44. Pulmonary Arterial Hypertension (PAH) & Pulmonary Fibrosis (PF) Flashcards
Which of the following are common side effects for epoprostenol? (Select ALL that apply.)
A. Tachycardia and hypotension
B. Jaw pain
C. Flushing
D. Hepatotoxicity
E. Diarrhea, nausea, and vomiting
A, B, C, E. Tachycardia, hypotension, flushing, jaw pain, and N/V/D are all side effects of epoprostenol.
epoprostenol (Flolan, Veletri)
You receive a prescription for Revatio 20 mg TID. What is the generic name of Revatio?
A. Vardenafil
B. Tadalafil
C. Sildenafil
D. Avanfil
E. Ambrisentan
C. The generic name for Revatio is sildenafil.
sildenafil (Revatio)
tadalafil (Adcirca)
A 48-year-old HIV-positive man presents to the ID clinic complaining of dyspnea upon exertion, chest pain, fatigue, and lower extremity edema. His past medical history is significant for coronary artery disease, diabetes, hypertension, depression, prior cocaine and tobacco abuse, and intermittent transaminitis. His medications include aspirin, metoprolol, lisinopril, simvastatin, isosorbide mononitrate, metformin, paroxetine, and protease-inhibitor based HAART. After cardiac work-up, myocardial infarction is ruled out. However, his mean pulmonary artery pressure is 54 mmHg. His physician orders warfarin and oxygen for his pulmonary hypertension and consults you regarding therapy. What do you recommend?
A. Riociguat 0.5 mg PO TID
B. Sildenafil 20 mg PO TID
C. Verapamil XR 180 mg PO daily
D. Bosentan 62.5 mg PO BID
E. Epoprostenol 2 ng/kg/minute
E. Riociguat and sildenafil should be avoided in patients receiving nitrates. The patient is also receiving a protease inhibitor, which should be avoided with sildenafil. Verapamil is not recommended in PAH due to its negative inotropic effects. Given the patient’s transaminitis, bosentan is a poor choice. Overall, epoprostenol is the best option.
A 210-lb man receives Flolan 74 mL/day. His cassette is made with three Flolan 1.5 mg vials and diluted to a total volume of 100 mL. What is his dose in ng/kg/min?
A. 24 ng/kg/min
B. 37 ng/kg/min
C. 59 ng/kg/min
D. 102 ng/kg/min
E. 156 ng/kg/min
A. 210 lb is 95.5 kg [74 mL/day x 4.5 mg/100 mL x 1000 mcg/1 mg x 1000 ng/1 mcg x 1 day/24 h x 1 h/60 min]/95.5 kg = 24 ng/kg/min
What non-pharmacologic treatment options should be recommended in patients with pulmonary arterial hypertension (PAH)? (Select ALL that apply).
A. Sodium restriction (< 2.4 grams/day)
B. Avoiding exposure to high altitudes
C. Receive routine immunization against influenza
D. Receive routine immunization against pneumonia
E. Maintaining a constant fluid volume status
A, B, C, D, E. Sodium restriction, avoiding high altitudes, maintaining a constant fluid volume status are all important treatments for patients with PAH.
A doctor wants to know options for IV prostacyclin treatment for PAH. Which of the following is an IV prostacyclin?
A. Remodulin
B. Tyvaso
C. Ventavis
D. Letairis
E. Adcirca
A. Remodulin is IV treprostinil, the others are either inhaled or oral fomulations.
epoprostenol (Flolan, Veletri): IV
treprostinil: Remodulin (IV/SQ), Tyvaso (inhaled)
iloprost (Ventavis): inhaled
A doctor writes a prescription for Adcirca 40 mg once daily. The pharmacist should:
A. Fill the prescription with vardenafil.
B. Counsel the patient that side effects could include dizziness, headache, drop in blood pressure and priapism
C. Ensure that the patient is not currently taking any calcium channel antagonists.
D. Ensure that the patient is enrolled in the Adcirca Education and Access Program.
E. Fill the prescription with sildenafil.
B. The endothelin receptor antagonists require enrollment in special programs to receive the medications (REMS), not the PDE-5 inhibitors.
Adcirca (tadalafil)
Revatio (sildenafil)
Which statement is true regarding the pulmonary fibrosis drugs pirfenidone and nintedanib?
