Exam 2: PAH

Question 1

Sildenafil: Drug Class

Answer 1

PDE5 inhibitor

Question 2

Sildenafil brand name

Answer 2

Revatio

Question 3

Revatio generic name

Answer 3

Sildenafil

Question 4

Sildenafil dosing

Answer 4

20mg TID

Question 5

Tadalafil drug class

Answer 5

PDE5 inhibitor

Question 6

Tadalafil brand name

Answer 6

Adcirca

Question 7

Adcirca generic name

Answer 7

Tadalafil

Question 8

Tadalafil dosing

Answer 8

40mg daily

Question 9

Bosentan drug class

Answer 9

ET1-RA

Question 10

Bosentan brand name

Answer 10

Traceleer

Question 11

Bosentan Dosing

Answer 11

62.5mg BID

Max 250mg BID

Question 12

Traceleer generic name

Answer 12

Bosentan

Question 13

Amrbisentan brand name

Answer 13

Letairis

Question 14

Amrbisentan drug class

Answer 14

ET1-RA

Question 15

Ambrisentan Dosing

Answer 15

2.5mg daily

Max 10mg daily

Question 16

Bosentan MOA

Answer 16

Dual ET-A and ET-B antagonist

Question 17

Ambrisentan MOA

Answer 17

Selective ET-A antagonist

Question 18

Effects of increased ET-1

Answer 18

Proliferation (Vascular smooth muscle and fibroblast)
Vasoconstriction
Hypertrophy (cardiac/vascular)
Inflammation
Fibrosis (fibroblast proliferation, extracellular matrix proteins, collagenase production)

Question 19

Bosentan pregnancy risk

Answer 19

Category X

Question 20

Ambrisentant pregnancy risk

Answer 20

Category X

Question 21

Which has higher hepatotoxicity: Bosentan or Ambrisentan

Answer 21

Bosentan 11% (vs amrbisentan 1%)

Question 22

Which drug has NOT been shown to benefit WHO-E group 2 patients?

Answer 22

Bosentan

Question 23

Epoprostenol brand name

Answer 23

Flolan

Question 24

Epoprostenol Drug Calss

Answer 24

Prostacyclin analog

Question 25

Epoprostenol dosing

Answer 25

IV ONLY 3ng/kg/mg Target: 30ng/kg/mg Titrate up every hour, down q15min for side effects Half life 3min

Question 26

Treprostinil brand name (IV/SC)

Answer 26

Remodulin

Question 27

Treprostinil brand name (inhaled)

Answer 27

Tyvaso

Question 28

Remodulin generic name

Answer 28

Treprostinil (IV/SC)

Question 29

Tyvaso generic name

Answer 29

Treprostinil (inhaled)

Question 30

Treprostinil dosing

Answer 30

1.25ng/kg/min Target 12.5ng/kg/min Titrate: every 3 days, down every 1 day for side effects Half life: 20 min

Question 31

Conversion: 1 mg = ___ mcg = ____ ng

Answer 31

1000mcg = 1,000,000 ng

Question 32

Epoporstenol dose limiting effects

Answer 32

headache, musculoskeletal pain, hypotension, bradycardia, diarrhea, flushing

Question 33

Epoprostenol: both bottle and IV tubing must be changed every ____

Answer 33

8 hours

Question 34

Which drugs have NOT been shown to benefit WHO-E group 1 patients?

Answer 34

Prostacyclin analogs

Question 35

Give diltiazem when right heart cath and vasodilation resting with inhaled nitric oxide with ≥ __ decrease in mean PAP and ≤ ____

Answer 35

10mmHg decrease in mean PAP | ≤40mmHg

Question 36

HTN in your pulmonary artery when mean pulmonary artery pressure ≥ ___ at rest

Answer 36

25mmHg

Question 37

PAH often leads to _____-sided HF

Answer 37

Right sided

Question 38

WHO E group 1 def

Answer 38

``` Idiopathic (IPAH) -- genetic Associated with (APAH): collagen vascular disease (lupus), portal hypertension secondary to liver failure, HIV, drugs (stimulants) ```

Question 39

WHO E group 2 def

Answer 39

Left-HF | **most common cause of PAH

Question 40

WHO E Group 3 def

Answer 40

Associated with lung disease or chronic hypoxemia | COPD, ILD, ONA, high altitudes

Question 41

WHO E group 4 def

Answer 41

Chronic thromboembolic disease, pulmonary embolism

Question 42

WHO E Group 5

Answer 42

Unclear or multifactorial causes

Question 43

WHO F Class 1

Answer 43

No dyspnea or fatigue with usual physical activity

Question 44

WHO F Class II

Answer 44

SOB or fatigue when doing usual physical activity

Question 45

WHO F Class III

Answer 45

SOB or fatigue when doing simple activities

Question 46

WHO F Class IV

Answer 46

SOB/fatigue at rest

Question 47

PCWP > ____ means left sided heart failure likely

Answer 47

PCWP >15

Question 48

Normal 6MWT results in healthy elderly adults is ___

Answer 48

600m