Cardiac/pulm Flashcards

1
Q

Cat eye syndrome is associated with which cardiac anomalies

A

TAPVR (and coloboma)

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2
Q

DiGeorge is associated with which cardiac anomalies

A

Truncus (MC), IAA, RAA

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3
Q

Holt Oram is associated with which cardiac anomaly and noncardiac anomaly

A

ASD and upper limb anomaly

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4
Q

T13 and T18 is associated with which cardiac anomalies

A

VSD

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5
Q

Williams is associated with which cardiac anomalies

A

Supravalvar aortic stenosis and branch pulmonary artery stenosis

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6
Q

Ellis van Crevald is associated with which cardiac anomalies

A

Common atrium

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7
Q

Cornelia de Lange is associated with which cardiac anomaly

A

VSD

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8
Q

What does a Qp:Qs >1 signify? What is a large shunt?

A

More pulmonary blood flow; >2 is large

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9
Q

What does Qp:Qs <1 signify? What is a large shunt?

A

Not enough pulmonary blood flow; 0.7 or less is large

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10
Q

What do alpha-1 receptors do

A

increase SVR and increase contractility

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11
Q

What do beta-1 receptors do

A

increase contractility and increase HR

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12
Q

What receptors does dopa work on (low, medium, high)?

Is it good for warm or cold shock?

A

low - dopamine
medium - B1
high - A1

Good for warm shock because it will promote systemic vasoconstriction (increase SVR)

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13
Q

What receptors does dobutamine work on?

Is it good for warm or cold shock?

A

B1 and B2 (increase contractility and HR, but decreases SVR due to B2 affect) - all the B’s

Good for cold shock because it will vasodilate

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14
Q

What receptors does epi work on (low and high)?

A

low - B1 and B2 (like dobutamine - increases contractility and HR, but decreases SVR)
high - A1 and B1 (like dopa and norepi - increases SVR, contractility, and HR)

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15
Q

What receptors does norepi work on?

A

A1 and B1 (increases SVR, contractility, and HR)

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16
Q

What does milrinone work on and what is the effect?

A

Inhibits PDE-3 and increases cAMP to result in vasodilation

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17
Q

What defect is heart block Mobitz II associated with? (one thing)

A

L-TGA

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18
Q

What is complete heart block associated with? (1 disease, 2 cardiac defects)

A

SSA (anti-Ro - worse than anti-La), L-TGA, AV canal

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19
Q

What is first degree heart block associated with? (three things)

A

digoxin, hyperkalemia, and myocarditis

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20
Q

MCC of PAC’s? Other causes?

A

MCC: increased vagal tone - goes away when infant starts crying or whenever the sinus node speeds up
Others: UVC

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21
Q

What arrhythmia is Ebstein’s associated with?

A

WPW

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22
Q

What stage do the bronchioles branch?

A

Pseudoglandular

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23
Q

Which pulmonary phase does angiogenesis start?

A

Canalicular

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24
Q

When does surfactant synthesis begin?

A

Canalicular

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25
Q

Which one of the following increases expression of surfactant protein: FOXa1, GATA6, TTF-1, FGF?

A

TTF-1

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26
Q

When do alveoli start to form?

A

Saccular

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27
Q

Which type of pneumocyte makes surfactant?

A

Type II

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28
Q

Which two of the following stimulates alveolarization: T3, T4, TSH, Vitamin A?

A

T4 and vitamin A

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29
Q

Where is surfactant made and where is it stored?

A

Made in the golgi of type II pneumocytes, stored in lamellar bodies

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30
Q

What is the largest component of surfactant?

A

80% is phospholipid (10% protein, 10% cholesterol)

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31
Q

What are the two components of the phospholipid in surfactant and what are they good for?

A
  • most abundant is phosphotidylcholine (lecithin; mostly saturated)
  • phosphotidylglycerol is only 5%, but is important for spreading
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32
Q

What is SP-B good for? Hydrophobic/philic?

A
  • reduce surface tension, tubular myelin formation, and type II cell functions
  • hydrophobic
33
Q

What is SP-C good for? Hydrophobic/philic?

A
  • surface tension reduction, film stability

- hydrophobic

34
Q

What is SP-A good for? Hydrophobic/philic?

A
  • required for tubular myelin formation and host defense. A/D show up earlier than B/C
  • hydrophilic
35
Q

What is SP-D good for? Where is it not stored? Hydrophobic/philic?

A
  • antioxidation and host defense. A/D show up earlier than B/C
  • not stored in lamellar bodies
  • hydrophilic
36
Q

Role of ABCA3?

A

surfactant protein that brings lipids into lamellar bodies to make more surfactant; also involved in formation of lamellar bodies

37
Q

What is the lining cell in the pseudoglandular phase?

A

columnar –> cuboidal distally

38
Q

What is the lining cell in the canalicular phase?

