PAH-final

Question 1

PAH symptoms

Answer 1

dyspnea
fatigue
chest pain
lower extremity edema
Raynaud

Question 2

PAH diagnosis

Answer 2

right heart cath: mPAP> 20
PCWP/LVEDP <15
PVR > 3 wood units
pulmonary vaso reactivity:
positive= mPAP<40 and must be >10 from baseline

Question 3

3 STRATA

Answer 3

done at diagnosis
1 point= <5% low risk
2 points= 5-20% intermediate risk
3 points= >20% high risk

Question 4

4 STRATA

Answer 4

done at follow up
1 point= 0-3% low risk
2 points= 2-7% low-intermediate risk
3 points= 9-19% intermediate- high risk
4points= >10% high risk

Question 5

PAH non pharm

Answer 5

oxygen (<90%)
<2g sodium/day for hypervolemia
surgery (etiology)

Question 6

PAH pharm supportive therapies

Answer 6

warfarin- for idiopathic, heritable, and CTEPH (INR goal 1.5-2.5)
loops- for volume overload and congestion (edema and increased venous pressure)
digoxin- for right ventricular failure (target goal 0.5-0.8)
contraceptives
vaccines

Question 7

PAH pharm targeted therapies

Answer 7

CCB- (for vasoreactive responders) amlodipine, nifedipine, or diltiazem
prostacyclins
prostacyclin recepor agonist
endothelia receptor antagonist
phosphodiesterase inhibitor
guanylate cyclase stimulator

Question 8

prostacyclins

Answer 8

epoprostenol
treprostinil
iloprost

Question 9

epoprostenol

Answer 9

flown and veletri
IV

Question 10

treprostinil subQ/IV

Answer 10

remodulin

Question 11

treprostinil inhaled

Answer 11

tyvaso

Question 12

teprostinil oral

Answer 12

orenitram
MUST BE TAKEN W/ FOOD

Question 13

iloprost

Answer 13

ventavis
inhaled

Question 14

prostacyclin IP agonist

Answer 14

selexipag

Question 15

selexipag

Answer 15

uptarvi
oral
>3 days interruption= re titration

Question 16

endothelin antagonists

Answer 16

bosentan
ambrisentan
macitentan

Question 17

bosentan

Answer 17

tracleer
oral
CYP3A4
X pregnancy

Question 18

ambrisentan

Answer 18

letaris
oral
X pregnancy

Question 19

macitentan

Answer 19

opsumit
oral
X pregnancy

Question 20

phosphodiesterase inhibitors

Answer 20

sildenafil
tadalafil

Question 21

sildenafil

Answer 21

revatio
oral
stop if vision changes

Question 22

tadalafil

Answer 22

adcirca
oral

Question 23

combo product (endothelin antagonist/phosphodiesterase inhibitor)

Answer 23

Opsynvi

Question 24

opsynvi

Answer 24

macitentan/tadalafil
oral
fixed doses: 10/20mg and 10/40mg

Question 25

guanylate cyclase stimulator

Answer 25

riociguat

Question 26

rioiguat

Answer 26

adempas
oral
X pregnancy and PDE5

Question 27

active signaling inhibitor

Answer 27

sotatrercept

Question 28

sotatrercept

Answer 28

winrevair
subQ
X pregnancy

Question 29

treatment 3 strata

Question 30

vasoreactive positive

Answer 30

CCB

Question 31

cardiopulmonary comorbities

Answer 31

PDE5 or ERA

Question 32

without cardiopulmonary comrobities
low-intermediate risk
high risk

Answer 32

low-intermediate risk: ERA or PDE5
high risk: ERA
PDE5
PCA (IV or subQ)

Question 33

preferred PDE5 and ERA

Answer 33

tadalafil and ambrisentan
tadalafil and macitentan

Question 34

treatment 4 STRATA

Answer 34

reassess CCB effectiveness

Question 35

low risk

Answer 35

continue current therapy

Question 36

low-intermediate risk

Answer 36

combo therapy if on mono (PDE5 and ERA)

Question 37

intermediate-high and high risk

Answer 37

add IV/subQ PCA

Question 38

if on mono therapy (PDE5, ERA, pr secs) what can you add

Answer 38

treprostinil

Question 39

what can you add to PDE5 or oral/inhaled PCA

Answer 39

macitentan