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OU Practice Spring 2018 > Pulmonary Arterial Hypertension > Flashcards

Pulmonary Arterial Hypertension Flashcards

1
Q

Riociguat

A
  • Adempas®
  • therapeutic calss: sGC stimulator; sensitize NO to cause vasodilation
  • tablet
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2
Q

Riociguat adverse effects

A
  • Fetal harm
  • Hypotension
  • Headache
  • Dizziness
  • Dyspepsia
  • Bleeding
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3
Q

Riociguat dosing

A
  • 1 mg PO three times daily; may initiate dose at 0.5 mg PO three times daily who become intolerable to hypotension
  • If SBP remains >95 mm Hg and the patient has no signs or symptoms of hypotension, increase the dose by 0.5 mg PO three times daily at intervals of ≥2 weeks to the highest tolerated dosage.
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4
Q

Riociguat Clinical Pearls/Counseling Points

A
  • Restricted through REMS program in females
  • Metabolized via BCRP, CYP2C8/3A4; eliminated via P-gp
  • Dose adjustment in: smokers & patients with strong multi-pathway CYP and P-gp/BCRP inhibitors
  • Contraindicated: pregnancy; co-administration of nitrates, specific/non-specific PDE5 inhibitors
  • No dose adjustments provided for CrCl ≥ 15 mL/min or mild-moderate hepatic impairment
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5
Q

PDE5 Inhibitors

A
  • NO -> guanylate cyclase -> ↑cGMP = vasodilation
  • Sildenafil Citrate (Revatio®)
  • Tadalafil (Adcirca®)
  • Vardenafil (not approved for treatment of PAH)
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6
Q

Sildenafil Citrate (Revatio®) dosing

A

5 - 40 mg PO three times daily (240 mg/day)

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7
Q

Sildenafil Citrate (Revatio®) Clinical Pearls/Counseling Points

A
  • Primarily metabolized via CYP 3A4
  • Avoid grapefruit juice
  • Contraindicated in patients concomitantly receiving nitrates or riociguat; use with protease inhibitors
  • Priapism
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8
Q

Sildenafil Citrate (Revatio®) adverse effects

A
  • Flushing
  • Headache
  • Hypotension*
  • Diarrhea
  • Visual disturbances
  • Reports of hearing loss
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9
Q

Tadalafil (Adcirca®) dosing

A

40 mg once daily (40 mg)

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10
Q

Tadalafil (Adcirca®) Clinical Pearls/Counseling Points

A
  • Metabolized via CYP 3A4
  • Avoid grapefruit juice
  • Contraindicated in patients concomitantly receiving nitrates or riociguat; use with protease inhibitors
  • Priapism
  • Dose adjust with ritonavir
  • CrCl 31-80 mL/min: initiate 20 mg once daily
  • Mild-moderate hepatic impairment: initiate 20 mg once daily
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11
Q

Tadalafil (Adcirca®) adverse effects

A
  • Flushing
  • Headache
  • Hypotension*
  • Nausea
  • Myalgia
  • Visual disturbances
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12
Q

Endothelin Receptor Antagonists (ERAs)

A
  • Competitively inhibit endothelin-1 receptors
  • ETa located primarily in pulmonary vascular smooth muscle and is responsible for vasoconstriction; difficult to target this only
  • Ambrisentan (Letairis®)
  • Bosentan (Tracleer®)
  • Macitentan (Opsumit®)
  • all FDA approved for PAH and all restricted through REMS
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13
Q

Why are the ERA’s restricted via REMS?

A
  • all cause birth defects

- Bosentan causes hepatotoxicity

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14
Q

ERA’s agents’ affinity for receptors

A
  • Ambrisentan more affinity for ETa but associated with more adverse effects
  • Bosentan more affinity for A but dual antagonism
  • Macitentan more affinity for A but dual antagonism but tissue selective
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15
Q

Endothelin Receptor Antagonists (ERAs) adverse effects

A 
▪ Headache 
▪ Anemia 
▪ Fluid retention/peripheral edema 
▪ Respiratory complications 
▪ Decrease in spermatogenesis 
▪ Hepatic impairment 
▪ Flushing & dyspepsia 
▪ Ambrisentan
▪ ALL are contraindicated in pregnancy!!
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16
Q

Ambrisentan (Letairis®) dosing

A

5 mg once daily (10 mg/day)

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17
Q

Ambrisentan (Letairis®) Clinical Pearls/Counseling Points

A
  • Minor metabolism via CYP2C19/3A4; eliminated via P-gp
  • Do not exceed 5 mg/day if using concomitantly with cyclosporine
  • Dose adjustment in hepatic impairment that develops during therapy
  • Do not crush, split, or chew tablets
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18
Q

