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NAPLEX 2024 > 40. Pulmonary Arterial Hypertension > Flashcards

40. Pulmonary Arterial Hypertension Flashcards

1
Q

Drug list that can cause pulmonary arterial HTN

A

Cocaine
Fenfluramine
Methamphetamine/amphetamine
SSRI use during pregnancy (increase risk of persistent pulmonary hypertension of a newborn (PPHN))
Weight-loss drugs (diethylpropion, phendimetrazine, phentermine)

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2
Q

Pulmonary HTN is characterized by mPAP ≥____ (normal range is ___)

A

≥25mmHg
Normal range 8-20 mmHg

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3
Q

PAH stems from an imbalance in vasoconstrictor and vasodilator substances. Vasoconstrictor substances (e.g. ____) are increased and vasodilating substances (e.g. ____) are decreased

A

vasoconstrictor = endotheline-1, TXA2
Vasodilator = prostacyclins

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4
Q

____ is the most common cause of death in pts who have PAH

A

HF

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5
Q

S/sx of PAH

A

Fatigue, dyspnea
Others: chest pain syncope edema, tachycardia and/or Raynaud’s phenomenon

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6
Q

T/F: There is no cure for PAH but there are treatment options and in some cases, a lung or heart transplant may be an option for younger patients

A

True

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7
Q

Non-drug treatments for PAH

A

Sodium restriction (<2.4 grams/day)
Avoid meds that increase sodium/water retention (NSAIDs)
Influenza and pneumococcal vaccines recommended

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8
Q

____ is performed to confirm the diagnosis of PAH

A

Right heart catheterization

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9
Q

During a right heart catheterization, what meds are administered for vasoreactivity testing?

A

short-acting vasodilators (e.g. inhaled nitric oxide, IV epoprostenol, IV adenosine)

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10
Q

What makes someone a responder during vasoreactivity testing? What treatment do you recommend for responders?

A

If mPAP falls by at least 10mmHg to an absolute value <40mmHg
Treatment: oral CCB

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11
Q

What are common CCBs used in responders?

A

Long-acting nifedipine, diltiazem, and amlodipine
Verapamil is NOT recommended d/t more pronounced negative inotropic effects compared to diltiazem

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12
Q

What are treatment options for non-responders or responders who failed CCBs?

A

Prostacyclin analogues and receptor agonists, endothelin receptor antagonists (ERAs), PDE-5i, and/or soluble guanylate cyclase (sGC) stimulator

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13
Q

Parenteral prostacyclin analogues, specifically ___, have shown to decrease mortality

A

IV epoprostenol

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14
Q

Supportive therapies for PAH may include ___ (for volume overload) and ___ (to improve cardiac output or control HR in Afib)

A

Loop diuretics
Diogxin

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15
Q

T/F: PAH leads to a higher risk of bleeding

A

False - leads to a pro-thrombic state and increased risk of blood clots

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16
Q

What is the preferred anticoagulation if needed in PAH pts?

A

Warfarin

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17
Q

MOA of prostacycline analogues

A

Potent vasodilators and inhibitors of platelet aggregation

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18
Q

Which prostacyclin analogues can be administered by continuous IV at home using an ambulatory infusion pump?

A

Epoprostenol and treprostinil

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19
Q

Treprostrinil (Remodulin) starting dose

A

1.25ng/kg/min, titrate

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20
Q

Epoprostenol (Flolan) starting dose

A

2ng/kg/min, titrate

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21
Q

CI with epoprostenol

A

HF with decreased LVEJ

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22
Q

CI with treprostinil (oral)

A

severe hepatic impairment (Child-Pugh C)

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23
Q

Warnings for prostacyclin analogues

A

Vasodilation reactions (hypotension, flushing, HA, dizziness)
Rebound pulmonary HTN (with interruption or large decreases in dose, can be fatal)
Chronic IV infusions: sepsis and bloostreen infections
Treprostinil (Orenitram): PO ghost shell, can lodge in diverticulum

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24
Q

Side effects of prostacyclin analogues

A

Hypotension, flushing, jaw pain, HA, dizziness, N/V/D, edema, myalgia, tachycardia, flu-like syndrome, anxiety, tremor, thrombocytopenia
IV/SC infusions: infusion-site pain (esp SC Remodulin)
Treprostinil (inhaled) and iloprost: cough and mouth/throat irritation

25
Q

Parenteral prostacyclin analogues (___,____,____) are very potent vasodilators; aoid interruptions and sudden large dose reductions

A

Epoprostenol - Flolan, Veletri
Treprostinil - Remodulin

26
Q

Which prostacyclin analogues require light protection before reconstitution and during infusion? Which ones require ice packs for stability?

A

Epoprostenol - protect from light before and during infusion
Flolan (epoprostenol) - use ice packs for stability; Veletri and Remodulin are thermostable (no need for ice packs)

27
Q

ng to mg conversion

A

1,000,000 ng = 1 mg

28
Q

Endothelin receptor antagonists MOA

A

Endothelin is a vasoconstrictor with cellular proliferative effects
ERA block endothelin receptors on pulmonary artery smooth muscle cells

29
Q

What are examples of prostacyclin analogues and receptor agonists?