A

cuboidal –> lamellar bodies later in gestation

39
Q

What is the lining cell in the saccular phase?

A

type I and II pneumocytes

40
Q

What are flat membrane/epithelial cells?

A

type I pneumocytes

41
Q

Which cells secrete mucus?

A

goblet cet

42
Q

Which cells make surfactant?

A

type II pneumocytes

43
Q

What is the most common surfactant protein deficiency and how is it inherited?

A

ABCA3
Auto recessive
Can see wide variations among family members, some die and some survive

44
Q

What is the Hering-Breuer reflex?

A

The stretch receptors are stimulated by lung inflation, after which the vagal nerve inhibits further inspiration

45
Q

Where are central chemoreceptors and what do they respond to?

A

In the medulla; respond to CO2

46
Q

Where are the peripheral chemoreceptors and what do they respond to?

A

In the carotid artery bifurcation; respond to O2

47
Q

What is most of the lining of the lung?

A

Type I pneumocytes

48
Q

What is the first pneumocyte?

A

Type II is progenitor to type I

49
Q

MCC of vascular ring? 2nd MC?

A

DAA

RAA with aberrant left subclavian

50
Q

What congenital cardiac disease can you miss only looking at preductal saturation?

A

severe coarc or IAA (normal pre-ductal sat; need to look at post-ductal sat too)

51
Q

Sat and PaO2 in the umbilical vein?

A

70% and 30 mmHg

52
Q

Noonan’s is associated with what three things?

A

dysplastic pulmonary valve, chylothorax, cryptorchidism

53
Q

MC CHD in VACTERL?

A

VSD

54
Q

CHD associated with asplenia?

A

situs inversus

55
Q

dose of cardioversion?

A

0.5-1 joules/kg, up to 2

56
Q

dose of defibrillation?

A

2-4 joules/kg, up to 10

57
Q

Hypokalemia - prolonged ___, depressed ___

A

prolonged QT, depressed ST, U wave

58
Q

Hyperkalemia at >6, >7.5, and >9?

A

> 6: peaked T waves
7.5: wide PR, wide QRS
9: absent P, sinusoidal

59
Q

digoxin toxicity? (2 things)

A
prolonged PR (not really toxicity, but expected)
AV block
60
Q

Treatment of A flutter?

A

cardioversion

61
Q

3rd arch?

A

carotid artery

62
Q

4th arch?

A

aortic arch

63
Q

What infection is R CDH associated with?

A

GBS pneumonia

64
Q

How many alveoli are there in the neonate and adult?

A

100 million in neonate, 500 million in adult

65
Q

Which of the following is higher in FLF than plasma: sodium, potassium, chloride, bicarb, protein?

Is FLF more or less acidic than plasma?

A

High sodium, potassium, and chloride
Low bicarb and protein
More acidic

66
Q

6th arch?

A

pulmonary arteries

67
Q

What medication inhibits ENaC? What medication inhibits Na-K-2Cl transporter?

A

Amiloride inhibits ENac

Bumetinide and vasopressin inhibits Na-K-2Cl

68
Q

What is the rate limiting channel for FLF excretion?

A

ENaC

69
Q

What ion drives FLF production? What ion drives FLF excretion?

A

production: Cl into airspace
excretion: Na into interstitial space

70
Q

Match the meds with the channel they work on:

  • epinephrine, vasopressin, glucocorticoids, aldosterone
  • ENaC, Na-K-2Cl, Na-K-ATPase
A
  • epinephrine and glucocorticoids (including BMZ and cortisol) work on ENaC
  • vasopressin works on Na-K-2Cl
  • aldosterone works on Na-K-ATPase
71
Q

Which of the following do NOT stimulate surfactant:

steroids, thyroid hormones, PGE1, estrogen, indomethacin?

A

indomethacin

72
Q

MC site of upper airway obstruction?

A

Pharynx

73
Q

Haldane effect is binding of __ to hemoglobin causes unloading of ___

Bohr effect is binding of __ to hemoglobin causes unloading of ___

A

Haldane effect is binding of O2 to hemoglobin causes unloading of CO2

Bohr effect is binding of CO2 to hemoglobin causes unloading of O2

74
Q

Terminal bronchioles complete development when?

Respiratory bronchioles complete development when?

A

at 16 weeks

at 3 years of age

75
Q

FLF transported via which apical transporters?

A

Cl- (pushes chloride in during gestation) and ENaC (pulls sodium out prior to delivery)

76
Q

Truncus - what do you hear with stethoscope?

A

loud single S2, mid-systolic ejection click, pansystolic murmur

  • truncal valve function is important prognosticator
  • early CHF from pulmonary overload
  • 1/3 have DiGeorge
77
Q

On volume/pressure curve, what order do the meds go on the curve L -> R?

A

D - V - I

diuretic, vasodilator, inotrope

78
Q

MCC of hypertrophic cardiomyopathy?

A

Noonan’s (also Pompe’s)