PAH

Question 1

What is PH

Answer 1

Vascular disease with structural changes

Question 2

PAH 3 Hemodynamic levels

Answer 2

mPAP > 20 mmHg
PAWP ≤15 mmHg
PVR > 2 woods unit

Question 3

Why is diagnosis of PAH difficult

Answer 3

Fatigue
SOB
Edema
sounds like HF

Question 4

Goal of treatment for PAH

Question 5

When can we use CCB

Answer 5

Positive vasodilator test

Question 6

what are the 3 CC for PAH

Answer 6

amlodipine
diltiazem
Nifedipine

Question 7

Amlodipine Dose

Question 8

Diltiazem Dose

Question 9

Nifedipine (XL) Dose

Question 10

CCB is the 4 AD most concerning

Answer 10

1. Peripheral edema
2. Flushing
3. Muscle cramps
4. Gingival hyperplasia

Question 11

What do we have to consider before giving ERA

Answer 11

Rems

Question 12

What are the warnings of ERA

Answer 12

1. All ERAs are teratogenic
2. Require monthly pregnancy test – 2 methods of contraception
3. Liver complications
4. Drug interactions
5. Edema

Question 13

Bosentan

Question 14

issue with Bosentan

Answer 14

Never recommend

Question 15

Ambrisentan

Question 16

Why Ambrisentan

Answer 16

generic
less DDI
no UTI like macitentan

Question 17

Macitentan

Question 18

why Macitentan

Answer 18

less edema

Question 19

ERA class effec

Answer 19

Edema
nasal congestion

Question 20

Warning of NO pathway durgs

Answer 20

1. Hypotension
2. nitrates

Question 21

Sildenafil

Question 22

Tadalafil

Question 23

Riociguat

Question 24

Riociguat warnings

Answer 24

• CYP inhibitors / inducers * GERD
• REMS – teratogenic

Question 25

Biggest thing to monitor with Riociguat

Answer 25

BP

Question 26

smoking and Riociguat

Question 27

Opsynvi

Question 28

What best NO i to poick

Answer 28

tadalifil

Question 29

when would i use Riociguat

Answer 29

when they need more power

Question 30

Warning of Prostacyclins

Answer 30

Serious side effects central line infections Risk of abrupt discontinuations

Question 31

Epoprostenol

Question 32

issues with Epoprostenol

Question 33

Treprostinil

Question 34

Treprostinil formulation

Question 35

Why Treprostinil

Answer 35

live far from hospital pumps are smaller

Question 36

Treprostinil Sq good?

Answer 36

dont want IV access but pain for a week then get better Try to use the same site

Question 37

why nebulizer

Answer 37

group 1 and group 3

Question 38

why dry powder

Answer 38

group 1 and group 3 easier

Question 39

know the pump rate

Question 40

Iloprost

Question 41

Selexipag

Question 42

bonus of Selexipag

Answer 42

selective

Question 44

Flolan® and Veletri®

Question 45

prost class AD

Answer 45

Headache * Jaw pain * Flushing * Nausea/diarrhea * Skin rash * Musculoskeletalpain * Infections * Interruptionininfusionlifethreatening

Question 46

sotatercept

Question 47

sotatercept need to happen

Answer 47

need to be monitored

Question 48

sotatercept hemoglobin paramenters

Answer 48

Delay treatment for at least 3 weeks if any of the following occur: * Hgb increases more than 2 g/dL from the previous value and is above the upper limit of normal (ULN) * Hgb increases more than 4 g/dL from baseline * Hgb increases more 2 g/dL above ULN * Platelet count decreases to less than 50,000/mm3

Question 49

what happens have to hold more than 9 weeks?

Question 50

game changer drug sotatercept

Question 51

biggest thing to counsle on sotatercept

Answer 51

nose bleeds

Question 52

how do we treat patietns

Answer 52

1-4 drugs depending on patient

Question 53

treatment pathway

Answer 53

Question 54

non-pharm treatments

Answer 54

surgery ( CTEPH, BPA) lung transplant

Question 54

PAH and pregnancy

Answer 54

must be avoided bc it messes up everything can do it but must be planned

Question 55

supportive thearpies

Answer 55

Dietsodium restriction * Exercisenot heavy physical, low level aerobic * Appropriate vaccinations (influenza ; pneumococcal pneumonia, COVID19) * Avoidance of pregnancy (hemodynamic changes) * Anticoagulation (if not contraindicated) * Diuretics (RV overload, peripheral edema) * Oxygen (keep O2 sat>90%) * Caution in high altitude * Psychological support