Cardiac/pulm Flashcards
Cat eye syndrome is associated with which cardiac anomalies
TAPVR (and coloboma)
DiGeorge is associated with which cardiac anomalies
Truncus (MC), IAA, RAA
Holt Oram is associated with which cardiac anomaly and noncardiac anomaly
ASD and upper limb anomaly
T13 and T18 is associated with which cardiac anomalies
VSD
Williams is associated with which cardiac anomalies
Supravalvar aortic stenosis and branch pulmonary artery stenosis
Ellis van Crevald is associated with which cardiac anomalies
Common atrium
Cornelia de Lange is associated with which cardiac anomaly
VSD
What does a Qp:Qs >1 signify? What is a large shunt?
More pulmonary blood flow; >2 is large
What does Qp:Qs <1 signify? What is a large shunt?
Not enough pulmonary blood flow; 0.7 or less is large
What do alpha-1 receptors do
increase SVR and increase contractility
What do beta-1 receptors do
increase contractility and increase HR
What receptors does dopa work on (low, medium, high)?
Is it good for warm or cold shock?
low - dopamine
medium - B1
high - A1
Good for warm shock because it will promote systemic vasoconstriction (increase SVR)
What receptors does dobutamine work on?
Is it good for warm or cold shock?
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
What receptors does epi work on (low and high)?
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)
What receptors does norepi work on?
A1 and B1 (increases SVR, contractility, and HR)
What does milrinone work on and what is the effect?
Inhibits PDE-3 and increases cAMP to result in vasodilation
What defect is heart block Mobitz II associated with? (one thing)
L-TGA
What is complete heart block associated with? (1 disease, 2 cardiac defects)
SSA (anti-Ro - worse than anti-La), L-TGA, AV canal
What is first degree heart block associated with? (three things)
digoxin, hyperkalemia, and myocarditis
MCC of PAC’s? Other causes?
MCC: increased vagal tone - goes away when infant starts crying or whenever the sinus node speeds up
Others: UVC
What arrhythmia is Ebstein’s associated with?
WPW
What stage do the bronchioles branch?
Pseudoglandular
Which pulmonary phase does angiogenesis start?
Canalicular
When does surfactant synthesis begin?
Canalicular
Which one of the following increases expression of surfactant protein: FOXa1, GATA6, TTF-1, FGF?
TTF-1
When do alveoli start to form?
Saccular
Which type of pneumocyte makes surfactant?
Type II
Which two of the following stimulates alveolarization: T3, T4, TSH, Vitamin A?
T4 and vitamin A
Where is surfactant made and where is it stored?
Made in the golgi of type II pneumocytes, stored in lamellar bodies
What is the largest component of surfactant?
80% is phospholipid (10% protein, 10% cholesterol)
What are the two components of the phospholipid in surfactant and what are they good for?
- most abundant is phosphotidylcholine (lecithin; mostly saturated)
- phosphotidylglycerol is only 5%, but is important for spreading
What is SP-B good for? Hydrophobic/philic?
- reduce surface tension, tubular myelin formation, and type II cell functions
- hydrophobic
What is SP-C good for? Hydrophobic/philic?
- surface tension reduction, film stability
- hydrophobic
What is SP-A good for? Hydrophobic/philic?
- required for tubular myelin formation and host defense. A/D show up earlier than B/C
- hydrophilic
What is SP-D good for? Where is it not stored? Hydrophobic/philic?
- antioxidation and host defense. A/D show up earlier than B/C
- not stored in lamellar bodies
- hydrophilic
Role of ABCA3?
surfactant protein that brings lipids into lamellar bodies to make more surfactant; also involved in formation of lamellar bodies
What is the lining cell in the pseudoglandular phase?
columnar –> cuboidal distally
What is the lining cell in the canalicular phase?
cuboidal –> lamellar bodies later in gestation
What is the lining cell in the saccular phase?
type I and II pneumocytes
What are flat membrane/epithelial cells?
type I pneumocytes
Which cells secrete mucus?
goblet cet
Which cells make surfactant?
type II pneumocytes
What is the most common surfactant protein deficiency and how is it inherited?
