Pulmonary Arterial Hypertension (PAH)

Question 1

What is PAH?

Answer 1

• Continuous high BP in the pulmonary arteries

Question 2

Key Drugs That Can Cause PAH

Answer 2

• Cocaine
• SSRI use during pregnancy
• Weight loss drugs (diethylpropion, lorcaserin, phendimetrazine, phentermine)
• Methamphetamines/Amphetamines

Question 3

Prostacyclin Analogues and Receptor Agonists

Answer 3

• Potent vasodilators and inhibitors of platelet aggregation
• Epoprostenol (Flolan)
• Continious IV infusion - start at 2 ng/kg/min
• Warnings: vasodilation reactions (hypotension, flushing), rebound PH, infections (due to chronic IV infusions)
• SEs: hypotension, flushing, jaw pain, infusion site pain
• These are very potent vasodilators; avoid interrupttions and sudden, large dose reductions
• Epoprostenol: protect from light before and during infusion
• Flolan: require ice packs for stability

Question 4

Endothelin Receptor Antagonists

Answer 4

• Bosentan (Tracleer)
• Ambrisertan (Letairis)
• Macitentan (Opsumit)
• BW:
  *teratogenic - negative pregnancy test prior and monthly)
  *Bosentan: hepatotixicity
  *REMS
• CIs: pregnancy
• Warnings: hepatotoxicity, decreases Hgb/Hct, fluid retention
• SEs: headache

Question 5

Endothelin Receptor Antagonists - DDIs

Answer 5

• Decrease the effectiveness of hormonal contraceptives

Question 6

Phosphodiesterase-5 Inhibitors

Answer 6

• Increases cGMP concentrations which leads to pulmonary vasculature relaxation and vasodilation
• Sildenafil (Revatio) (Viagra - ED)
• Tadalafil (Adcirca) (Cialis - ED)
• CIs: use with nitrates
• Warnings: hearing loss, vision loss (NAION), hypotension, priapism
• SEs: headache

Question 7

PDE-5 Inhibitors DDIs

Answer 7

• Do not use with nitrate medications or riociguat as low BP potential is increased

Question 8

sGC Stimulators

Answer 8

• sGC is a receptor for endogenous nitric oxide. Riociguat sensitizes sGC and directly stimulates the receptor. This increases cGMP, leading to relaxation
• BW: teratogenic - negative pregnancy test prior and monthly thereafter
• REMS program is required
• CIs: pregnancy, use of PDE-5 inhibitors and nitrates
• Warnings: hypotension
• SEs: headache

Question 9

Riociguat DDIs

Answer 9

• Do not use with nitrate medications due to low BP risk

Question 10

Pulmonary Fibrosis

Answer 10

• It is a scarred and damaged lung tissue
• Presents with dyspnea

Question 11

Key Drugs Than Can Cause Pulmonary Fibrosis

Answer 11

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