Pulmonary Hypertension

Question 1

What is the normal pressure range for the Pulmonary Artery?

Answer 1

8 - 20 mmHg

Question 2

What Pulmonary Artery pressure would indicate Pulmonary Hypertension?

Answer 2

≥ 20 mm Hg
(At Rest)

Question 3

Causes of Pulmonary HTN: Group 1

Answer 3

Idiopathic
Inherited Mutations
Drug or Toxin Induced

Associated with:
Connective Tissue Disease
HIV
Portal HTN
Congenital Heart Disease

Question 4

Causes of Pulmonary HTN: Group 2

Answer 4

Left Sided Heart Disease

Question 5

Causes of Pulmonary HTN: Group 3

Answer 5

Chronic Lung Disease
Sleep Apnea

Question 6

Causes of Pulmonary HTN: Group 4

Answer 6

Chronic Thromboembolic Pulmonary HTN
- Unresolved Pulmonary Embolism

Question 7

Causes of Pulmonary HTN: Group 5

Answer 7

Unclear Causes

Question 8

Symptoms do not limit physical activity. Ordinary physical activity does not cause undue discomfort.

Answer 8

NYHA Class 1

Question 9

Slight limitation of physical activity. Patient is comfortable at rest yet experiences symptoms with ordinary physical activity.

Answer 9

NYHA Class 2

Question 10

Marked limitation physical activity. The patient is comfortable at rest yet experiences symptoms with minimal activity.

Answer 10

NYHA Class 3

Question 11

Inability to carry out any physical activity. Symptoms at rest. Discomfort is increased by any physical activity.

Answer 11

NYHA Class 4

Question 11

PAH Treatment Algorithm
(Step 1)

Answer 11

Anticoagulation
Diuretics
Oxygen
Digoxin

Question 12

PAH Treatment Algorithm with
(+) Acute Vasoreactivity Testing

Answer 12

Oral CCB

Question 13

PAH Treatment Algorithm with
(-) Acute Vasoreactivity Testing
(Lower Risk)

Answer 13

ERAs or PDE-5 (oral)

Question 14

PAH Treatment Algorithm with
(-) Acute Vasoreactivity Testing
(Higher Risk)

Answer 14

Epoprostenol or Treprostinil (IV)

Question 15

In order to use a Calcium Channel Blocker (CCB) on a patient with PAH, what must you do first?

Answer 15

Vasoactive Testing
(must be a responder)

Question 16

Name two Dihydropyridine CCBs

Answer 16

Nifedipine
Amlodipine

Question 17

What are the most common adverse drugs reactions to Dihydropyridine CCBs?

Answer 17

Reflex Tachycardia
Peripheral Edema

Question 18

Name two Non-dihydropyridine CCBs

Answer 18

Diltiazem
Verapamil

Question 19

What are the most commons adverse drugs reactions to Non-dihydropyridine CCBs

Answer 19

Bradycardia
Peripheral Edema

Question 20

Medications that blocks the vasoconstrictor effects of Endothelin by binding to two distinct receptors in the pulmonary vascular smooth muscle cells, types A and B.

Answer 20

Endothelial Receptor Antagonist (ERA)

Question 21

Name some ERAs medications

Answer 21

Ambrisentan
Bosentan
Macitentan

Question 22

Which ERA medication binds preferentially to Type A receptors?

Answer 22

Ambrisentan

Question 23

How are all Endothelia Receptor Antagonists administered?

Answer 23

Orally

Question 24

What medications are the first line options for WHO Class II-III and Second Line in WHO Class IV?

Answer 24

ERAs

Question 25

What type of Block Box Warning does Ambrisentan have?

Answer 25

Pregnancy - must have monthly pregnancy tests during and for 1 month after treatment - must have 2 reliable forms of contraception

Question 26

Adverse Effects of Ambrisentan

Answer 26

Peripheral Edema ↑ Liver Enzymes ↓ Hemoglobin ↓ Spermatogenesis

Question 27

Block Box Warning for Bosentan

Answer 27

Pregnancy Hepatotoxicity - monitor AST/ALT monthly - more than 3x upper limit, adjust dose - avoid in baseline elevations

Question 28

When is Bosentan contraindicated?

Answer 28

3A4 and 2C9 Inhibitors

Question 29

Macitentan Black Box Warnings

Answer 29

Pregnancy MUCH lower incidence of Hepatotoxicity

Question 30

Contraindications for Macitentan

Answer 30

3A4 Inhibitors or Inducers

Question 31

Medications that increase NO (Nitric Oxide) signaling and cause vasodilation through NO/cGMP pathway. Slows the degradation of cGMP.