Bosentan (Tracleer®) dosing

A

<40 kg: Initial and maintenance: 62.5 mg twice daily ≥40 kg: Initial: 62.5 mg twice daily for 4 weeks; increase to maintenance dose of 125 mg twice daily

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19
Q

Bosentan (Tracleer®) Clinical Pearls/Counseling Points

A
  • Minor metabolism via CYP 2C9/3A4; induces CYP2C9 (weak/moderate)/3A4 (moderate)
  • Avoid grapefruit juice
  • Dose adjust in patients currently receiving ritonavir
  • Dose adjust in patients that develop hepatic dysfunction during treatment & in patients with pre-existing moderate-severe hepatic impairment
  • Contraindicated: concurrent use of cyclosporine or glyburide
20
Q

Macitentan (Opsumit®) dosing

A

10 mg once daily (10 mg/day)

21
Q

Macitentan (Opsumit®) Clinical Pearls/Counseling Points

A
  • Metabolized by CYP3A4

* Do not crush, split, or chew tablets

22
Q

Prostacyclin Analogs

A

▪ Vasodilate pulmonary arteries
▪ Impair platelet aggregation
▪ Anti-proliferative effect on pulmonary endothelial cells
▪ All agents are FDA-approved for the treatment of PAH
▪ IV and SQ formulas

23
Q

Prostacyclin Analogs drugs and dosage forms

A 
▪ Intravenous (IV) 
- Epoprostenol (Flolan®, Veletri®) 
- Treprostonil (Remodulin®)
▪ Subcutaneous (SQ) 
- Treprostonil (Remodulin®)
▪ Inhaled 
- Treprostonil (Tyvaso®) 
- Iloprost (Ventavis®)
▪ Oral 
- Treprostonil (Orenitram®)
24
Q

Epoprostenol (Flolan®, Veletri®) dosing

A
  • Delivered by continuous IV infusion
  • Started in the hospital at 2 ng/kg/min via a central venous catheter
  • Dose adjusted based on PAH symptoms and tolerability to side effects
  • Maintenance dose: 25-40 ng/kg/min
25
Q

Epoprostenol (Flolan®, Veletri®) clinical pearls

A
  • Special ambulatory infusion pumps
  • Veletri® is stable at room temperature
  • Flolan® must be kept on ice packs
  • Drug only available via two distributors
  • Use with caution in patients with other risk factors for bleeding
  • Chronic continuous IV infusion of epoprostenol via a chronic indwelling central venous catheter has been associated with local infections and serious blood stream infections
  • Discontinue and do not re-administer if patient develops pulmonary edema on epoprostenol
26
Q

Epoprostenol (Flolan®, Veletri®) adverse effects

A 
▪ Flushing 
▪ Headache 
▪ Nausea/vomiting 
▪ Hypotension 
▪ Anxiety/agitation 
▪ Chest pain 
▪ Jaw pain
▪ Contraindications - Heart failure due to severe left ventricular dysfunction
27
Q

Treprostonil (Remodulin®) SQ dosing

A
  • Administered via an ambulatory pump
  • 1.25 ng/kg/minute
  • Can decrease to 0.625 ng/kg/min if experience adverse effects
  • Can be administered up to 72 hours at > 98 degrees Fahrenheit
28
Q

Treprostonil (Remodulin®) IV dosing

A
  • Infused via a central venous catheter use an infusion pump
  • 1.25 ng/kg/minute
  • Can decrease to 0.625 ng/kg/min if experience adverse effects
  • Can be administered up to 48 hours at > 98 degrees Fahrenheit
  • Significantly greater increased risk of catheter-related gram (-) bloodstream infections compared to epoprostenol
29
Q

Treprostonil (Remodulin®) adverse effects

A
  • Infusion site pain/reaction in ~85% of patients
  • Headache*
  • Nausea/diarrhea*
  • Jaw pain*
  • Rash
30
Q

Treprostonil (Tyvaso®) inhaled dosing

A
  • 18 mcg (or 3 inhalations) every 4 hours 4 times/day
  • If tolerated, increase dose by an additional 3 inhalations at approximately 1to 2-week intervals; target dose and maximum dose: 54 mcg (or 9 inhalations) 4 times/day
  • Safety and efficacy have not been established in patients with underlying pulmonary disease (e.g., asthma, COPD).
31
Q

Treprostonil (Tyvaso®) adverse effects

A
  • Headache*
  • Dizziness*
  • Nausea/diarrhea*
  • Flushing*
  • Cough/throat irritation
32
Q

