Chapter 40: Pulmonary Arterial Hypertension

Question 1

What are common drugs that can cause PAH

Answer 1

• Cocaine
• Methamphetamines/amphetamines
• SSRI use during pregnancy ↑ risk of persistent PH of a newborn
• Weight loss drugs (diethylpropion, lorcaserin, phendimetrazine, phentermine)

Question 2

PAH stems from an imbalance in vasoconstrictor & vasodilator substances. Vasoconstrictor substances (e.g., ________) are increased and vasodilating substances (e.g., ______) are decreased.

Answer 2

endothelin-1 and TXA2

prostacyclins

Question 3

_____ is the most common cause of death in people who have PAH

Answer 3

Heart failure

Question 4

Symptoms of PAH include:

Answer 4

fatigue, dyspnea, chest pain, syncope, edema, tachycardia, and/or Raynaud’s phenomenon

Question 5

What are some non-drug treatments of PAH

Answer 5

Sodium-restricted diet of < 2.4 grams/day to help manage volume status

NSAIDs, which increase Na and water retention, should be avoided

Question 6

Routine immunizations against _____ and _____ are advised

Answer 6

influenza, pneumococcal pneumonia

Question 7

______ is performed to confirm the diagnosis of PAH

Answer 7

Right heart catheterization

Question 8

If the mPAP falls by at least 10 mmHg to an absolute value less than 40 mmHg during vasoreactivity testing, the patient is considered a ____ and should be initially treated with an oral ____.

Answer 8

responder; CCB (long-acting nifedipine, diltiazem, and amlodipine). Verapamil is not recommended

Question 9

Non-responders to vasoreactivity testing and positive responders who fail CCB therapy need to be treated with _________

Answer 9

prostacyclin analogues and receptor agonists, endothelin receptor antagonists (ERAs), PDE-5 inhibitors, and/or a soluble guanylate cyclase (sGC) stimulator

Question 10

Which parenteral prostacyclin analogue has been shown to decrease mortality in PAH

Answer 10

IV esoprostenol

Question 11

Supportive therapies (similar to systolic HF) include ____ and _____

Answer 11

loop diuretics (for volume overload); digoxin (to improve CO or control HR in AFib)

Question 12

Biochemical changes (↑ TXA2, ↓ prostacyclin) lead to a pro-thrombotic state and increased risk of blood clots. If warfarin is used for PAH, it should be titrated to a goal INR of:

Answer 12

1.5-2.5

Question 13

What is the MOA of prostacyclin analogues (prostanoids)

Answer 13

They are potent vasodilators and inhibitors of platelet aggregation

Question 14

How are epoprostenol and treprostinil administered?

Answer 14

continuous IV infusion at home

Question 15

What units are epoprostenol and treprostinil dosed in?

Answer 15

ng/kg/min

Question 16

What are the main warnings of prostaglandin analogues

Answer 16

• vasodilation reactions (hypotension, flushing)
• rebound PH
• Chronic IV influsions: sepsis and blood stream infections

Question 17

What are the main side effects of prostaglandin analogues

Answer 17

Hypotension
flushing
jaw pain
infusion-site pain with IV/SC infusions

Question 18

What is the brand name for epoprostenol

Answer 18

Flolan

Question 19

Since parenteral prostaglandin analogues are very potent vasodilators, what should be avoided

Answer 19

interruptions and sudden, large reductions in dose

Question 20

Which prostaglandin analogue should be protected from light before reconstitution and during infusion

Answer 20

Epoprostenol

Question 21

Reconstituted formulations of Flolan require use of ___ for stability

Answer 21

ice packs

Question 22

Endothelin receptor antagonists such as bosentan, ambrisentan, and macitentan have a boxed warning for:

Answer 22

Teratogenic; women of childbearing potential must have a negative pregnancy test prior to initiation of therapy and monthly thereafter. All are only available through a REMS program

Question 23

Bosentan has a boxed warning of

Answer 23

hepatotoxicity

Question 24

Endothelin receptor antagonists are CI in

Answer 24

pregnancy

Question 25

What are the main warnings of Endothelin receptor antagonists

Answer 25

hepatotoxicity, ↓ Hgb/Hct, fluid retention

Question 26

Main side effect of Endothelin receptor antagonists

Answer 26

headache

Question 27

Bosentan can decrease the effectiveness of which drug class

Answer 27

hormonal contraceptives

Question 28

What is the MOA of PDE5-i

Answer 28

Blocks the degradation of cGMP. Increased cGMP concentrations lead to pulmonary vasculature relaxation and vasodilation

Question 29

What is the brand name for sildenafil in PAH

Answer 29

Revatio

Question 30

What is the brand name for tadalafil in PAH

Answer 30

Adcirca

Question 31

What drug class is CI with PDE5-i

Answer 31

Use with nitrates

Question 32

What are the main warnings for PDE5-i

Answer 32

Hearing loss, vision loss (NAION), hypotension, priapism

Question 33

What is the main SE of PDE5-i

Answer 33

Headache

Question 34

What is the MOA of the soluble guanylate cyclase, Riociguat

Answer 34

sensitizes sGC to endogenous nitric oxide and directly stimulates the receptor at a different binding site. This increases cGMP, leading to relaxation and antiproliferative effects in the pulmonary artery smooth muscle cells

Question 35

What is a boxed warning for Riociguat (Adempas)

Answer 35

Teratogenic; women of childbearing potential must have a negative pregnancy test prior to initiation of therapy and monthly thereafter. Only available through a REMS program

Question 36

What is a CI for riociguat

Answer 36

Pregnancy, use of a PDE5-i or nitrates

Question 37

What is the main warning for Riociguat

Answer 37

Hypotension

Question 38

What is the main SE for Riociguat

Answer 38

Headache

Question 39

The common presentation for pulmonary fibrosis is

Answer 39

external dyspnea with a non-productive cough

Question 40

Common drugs that can cause pulmonary fibrosis

Answer 40

Amiodarone/dronedarone
Bleomycin
Busulfan
Carmustine
Lomustine