ABCA3
Auto recessive
Can see wide variations among family members, some die and some survive
What is the Hering-Breuer reflex?
The stretch receptors are stimulated by lung inflation, after which the vagal nerve inhibits further inspiration
Where are central chemoreceptors and what do they respond to?
In the medulla; respond to CO2
Where are the peripheral chemoreceptors and what do they respond to?
In the carotid artery bifurcation; respond to O2
What is most of the lining of the lung?
Type I pneumocytes
What is the first pneumocyte?
Type II is progenitor to type I
MCC of vascular ring? 2nd MC?
DAA
RAA with aberrant left subclavian
What congenital cardiac disease can you miss only looking at preductal saturation?
severe coarc or IAA (normal pre-ductal sat; need to look at post-ductal sat too)
Sat and PaO2 in the umbilical vein?
70% and 30 mmHg
Noonan’s is associated with what three things?
dysplastic pulmonary valve, chylothorax, cryptorchidism
MC CHD in VACTERL?
VSD
CHD associated with asplenia?
situs inversus
dose of cardioversion?
0.5-1 joules/kg, up to 2
dose of defibrillation?
2-4 joules/kg, up to 10
Hypokalemia - prolonged ___, depressed ___
prolonged QT, depressed ST, U wave
Hyperkalemia at >6, >7.5, and >9?
> 6: peaked T waves
7.5: wide PR, wide QRS
9: absent P, sinusoidal
digoxin toxicity? (2 things)
prolonged PR (not really toxicity, but expected) AV block
Treatment of A flutter?
cardioversion
3rd arch?
carotid artery
4th arch?
aortic arch
What infection is R CDH associated with?
GBS pneumonia
How many alveoli are there in the neonate and adult?
100 million in neonate, 500 million in adult
Which of the following is higher in FLF than plasma: sodium, potassium, chloride, bicarb, protein?
Is FLF more or less acidic than plasma?
High sodium, potassium, and chloride
Low bicarb and protein
More acidic
6th arch?
pulmonary arteries
What medication inhibits ENaC? What medication inhibits Na-K-2Cl transporter?
Amiloride inhibits ENac
Bumetinide and vasopressin inhibits Na-K-2Cl
What is the rate limiting channel for FLF excretion?
ENaC
What ion drives FLF production? What ion drives FLF excretion?
production: Cl into airspace
excretion: Na into interstitial space
Match the meds with the channel they work on:
- epinephrine, vasopressin, glucocorticoids, aldosterone
- ENaC, Na-K-2Cl, Na-K-ATPase
- epinephrine and glucocorticoids (including BMZ and cortisol) work on ENaC
- vasopressin works on Na-K-2Cl
- aldosterone works on Na-K-ATPase
Which of the following do NOT stimulate surfactant:
steroids, thyroid hormones, PGE1, estrogen, indomethacin?
indomethacin
MC site of upper airway obstruction?
Pharynx
Haldane effect is binding of __ to hemoglobin causes unloading of ___
Bohr effect is binding of __ to hemoglobin causes unloading of ___
Haldane effect is binding of O2 to hemoglobin causes unloading of CO2
Bohr effect is binding of CO2 to hemoglobin causes unloading of O2
Terminal bronchioles complete development when?
Respiratory bronchioles complete development when?
at 16 weeks
at 3 years of age
FLF transported via which apical transporters?
Cl- (pushes chloride in during gestation) and ENaC (pulls sodium out prior to delivery)
Truncus - what do you hear with stethoscope?
loud single S2, mid-systolic ejection click, pansystolic murmur
- truncal valve function is important prognosticator
- early CHF from pulmonary overload
- 1/3 have DiGeorge
On volume/pressure curve, what order do the meds go on the curve L -> R?
D - V - I
diuretic, vasodilator, inotrope
MCC of hypertrophic cardiomyopathy?
Noonan’s (also Pompe’s)