
- C spine disorders
29
Trisomy 21 anesthesia concerns
- OSA
- MR
- Thyroid disease
- Poor vascular access
30
CV defect in 50% of trisomy 21, what are they?
- AV septal
- VSD
- TOF
- PDA
31
What effect does anesthesia have on trisomy 21?
- Bradycardia
| - Pulmonary HTN
32
Turner syndrome abnormalities
- X linked
- Webbed neck
- Low ears
- Small jaw
- Lymphedema
- Ovarian failure
- DM
- Liver disease
- Obesity
- Hypothyroid
33
Turner CV defects
- Aortic coarctation
- Bicuspid aortic valve
- HTN
34
What is williams syndrome?
- Chromosome 7
- Deletion of elastin gene
- Elfin faces
35
Williams syndrome abnormalities
- Hypercalcemia
- Hypothyroid
- MR
- Growth deficiency
- Altered neurodevelopment
- Autism
36
Williams syndrome CV problems
- Aortic stenosis
- Aortic coarctation
- Coronary artery stenosis
- Narrow abd and renal arteries
37
What must be avoided in williams syndrome anesthesia?
-Hypotension
38
What is the leading cause of cardiac arrest in the perioperative cardiac arrest registry?
William's syndrome
39
What is DiGeorge / Velocardiofacial Syndrome
-22q11.2 deletion syndrome
40
What does DiGeorge cause?
- Cardiac deficits
- Thymic hypoplasia
- Cleft plaate
- hypocalcemia
41
What cardiac abnormalities are seen in DiGeorge?
- Conotruncal abnormalities
| - Outflow tract
42
What type of blood do DiGeorge patients need and why?
- Irradiated
| - Thymic hypoplasia
43
What type of CHD does noonan syndrome have?
- Pulmonary valve dysplasia
| - Pulmonary stenosis
44
Why does Noonan need coags?
-Bleeding diathesis
45
What is marfans syndrome?
- Mutation of fibrillin gene
| - Connective tissue protein
46
What is unique about marfans?
- Aortic dissection can occur at anytime?
- Arrhythmias
- Beta Blockers
- BP control
- Ventricular dilation
47
What type of pulmonary deformities presents with marfans?
- Chest wall deformities
- Scoliosis
- Restrictive lung disease
48
What is VACTERL association
- Group of non random abnormalities
- Vertebral
- Anal
- CV
- Trachesophegal
- Renal
- Limbs
49
An appropriate blood pressure for a neonate should be:
65/40
50
What is CHARGE association?
- Coloboma (eye)
- Heart defects
- Choanal atresia (blocked nose)
- Retardation of growth
- GU problems
- Ear abnormalities
51
How do peds compensate for chronic hypoxia?
- ↑ erythropesis
- ↑ volume
- ↑ 2,3 DPG
52
Why should cyanotic children not be NPO any longer than guidelines?
- Dehydration leads to ↑ viscosity
| - ↑ viscosity leads to thrombi formation
53
HCT over ______ will impair microvascular perfusion.
65%
54
What 2 things increase a Right to Left shunt?
- ↓ SVR
- ↑ PVR
- Will lead to desaturation
55
What 2 things will increase a left to right shunt?
- ↑ SVR
| - ↓ PVR
56
Left to right shunt does what?
- Blood is hunted to lungs
- Hypotension
- Acidosis
- ↓ coronary perfusion
57
CHF signs in PEDS
- Failure to thrive
- Difficult feeding
- Breathlessness
- Chest infection
- Tachycardia
- Murmur
- Hepatomegaly
- Cardiamegaly
- Pulm plethora
- Wheeze
58
Does the premature infant’s heart exhibit greater or lesser sensitivity to catecholamines?
- Less sensitive
| - Already near max
59
What 2 structures does the PDA connect?
-Descending aorta to main pulmonary artery
60
When is PDA common?
-Preterm infants
61
Things needed preop for PDA repair.
- Cross match blood
- ABX
- Vit K
62
3 problems w/ PDA repair
- Difficult ventilation
- Hemorrhage
- ligation of aorta/pulm artery
63
Where are pulse ox monitors placed in PDA repair and why?
- R hand
- Lower limb
- If lower limb is lost, indication of aorta clamp
64
What is the most common cyanotic CHD defect?
Tetralogy of fallot
65
4 common things in tetraolgy of fallot?
- VSD
- Overriding aorta
- RV outflow tract obs
- RV hypertophy
66
What is a TET spell?
Dynamic narrowing that leads to a hypercyanotic episode.
| -Increases R to L shunt
67
In tetralogy of fallot, the R to L shunt is dependent on what?
- R ventricular outflow tract obstruction
| - VSD
68
In tetralogy of fallot, hypoxemia depends on what?
relationship between RVOTO and SVR
69
tetralogy of fallot is associated with what?
DiGeorge
| Trisomy 21
70
In tetralogy of fallot, what causes the hypercyanotic flow?
- Crying
- Feeding
- Metabolic acidosis
- ↑ PaCo2
- Cathecholamine
- Surgical stimulation
71
What interventions are needed for a TET spell?
- Phenylepherine .5-1 mcg/kg
- 100% O2
- Fluid Bolus
- Sedation (fent, morphine)
- Bicarb
- Beta blocker
72
Why beta blockers for Tet spell?
- Relaxes infundiblar spasm
| - propanolol
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