Pulmonary Arterial Hypertension Flashcards

Question

Epoprostenol (Flolan®, Veletri®) clinical pearls

Answer 1

- 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

Answer 2

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

Answer 3

- 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

Answer 4

- 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

Answer 5

- Infusion site pain/reaction in ~85% of patients - Headache* - Nausea/diarrhea* - Jaw pain* - Rash

Answer 6

- 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).

Answer 7

- Headache* - Dizziness* - Nausea/diarrhea* - Flushing* - Cough/throat irritation

Answer 8

▪ 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)

Answer 9

- 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

Answer 10

``` ▪ Cough ▪ Influenza-like symptoms ▪ Nausea* ▪ Headache* ▪ Hypotension* ▪ syncope ▪ Flushing* ▪ Jaw pain* ```

Answer 11

▪ 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)

Answer 12

▪ 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

Answer 13

- Selexipag (Uptravi®) | ▪ Oral, non-prostanoid, selective agonist of the prostacyclin IP receptor

Answer 14

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)

Answer 15

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

Answer 16

* Flushing * Headache * GI (n/v/d) * Myalgia * Jaw pain * Pulmonary edema

Answer 17

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

Answer 18

Journal of the American College of Cardiology (JACC)

Answer 19

- Riociguat (Adempas®) - Ambrisentan (Letairis®) - Bosentan (Tracleer®) - Macitentan (Opsumit®)

Answer 20

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

Answer 21

``` ▪ Cocaine ▪ Phenylpropanolamine ▪ St. John’s Wort ▪ SSRIs in newborns ▪ Amphetamine-like drugs ▪ Interferon alpha & beta ```

Answer 22

▪ 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

Pulmonary Arterial Hypertension Flashcards

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