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NAPLEX 25 > Chapter 40: Pulmonary Arterial Hypertension > Flashcards

Chapter 40: Pulmonary Arterial Hypertension Flashcards

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1
Q

What is pulmonary hypertension characterized by?

A

Continuous high blood pressure in the pulmonary arteries

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2
Q

List drugs that can cause PAH

A
  1. Cocaine
  2. Fenfluramine
  3. Methamphetamine
  4. SSRI during pregnancy
  5. Weight loss drugs like phentramine
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3
Q

What causes pulmonary arterial hypertension (PAH)?

A

An imbalance in vasoconstrictor and vasodilator substances.

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4
Q

Name two vasoconstrictor substances that are increased in PAH.

A
  • Endothelin-1
  • Thromboxane A2 (TXA2)
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5
Q

What is decreased in PAH that contributes to the condition?

A

Vasodilating substances, such as prostacyclins.

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6
Q

What effect does vasoconstriction have on the pulmonary vasculature?

A

It results in reduced blood flow and high pressure.

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7
Q

What happens to the pulmonary artery walls due to increased smooth muscle cells?

A

They thicken and form scar tissue (vasoproliferation).

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8
Q

What is the most common cause of death in people with PAH?

A

Heart failure.

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9
Q

List some symptoms of PAH.

A
  • Fatigue
  • Dyspnea
  • Chest pain
  • Syncope
  • Edema
  • Tachycardia
  • Raynaud’s phenomenon
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10
Q

What is the recommended sodium intake for patients with PAH?

A

< 2.4 grams/day

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11
Q

What routine immunizations are advised for patients with PAH?

A

Immunizations against influenza and pneumococcal pneumonia

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12
Q

Patients with PAH should avoid medications like _______ that increase sodium and water retention.

A

NSAIDs

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13
Q

What is performed to confirm the diagnosis of PAH?

A

A right heart catheterization

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14
Q

What type of testing is conducted during right heart catheterization for PAH?

A

Vasoreactivity testing
(Short-acting vasodilators like inhaled nitric oxide, IV epoprostenol, and IV adenosine are used.)

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15
Q

What indicates a positive response in acute vasoreactivity testing?

A

mPAP falling by at least 10 mmHg to an absolute value less than 40 mmHg

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16
Q

What is the initial treatment for patients who are responders to vasoreactivity testing?

A

Oral calcium channel blocker (CCB)

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17
Q

Which CCBs are most frequently used for PAH treatment?

A
  • Long-acting nifedipine
  • Diltiazem
  • Amlodipine
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18
Q

What should be done for non-responders to vasoreactivity testing?

A

Treat with more potent vasodilating drugs:
1. prostacyclin analogues
2. endothelin receptor antagonists
3. PDE-5 inhibitors
4. Soluble guanylate cyclase stimulator

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19
Q

What impact do parenteral prostacyclin analogues have on mortality?

A

They decrease mortality

IV epoprostenol

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20
Q

What supportive therapies may be used for PAH?

A
  • Loop diuretics (for volume overload)
  • Digoxin (to improve cardiac output or control heart rate)
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21
Q

What is the preferred anticoagulant in PAH management?

A

Warfarin

It should be titrated to an individualized patient INR goal based on thromboembolic risk.

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22
Q

What is prostacyclin I2 known for?

A

It is a vasodilator with anti-proliferative effects.

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23
Q

What are prostacyclin analogues also known as?

A

Prostanoids.

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24
Q

What effects do prostacyclin analogues have?

A

They are potent vasodilators and inhibitors of platelet aggregation.