A

Epoprostenol (Flolan, Veletri)
Treprostinil (Remodulin (continuous SC or IV), Tyvaso (inhalation), Orenitram (PO ER tab))
Iloprost (Ventavis (inhalation))
Selexipag (Uptravi (tab))

30
Q

Boxed warnings for endothelin receptor antagonists

A

Teratogenic - must have neg pregnancy test prior to initiation of therapy and monthly thereafter
Bosentan - hepatotoxicity
Requires REMS program

31
Q

Endothelin receptor antagonist examples

A

Bosentan (Tracleer)
Ambrisentan (Letairis)
Macitentan (Opsumit)

32
Q

Contraindications for endothelin receptor antagonist

A

Pregnancy

33
Q

Warnings for endothelin receptor antagonists

A

Hepatotoxicity
Decrease Hgb/Hct
Fluid retention (e.g. pulmonary edema, peripheral edema)
Decreased sperm counts

Bosentan: hypersensitivity reactions

34
Q

Side effects for endothelin receptor antagonists

A

HA, URTI, flushing, hypotension

35
Q

Bosentan is contraindicated in use with ___ or ___. Bosentan can decrease effectiveness of ____.

A

cylcosporine and glyburide
hormonal contraceptives

36
Q

Besentan is a substrate and inducer of CYP____ and___

A

3A4, 2C9

37
Q

PDE-5i MOA

A

PDE-5 is responsible for the degradation of cGMP. Increased cGMP concentrations lead to pulmonary vasculature relaxation and vasodilation

38
Q

Contraindications for PDE5i

A

Nitrates or riociguat
Sildenafil (Revatio) - avoid taking with protease inhibitors (e.g. atazanavir, ritonavir, others)

39
Q

Sildenafil brand for pulmonary HTN vs erectile dysfunction

A

Pulmonary HTN - Revatio
ED - Viagra

40
Q

Tadalafil brand for pulmonary HTN vs erectile dysfunction

A

Pulmonary HTN - Adcirca, Alyq, Tadliq
ED - Cialis

41
Q

Warnings for PDE-5i

A

Hearing loss, vision loss (NAION), hypotension, priapism (seek emergency medical care if erection > 4hrs), pulmonary edema

42
Q

Side effects for PDE-5i

A

HA, epistaxis, flushing, dyspepsia, extremity/back pain, N/D

43
Q

Use caution with concurrent use of ___ or other antihypertensives as PDE-5i increase risk of hypotension

A

Alpha-1 blockers

44
Q

Soluble guanylate cyclase stimulator examples

A

Riociguat (Adempas)

45
Q

Soluble guanylate cyclase stimulator (sGCs) MOA

A

SGC is a receptor for endogenous nitric oxide
Riociguat (Adempas) sensitizes sGC to endogenous nitric oxide and directly stimulates the receptor at a different binding site&raquo_space; increases cGMP, leading to relaxation

46
Q

Riociguat is approved for use in both PAH and ___

A

CTEPH

47
Q

Boxed warnings for riociguat (Adempas)

A

Teratogenic, must have negative pregnancy test prior to initiation and monthly
REMS program only

48
Q

Contraindications for riociguat (Adempas)

A

Pregnancy, use of PDE-5i or nitrates

49
Q

Warnings for riociguat (Adempas)

A

Hypotension, bleeding, pulmonary edema

50
Q

Side effects for riociguat (Adempas)

A

HA, dyspepsia, dizziness, N/V/D

51
Q

Riociguat should be separated from antacids by ___

A

> 1hr

52
Q

Riociguate should not be administered within ___ hrs of sildenafil or within ___ hrs before or ___ after tadalafil

A

within 24 hrs of sildenafil
24hrs before or 48 hrs after tadalafil

53
Q

Smoking (increases/decreases) riociguat clearance; dose may need to be (increased/decreased) with smoking cessation

A

increases
decreased

54
Q

Define pulmonary fibrosis (PF)

A

scarred and damaged lung tissue

55
Q

S/sx pulmonary fibrosis

A

External dyspnea with a nonproductive cough
As condition worsens, breathing becomes more labored

56
Q

Drugs that can cause pulmonary fibrosis

A

Amiodarone/dronedarone
Bleomycin
Busulfan
Carmustine
Lomustine

Other: nitrofurantoin, sulfasalazine

57
Q

Often the contributing factor for pulmonary fibrosis is not identified and it is called idiopathic pulmonary fibrosis (IPF). What are the 2 drugs no available for IPF?

A

Pirfenidone (Esbriet) and nintedanib (Ofev) - slows the rate of decline in lung function

58
Q

Which meds for PAH require monthly pregnancy tests + REMS?

A

ERAs - Bosentan, ambrisentan, macitentan
sGC stimulator - Riociguat

59
Q

Patient failed on diltiazem treatment for PAH but is currently on a nitrate. Which meds can they NOT use?

A

Riociguat and PDE-5i

NAPLEX 2024 (74 decks)

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