44. Pulmonary Arterial Hypertension (PAH) & Pulmonary Fibrosis (PF) Flashcards Preview

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Flashcards in 44. Pulmonary Arterial Hypertension (PAH) & Pulmonary Fibrosis (PF) Deck (23):

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


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)


Which of the following are correct patient counseling points for a patient just starting Flolan? (Select ALL that apply.)

A. This product does not require cooling packs for the pump.
B. You will need a back up pump and extra supplies to prevent interruptions in therapy.
C. Early side effects may include hallucinations, delirium, and loss of consciousness.
D. It is important to keep your catheter clean to reduce infections.
E. This medication can help to cure your disease.

B, D. Flolan needs to be given by a central catheter and proper maintenance of this catheter is essential to prevent infections, bleeding and clotting. Avoid large or sudden interruptions in therapy as this can be fatal.

Flolan (epoprostenol)


Which of the following is true regarding pulmonary hypertension?

A. Patients with pulmonary hypertension related to COPD are appropriate iloprost candidates
B. Riociguat is an approved treatment for chronic thromboembolic pulmonary hypertension
C. Patients with pulmonary hypertension due to systolic heart failure are often treated with eopprostenol
D. Bosentan is the drug of choice for PAH in pregnancy
E. All prostacyclin analogues require a Med Guide

B. Generally, pulmonary hypertension that is not PAH should have treatment directed at the underlying cause.  Riociguat is the only medication approved for PAH and another class of pulmonary hypertension (chronic thromboembolic pulmonary hypertension).


A doctor writes a prescription for Tracleer 125 mg BID. Which of the following statements are true regarding Tracleer? (Select ALL that apply.)

A. The generic name is bosentan
B. It is Pregnancy Category X
C. It is a prostacyclin analogue
D. It has a black box warning for hepatotoxicity
E. A common side effect is anemia

A, B, D, E. Bosentan (Tracleer) is Pregnancy Category X and can cause hepatotoxicity and anemia.


LA is a 37 year old Caucasian female who was diagnosed with pulmonary arterial hypertension (PAH) three years ago. She is anticoagulated with warfarin 3 mg each evening (INR 2.3) and is also on amlodipine 10 mg daily. She is admitted to the hospital for worsening PAH symptoms. Which of the following could be potential causes of LA's PAH? (Select ALL that apply.)

A. History of cocaine use
B. History of taking dasatinib
C. History of methamphetamine use
D. History of taking treprostinil
E. History of taking bosentan

A, B, C. Treprostinil and bosentan are treatments for PAH, not causes.


A patient receives Remodulin 96 ng/kg/min via continuous subcutaneous infusion with a 60 kg dosing weight.  She uses the 10 mg/mL concentration to fill her syringe.  How many milliliters per hour should her pump be programmed to deliver?

A. 0.005 mL/hr
B. 0.018 mL/hr
C. 0.026 mL/hr
D. 0.035 mL/hr
E. Inadequate information is available to calculate this

D. 60 kg x 96 ng/kg/min x 1 mcg/1000 ng x 1 mg/1000 mcg x mL/10 mg x 60 min/hr = 0.035 mL/hr


An 80 kg patient is receiving epoprostenol at a rate of 76.8 mL/day. The concentration is 60,000 nanograms/mL. What is the dose in nanograms/kg/min?

A. 0.4
B. 4
C. 40
D. 400
E. The dose cannot be calculated from the given information.

C. 60,000 ng/mL X 76.8 mL/day = 4,608,000 ng/day X 1 day/24 hr X 1 hr/60 min = 3,200 ng/min / 80 kg = 40 ng/kg/min


JC, a 34 year old pregnant woman, complains of bloating, nausea, heartburn and a non productive cough. She is diagnosed with idiopathic pulmonary fibrosis. Which drug therapy is FDA indicated to treat pulmonary fibrosis and appropriate for JC?

A. Prednisone
B. Azathioprine
C. Pirfenidone
D. Cyclophosphamide
E. Nintedanib

C. Nintedanib is Pregnancy Category D, patients should avoid becoming pregnant during therapy and 3 months after use.


Which of the following would be the least appropriate to monitor in a patient on bosentan?

A. Pregnancy test
B. Liver function tests
C. Serum creatinine
D. Hemoglobin and hematocrit
E. Drug interactions in the CYP450 enzyme system

C. Women need negative monthly pregnancy tests to continue to receive bosentan.  The drug can cause hepatotoxicity and anemia. Due to bosentan’s metabolism via and induction of CYP 3A4, the potential for numerous drug interactions exist.

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