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25
Name a prostacyclin receptor agonist.
Selexipag (Uptravi).
26
How can epoprostenol and treprostinil be administered?
By continuous IV infusion at home using an ambulatory infusion pump.
27
Name
28
What is the start dosing range for Epoprostenol?
Start t 2 ng/kg/min
29
Epoprostenol brand name
Flolan
30
List some warnings associated with prostacyclin analogues.
* Vasodilation reactions (hypotension, flushing, headache, dizziness) * Rebound PH * Increased risk of bleeding
31
What are common side effects of prostacyclin analogues?
* Hypotension * Flushing * Jaw pain
32
Why is immediate access to a backup pump essential?
Due to the short half-lives of epoprostenol and parenteral treprostinil
33
What is endothelin?
A vasoconstrictor with cellular proliferative effects
34
What do endothelin receptor antagonists (ERAs) block?
Endothelin receptors on pulmonary artery smooth muscle cells
35
List endothelin receptor antagonists (ERAs)
* Bosentan (Tracleer) * Ambrisentan (Letairis) * Macitentan (Opsumit)
36
What are the boxed warnings associated with Bosentan?
* Teratogenic * Hepatotoxicity (ALT/AST and liver failure) * Available only through individual REMS programs
37
What must women of childbearing potential do before starting Bosentan therapy?
Have a negative pregnancy test prior to initiation and monthly thereafter
38
What are the contraindications for ERAs?
* Pregnancy
39
What are the warnings associated with ERAs?
* Hepatotoxicity * Low Hgb/Hct * Fluid retention * Decreased sperm counts
40
What are common side effects of ERAs?
* Headache * Upper respiratory tract infections * Flushing * Hypotension
41
What should be monitored when using ERAs?
* LFTs * Bilirubin * Hgb/Hct * Pregnancy tests
42
What effect does Bosentan have on hormonal contraceptives?
Decreases their effectiveness
43
What effect do increased cyclic GMP concentrations have on the pulmonary vasculature?
Lead to pulmonary vasculature relaxation and vasodilation
44
What are the brand names for Sildenafil used for PAH?
Revatio, Liqrev
45
What are the brand names for Tadalafil used for PAH?
Adcirca, Alya, Tadlia
46
Which medications should PDE-5 inhibitors be avoided with?
Nitrates or riociguat
47
What are the warnings associated with PDE-5 inhibitors?
* Hearing loss * Vision loss * Hypotension * Priapism * Pulmonary edema
48
What are common side effects of PDE-5 inhibitors?
* Headache * Epistaxis * Flushing * Dyspepsia * Extremity or back pain * Nausea/Vomiting
49
What should be used with caution when taking PDE-5 inhibitors?
Concurrent use of alpha-1 blockers or other antihypertensives
50
What is an absolute contraindication to the use of PDE-5 inhibitors?
Taking nitrates
51
What is soluble guanylate cyclase (sGC)?
A receptor for endogenous nitric oxide
52
What is the function of Riociguat (Adempas) regarding sGC?
Sensitizes sGC to endogenous nitric oxide and directly stimulates the receptor
53
What effect does Riociguat have on cGMP?
Increases cGMP, leading to relaxation and antiproliferative effects in pulmonary artery smooth muscle cells
54
For what conditions is Riociguat approved?
Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)
55
What is a major boxed warning for Riociguat?
Teratogenic effects; a negative pregnancy test is required prior to initiation and monthly thereafter
56
What program must prescribers and patients enroll in to access Riociguat?
Adempas REMS Program
57
What are the contraindications for Riociguat?
Pregnancy, use of PDE-5 inhibitors or nitrates
58
What warnings are associated with Riociguat?
Hypotension, bleeding, pulmonary edema
59
What are some common side effects of Riociguat?
Headache, dyspepsia, dizziness, nausea/vomiting/diarrhea
60
How does smoking affect Riociguat clearance?
Increases clearance; dose may need to be decreased with smoking cessation
61
What is pulmonary fibrosis?
PF is scarred and damaged lung tissues
62
List key drugs that can cause pulmonary fibrosis
1. Amiodarone/dronedarone 2. Bleomycin 3. Busulfan 4. Carmustine 5. Nitrofurantoin 6. Sulfasalazine

NAPLEX 25 (31 decks)

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