PAH

Question 1

Drugs that Cause PAH

Answer 1

• Cocaine
• SSRI use in pregnancy (increased PPHN risk)
• Weight loss drugs (diethylpropion, lorcaseron, phendimetrazine, phentermine)
• Meth/Amphetamines

Question 2

Vasoconstrictor Substances

Answer 2

• Endothelin-1
• Thomboxane A2 (TXA2)

Increased in PAH

Question 3

Vasodilator Substances

Answer 3

-Prostacyclins

Decreased in PAH

Question 4

Non-Rx Recommendations

Answer 4

• Restrict Na <2.4 g/d (helps manage volume)
• Avoid medications like NSAIDs (increase Na/water retention)
• Recommend routine immunizations like flu/pneumonia

Question 5

PAH Diagnosis

Answer 5

• Right-heart catheterization to confirm pressure level is needed (PAP >= 25), must have normal fluid status
• Vasoreactivity test then done to determine responsiveness to CCB (drops more than 10 mmHg and under 40 mmHg overall)

Question 6

Responder Therapy

Answer 6

-Oral CCB
-Usually long-acting nifedipine, diltiazem, or amlodipine
NO VERAPAMIL (CI) - NEGATIVE INOTROPE

Question 7

Non-Responder Therapy Options

Answer 7

More potent vasodilating drugs

• Prostacyclin analogs/receptor agonists: IV epoprostenol (decreases mortality)
• ERAs (endothelin antagonists)
• PDE-5i
• Soluble guanylate cyclase stimulator

Question 8

PAH Supportive Therapy

Answer 8

• Loop diuretic (volume control)
• Digoxin (improve cardiac output/HR)
• *-Warfarin (due to prothrombotic state) - goal INR 1.5-2.5 (Note: lower than normal)**

Question 9

Flolan

Answer 9

• Epoprostenol
• IV prostacyclin analog
• Continuous IV infusion
• Dose: 2 ng/kg/min

Question 10

Treprostinil

Answer 10

• Another prostacyclin analog
• IV, SC, inh, and oral formulations
• IV/SQ dosing: 1.25 ng/kg/min (continuous)

Question 11

Prostacyclin Analog Information

Answer 11

• Warnings: vasodilation rxn (flushing, hypotension), rebound PH (stopping/dose decrease), bloodstream infections with continuous IV infusions
• SE: hypotension, flushing, jaw pain, infusion-site pain (IV/SQ)
• Epoprostenol must be protected from light, even during infusion
• Flolan specifically must also have ice packs for stability once reconstituted

Question 12

Tracleer

Answer 12

• Bosentan
• Endothelin Receptor Antagonist
• Box warning: hepatotoxic!

Question 13

ERAs Information

Answer 13

• Box Warning: teratogenic (negative preggo test at start/QMo), REMS program needed
• CI: preggo
• Warning: hepatotoxicity, decreases in Hgb/Hct, fluid retention
• SE: HA + vasodilation SE

Question 14

Revatio

Answer 14

• Sildenafil

- Viagra for ED

Question 15

Adcirca

Answer 15

• Tadalafil

- Cialis for ED/BPH

Question 16

PDE5i Information

Answer 16

• CI: Use with nitrates
• Warning: Hearing loss, vision loss, NAION, hypotension, priapism
• SE: HA + vasodilation SE

Question 17

sGC Stimulator Information

Answer 17

• Box Warning: teratogenic (negative preggo test at start/Qmo), REMS program needed
• CI: preggo, use with PDE5i or nitrates
• Warning: hypotension
• SE: HA

Question 18

Drugs that Cause Pulmonary Fibrosis

Answer 18

• Amiodarone/dronedarone
• Bleomycin
• Busulfan
• Carmustine
• Lomustine

Question 19

Letairis

Answer 19

• Ambrisentan

- Endothelin Receptor Antagonist

Question 20

Opsumit

Answer 20

• Macitentan

- Endothelin Receptor Antagonist

Question 21

Adempas

Answer 21

• Soluble guanylate cyclase
• Riociguat
• TONS of DDI