A. Nintedanib dose must be titrated
B. Nintedanib is a tyrosine kinase inhibitor
C. Pirfenidone and nintedanib must be taken on an empty stomach
D. Nintedanib can cause photosensitivity reactions
E. Pirfenidone can cause myocardial infarctions
B.
Both needs to be taken with food. Only pirfenidone (Esbriet) needs to be titrated and can cause photosensitivity. Nintendanib (Ofev) does not need titration or cause photosenitivity.
Which of following is true regarding epoprostenol?
A. It is a prostacyclin (PGI2) analogue which is a potent vasodilator.
B. It comes in an IV and inhaled formulation.
C. It has a boxed warning for progressive multifocal leukoencephalopathy.
D. The brand name is Tyvaso.
E. It has a boxed warning for cardiotoxicity.
A. Epoprostenol comes in an intravenous formulation only. There are no boxed warnings for epoprostenol use.
A doctor writes a prescription for Revatio. Upon further evaluation, the pharmacist notes the patient’s medication list which includes: acetaminophen 650 mg Q6H PRN mild pain, Norvasc 10 mg daily, calcium carbonate 500 mg BID, Cardura 1 mg daily, and nitroglycerin 0.2 mg patch daily. Which of the following are potential drug-drug interactions? (Select ALL that apply.)
A. Revatio and acetaminophen
B. Revatio and calcium carbonate
C. Revatio and Norvasc
D. Revatio and nitroglycerin patch
E. Revatio and Cardura
C, D, E. The drug-drug interactions include Revatio and Norvasc (decreased blood pressure), Revatio and nitroglycerin patch (decreased blood pressure) and Revatio and Cardura (decreased blood pressure).
A pregnant woman needs treatment for PAH. Which of the following are not Pregnancy Category X? (Select ALL that apply.)
A. Ambrisentan
B. Epoprostenol
C. Macitentan
D. Riociguat
E. Tadalafil
B, E. The endothelial receptor antagonists and the soluble guanylate cyclase stimulating agent riociguat are labeled as Pregnancy Category X.
Which of the following are true regarding Adempas? (Select ALL that apply.)
A. Unlike tadalafil, Adempas can be safely given with nitrates
B. It should not be administered during pregnancy
C. Common side effects include anemia and liver enzyme elevations
D. Doses may need to be increased in smokers
E. It increases the risk of thromboembolism
B, D. Riociguat should be avoided with nitrates due to the risk of hypotension. It is pregnancy category X. Side effects are predominately vasodilatory; it may be associated with bleeding. If a patient stops smoking, the riociguat dose may need to be decreased.
Which of the following is/are correct in terms of matching the pulmonary vasodilator medications to their mechanisms of action? (Select ALL that apply.)
A. Revatio increases cGMP levels
B. Adempas decreases cGMP levels
C. Letairis stimulates endothelin receptors
D. Ventavis increases cAMP levels
E. Adcirca decreases cAMP levels
A, D. PDE-5 inhibitors and soluble guanylate cyclase stimulators ultimately increase cGMP levels to cause pulmonary artery vasodilation. Prostacyclins increase cAMP to achieve the same. Antagonism of endothelin receptors causes pulmonary artery vasodilation.
Revatio (sildenafil)
Adempas (riociguat)
Letairis (ambrisentan)
Ventavis (iloprost)
Adcirca (tadalafil)
A patient is admitted on Veletri. The pharmacist should ensure which of the following?
A. It is administered via SC injection
B. The patient receives ibuprofen 800 mg TID concomitantly.
C. The patient avoids large amounts of protein in their diet.
D. Large, sudden reductions in dose should be avoided.
E. The patient has ice packs for proper cooling of Veletri.
D. Veletri is the heat stabilized formulation of epoprostenol so there is no need for ice packs. There are no food interactions with Veletri.
epoprostenol (Flolan, Veletri)
Which of the following is true regarding treprostinil?
A. It has a 5 minute half-life
B. Subuctaneous administration can cause significant pain at the infusion site
C. It should be kept on ice packs during infusion
D. Inhalational treprostinil is delivered 6 to 9 times daily
E. The brand name is Ventavis
B. Subcutaneous treprostinil is associated with pain at both old and current administration sites in a majority of patients.
treprostinil: Remodulin (IV/SQ), Tyvaso (inhaled)
