Pulmonary arterial hypertension Flashcards

1
Q

what is pulmonary arterial hypertension

A
  • progressive disease involving endothelial dysfunction -> elevated pulmonary arterial pressure and pulmonary vascular resistance
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2
Q

what is the mean pulmonary artery pressure (mPAP) in PAH

A

> 20 mmHg

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3
Q

what is pulmonary artery wedge pressure (PAWP) in PAH

A

<= 15 mmHg

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4
Q

what is the pulmonary vascular resistance (PVR) in PAH

A

> 2 Wood units

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5
Q

what is normal pulmonary arterial wedge pressure

A

4-12 mmHg

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6
Q

what is the gold standard way to diagnose PAH

A

right heart catheterization

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7
Q

what is another way other than right heart catheterization to evaluate PAH

A

echocardiogram

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8
Q

risk factors and associated conditions for PAH

A
  • collagen vascular disease
  • congenital heart disease
  • portal htn
  • HIV infection
  • drugs and toxins
  • pregnancy
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9
Q

main genetic factor for PAH

A

abnormal BMPR2 gene

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10
Q

what does PAH endothelial dysfunction cause

A
  • decreased NO2 synthase, prostacyclin production
  • increased thromboxane production, endothelin 1 production
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11
Q

what is PAH class 1

A

symptom free when physically active or resting

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12
Q

what is PAH class 2

A
  • slight limitation of physical activity - ordinary activity may cause Sx
  • comfortable at rest
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13
Q

what is PAH class 3

A
  • marked limitation in physical activity - less than ordinary activity causes Sx
  • comfortable at rest
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14
Q

what is PAH class 4

A
  • significant Sx w/ activity
  • Sx at rest
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15
Q

at what PAH class do we consider starting tx

A

class 2

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16
Q

PAH goals of therapy

A
  • alleviate Sx
  • improve QOL
  • prevent or delay disease progression
  • reduce hospitalization
  • improve survival
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17
Q

what is the result of a positive acute vasoreactivity test (AVT)

A

drop in mPAP >10 mmHg w/ PAP less than 40 mmHg w/ stable-improved cardiac output

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18
Q

when should CCBs be considered for PAH

A
  • positive responders to CCBs w/o right-sided failure or other CI to CCB
  • do not use w/o positive AVT
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19
Q

why should verapamil not be used for PAH

A

due to negative inotropic effects

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20
Q

what to do if patient does not improve to functional class I or II after CCB initation

A

start additional or alternative PAH therapy

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21
Q

recommended PAH first line CCBs if patient is a positive responder

A
  • long acting nifedipine 120-240 mg daily
  • long acting diltiazem 240-720 mg daily
  • amlodipine 20 mg daily
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22
Q

PDE-5i inhibition effects

A
  • decreases conversion of cGMP to GMP
  • increased levels of cGMP -> pulmonary vasodilation
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23
Q

PDE-5i meds for PAH

A
  • sildenafil
  • tadalfil
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24
Q

what do PDE-5i meds do that improves PAH

A
  • improved 6MWD
  • functional capacity
25
Q

when are PDE-5i meds first line for PAH

A

functional class II and III w/o rapid progression

26
Q

sildenafil (revatio) half life

A

5 hours

27
Q

tadalafil (adcirca) half life

A

15-35 hours

28
Q

sildenafil dose adjustments

A

none

29
Q

tadalafil dose adjustments

A

renal

30
Q

PDE-5i meds major drug interactions

A
  • avoid use with riociguat or nitrates (hypotension)
  • CYP3A4 substrates
31
Q

PDE-5i drugs AEs

A

flushing, headache, dyspepsia, visual disturbances, priapism, tinnitus/hearing loss, sudden vision loss, hypotension

32
Q

endothelin receptor antagonists moa

A
  • ET receptors on vascular smooth muscle mediate vasoconstriction
  • overexpression of ET-1 in PH patients, correlates with remodeling
  • blocking ET -> vasodilation
33
Q

when should endothelin receptor antagonists be used

A

an option in functional class II-IV

34
Q

what is combo first line for class II and III without rapid progression

A

tadalafil + ambrisentan

35
Q

what do endothelin receptor antagonists do

A

improve 6MWD, pro-BNP, delay time to clinical worsening, optimize hemodynamics

36
Q

how soon are improvements expected with endothelin receptor antagonists

A

8-10 weeks

37
Q

where are ETa receptors located

A

pulmonary smooth muscle walls

38
Q

ETa receptors effects

A

promotes vasoconstriction, proliferation, inflammation

39
Q

ETb receptors on endothelium effects

A

promote vasodilation, stimulate NO and prostacyclin production

40
Q

ETb receptors on muscle cells of vascular walls effects

A

cause vasoconstriction and cell proliferation

41
Q

what is going on with ET receptors in PAH

A

expression of ETb receptors are upregulated in the media of blood vessels (vasoconstriction)

42
Q

soluble guanylate cyclase stimulator med

A

riociguat (adempas)

43
Q

when could riociguat be used

A

may be used as an alternative to PDE-5i

44
Q

why can’t riociguat be used with PDE-5i

A

risk of hypotension

45
Q

prostacyclins moa

A

stimulates cAMP pathway to increase pulmonary vasodilation

46
Q

what is standard tx for severe PH w/ RV failure

A

parenteral prostacyclins -> subQ treprostinil

47
Q

when are prostacyclins used in PAH

A

reserved for class III and IV patients

48
Q

what other drug classes may prostacyclins be used with

A

combination w/ ERA + PDE-5i or riociguat

49
Q

treprostinil half life

A

4 hours

50
Q

what should not be done w/ IV treprostinil

A

IV infusion requires stable access, do not co-infuse with anything else

51
Q

when is IV used over subQ

A

for patients who cannot tolerate subQ

52
Q

why is subQ treprostinil better than IV

A
  • subQ avoids risk of central lines
  • utilizes undiluted drug
53
Q

common prostacyclin medication errors

A
  • flushing of line
  • calc or conc error
  • programming error
  • pump turned off
  • inappropriate change in weight
54
Q

when should lung transplantation be considered

A

for functional class III and IV patients w/ inadequate response to maximal pharmacotherapy

55
Q

when are adjunct therapy considered

A

treat underlying/contributing conditions like htn/sleep apnea

56
Q

when is anticoag adjunct therapy considered

A
  • consider depending on cardiac function
  • warfarin: INR goal 1.5-2.5
  • aspirin 81 mg daily
57
Q

when is diuretic adjunct therapy considered

A

to maintain euvolemia

58
Q

other supportive therapies

A
  • immunizations (influenza, pneumococcal, COVID, RSV)
  • supplemental oxygen (pulmonary vasodilation)
  • iron supplementation if deficient
  • avoid air travel/high altitudes (may need supplemental O2 to keep saturations >91%)
59
Q

why should pregnancy be avoided during PAH therapy

A
  • estrogen containing contraceptives may increase VTE risk
  • Bosentan can cause birth defects
  • ERAs and riociguat are category X