Treprostonil (Tyvaso®) clinical pearls

A

▪ Avoid abrupt discontinuation
▪ Metabolized through CYP2C8
▪ Concomitant diuretics, antihypertensives or other vasodilators may increase the risk of systemic hypotension.
▪ May inhibit platelet aggregation and increases the risk of bleeding
▪ IV/SQ: available via prescribing access restrictions
- dose adjust in hepatic impairment: 0.625 ng/kg/minute (ideal body weight)

33
Q

Iloprost (Ventavis®) dosing

A
  • 2.5 mcg inhaled specific inhalation system
  • If tolerated increase to 5 mcg inhaled 6-9 times per day (no more than q. 2 hours {45 mcg}) during waking hours
  • Do not initiate therapy if SBP < 85 mmHg
34
Q

Iloprost (Ventavis®) adverse effects

A 
▪ Cough 
▪ Influenza-like symptoms 
▪ Nausea* 
▪ Headache* 
▪ Hypotension* 
▪ syncope
▪ Flushing* 
▪ Jaw pain*
35
Q

Iloprost (Ventavis®) clinical pearls

A

▪ Do not initiate therapy is SBP < 85 mmHg
▪ Maximum daily dose: 45 mcg (5 mcg/dose 9 times daily)
▪ Safety and efficacy have not been established in patients with other concurrent pulmonary diseases (COPD, severe asthma, or acute pulmonary infections): may induce bronchospasm in patients with hyper-reactive airways
▪ May inhibit platelet aggregation and increases the risk of bleeding
▪ Hepatic impairment: Consider increasing dosing interval (every 3-4 hours)

36
Q

Prostacyclin IP-receptor agonists: what is an IP receptor?

A

▪ One of 4 different types of prostanoid receptors found in the lungs
▪ Regulates vascular tone, platelet activation, and immunologic cell responses
▪ Expressed in vascular smooth muscle cells (SMC) and platelets
▪ Activation of the IP receptor triggers SMC vasodilation and inhibition of SMC proliferation and platelet aggregation

37
Q

Prostacyclin IP-receptor agonist drugs

A
  • Selexipag (Uptravi®)

▪ Oral, non-prostanoid, selective agonist of the prostacyclin IP receptor

38
Q

Selexipag (Uptravi®) dosing

A

Initial: 200 mcg PO twice daily; increase by 200 mcg PO twice daily, usually at weekly intervals, to the highest tolerated dose (1,600 mcg twice daily)

39
Q

Selexipag (Uptravi®) Clinical Pearls/Counseling Points

A
  • Major substrate of BCRP & CPY 2C8
  • Take with food
  • Avoid use in severe hepatic impairment
  • Discontinue if patient develops pulmonary edema
40
Q

Selexipag (Uptravi®) adverse effects

A
  • Flushing
  • Headache
  • GI (n/v/d)
  • Myalgia
  • Jaw pain
  • Pulmonary edema
41
Q

What is the role of the pharmacist in the care of patients with pulmonary arterial hypertension (PAH)?

A 
▪ Formulary management 
▪ Medication Safety 
▪ Regulatory Compliance (REMS)
▪ Patient/Provider Education 
▪ Medication Access (only available via 2 carriers) 
▪ Adverse Effect Management
42
Q

What is the endorsing organization for guidelines to treat PAH?

A

Journal of the American College of Cardiology (JACC)

43
Q

Which medications are restricted via REMS?

A
  • Riociguat (Adempas®)
  • Ambrisentan (Letairis®)
  • Bosentan (Tracleer®)
  • Macitentan (Opsumit®)
44
Q

Which aspects of drug administration can pharmacy intervene on?

A

▪ Accidental bolus dosing
▪ Incorrect dosing
▪ Pump-related errors
▪ these are epoprostenol-related errors

45
Q

Drugs or toxins that may cause PAH

A 
▪ Cocaine 
▪ Phenylpropanolamine 
▪ St. John’s Wort 
▪ SSRIs in newborns 
▪ Amphetamine-like drugs 
▪ Interferon alpha &amp; beta
46
Q

General Considerations in Patients with PAH

A

▪ Avoid heavy lifting: Can cause syncope
▪ Participate in low level graded aerobic activity to improve peak oxygen consumption
▪ Avoid high altitudes: May need supplemental O2 on aircraft
▪ Salt restriction to < 2,400 mg/day: don’t want to increase preload on L ventricle that is already failing
▪ Women should avoid pregnancy: 60% mortality in pregnant women
▪ Immunizations: Influenza, Pneumococcal

OU Practice Spring 2018 (9 decks)

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