Answer 31

Phosphodiesterase V Inhibitors (PDE V Inhibitors)

Question 32

What are some common PDE V Inhibitors

Answer 32

Sildenafil (Viagra) Tadalafil (Cialis) Vardenafil

Question 33

What is Sildenafil known as when branded for PAH?

Answer 33

Revatio - 3x daily

Question 34

Sildenafil Cautions

Answer 34

MI Stroke

Question 35

Adverse Reactions of Sildenafil

Answer 35

Flushing Headache Visual Disturbances Dyspepsia Sudden cessation can result in PAH exacerbation

Question 36

Drug Interactions of Sildenafil

Answer 36

MAJOR Substrate of 3A4 May enhance hypotensive effects of other meds

Question 37

Dosage considerations for Tadalafil

Answer 37

Hepatic & Renal Impairment

Question 38

PDE-V Inhibitor that has less proven efficacy and has not been studied in hemodialysis patients or patients with severe hepatic dysfunction, use is not recommended.

Answer 38

Vardenafil

Question 39

Medications that stimulate the NO-cGMP pathway to enhance cGMP production and promote vasodilation. May have anti-proliferative and anti-remodeling properties.

Answer 39

Guanylate Cyclase Stimulator (Riociguat)

Question 40

Black Box Warning for Riociguat (Guanylate Cyclase Stimulator)

Answer 40

Teratogenicity

Question 41

A recent study of Riociguat found what negative outcome IF pulmonary HTN was associated with idiopathic interstitial pneumonias?

Answer 41

↑ Mortality

Question 42

Adverse effects of Riociguat (Guanylate Cyclase Stimulator)

Answer 42

Significant Hypotension Dizziness Headache Dyspepsia

Question 43

Riociguat is contraindicated in combination with what class of medications due to significant hypotension?

Answer 43

PDE-V Inhibitors - Also a Major 3A4 substrate

Question 44

What can reduce Riociguat levels by up to 50-60%

Answer 44

Smoking

Question 45

What effects do Prostacyclins have?

Answer 45

Vasodilation Inhibit platelet aggregation

Question 46

Name 3 Prostacyclin Analogs

Answer 46

Epoprostenol Iloprost Treprostinil

Question 47

IV Prostacyclin 3-5 Minute Half Life Only Stable at Room Temp. for 8 hours Initiation and Titration only done in ICU

Answer 47

Epoprostenol

Question 48

Adverse Effects of Epoprostenol

Answer 48

Tachycardia Flushing Hypotension Dizziness Headache

Question 49

What must ABSOLUTELY be avoided with Epoprostenol due to PAH rebound resulting in deterioration and even death?

Answer 49

Abrupt Interruption

Question 50

Cautions with Epoprostenol

Answer 50

Anti-platelet Meds. Anti-hypertensive Meds.

Question 51

Inhaled Prostacyclin Administered 6 - 9x daily Immediate access to medication is always necessary!

Answer 51

Iloprost

Question 52

Adverse Effects of Iloprost

Answer 52

Trismus + Jaw Pain

Question 53

What type of patients does Iloprost lack established safety in?

Answer 53

Asthma + COPD - may cause bronchospasm

Question 54

Prostacyclin that needs to be administered with a High Fat Meal. - Inhaled form has not been evaluated as Monotherapy

Answer 54

Treprostinil

Question 55

What increases the absorption of the oral dosage form of Treprostinil?

Answer 55

Alcohol

Question 56

Oral agent that selectively antagonizes prostacyclin receptors.

Answer 56

Selexipag (Prostacyclin IP Receptor Agonist)

Question 57

What is Selexipag highly bound to?

Answer 57

Protein

Question 58

Selexipag should be dose adjusted or avoided in what type of patients?

Answer 58

Hepatic

Question 59

Mortality Benefit: 43% reduction in all-cause mortality at 3 years

Answer 59

Initial Combination Therapy

Question 60

Long Term Benefit: Sequential add-on therapy improves outcomes when clinical response to monotherapy is insufficient

Answer 60

Sequential Combination Therapy

Question 61

Recommended in signs of R. Ventricular Heart Failure and Fluid Retention

Answer 61

Diuretics

Question 62

Recommended if Arterial Blood Oxygen is consistently < 60 mmHg (especially Group 3)

Answer 62

Oxygen