Pulmonary Hypertension Flashcards

1
Q

What is Pulmonary Hypertension?

A

When the normal blood pressure is higher in the LUNGS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the WHO classification of Pulmonary Hypertension?

A

Group 1[~3% of patients]
-With an unknown cause
-Treated with lung transplant or CCB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the epidemiology of PAH?

A

The epidemiology is unknown, but it could be caused by like certain medications
-2 to 8 cases per million each year [0.002%]
-Mainly causes problems on the right side of the heart

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the signs and symptoms of PAH?

A

SOB [most common], Chest Pain, Fatigue, Edema, Light-Headedness
-Could also resemble COPD/Asthma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How do you determine the diagnosis of PAH?

A

-Echocardiogram
-Walking for 6 minutes
-Right Heart Catheterization [CONFIRMS IT]
-Other biomarkers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the effects on the body caused by PAH?

A

The pulmonary arterial wall becomes damaged, causing a decrease in blood flow to the lungs.
-Right side of the heart becomes larger
-Left side of the heart becomes smaller

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What happens within the arteries when vascular injury occurs?

A

-Decrease in NO synthase
-Decrease Prostacyclin Production
-Increase Thromboxance Production
-Increase Endothelin 1 Production

*all will cause a start in vasoconstriction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What does Endothlin 1 do?

A

It is a potent vasoconstrictor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the WHO functional classes and difference between them?

A

-Class I: Symptom-free
-Class II: Slight Limitation; Comfortable at rest
-Class III: Marked Limitation; Comfortable at rest
-Class IV: Symptoms at rest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is an Acute Vasoreactivity Test?

A

It is a test to see whether or not a patient is going to need to use a CCB or not.
-Recommended CCBs: Amlodipine, Nifedipine, Diltalizem

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the treatment recommendations for a patient with WHO FC I?

A

Continue to monitor the disease progression and possibly determine a start of medication

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the treatment recommendation for a patient with WHO FC II?

A

See if they can tolerate combo therapy;
-Yes: do Ambreisentan + Tadalafil
-No: Use a ERA, Riocigant, or PDE5 Inhibitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the treatment recommendation for a patient with WHO FC III Low [without rapid progression]?

A

The same as WHO FC II

See if they can tolerate combo therapy;
-Yes: Ambrisentan + Tadalafil
-No: ERA, Riocigant, or PDE5 Inhibitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the treatment recommendation for a patient with WHO FC III High [with rapid progression]?

A

See if they are a candidate for Parenteral prostanoids
-Yes: SC Treprostinil
-No: Inhaled or Oral Prostanoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the treatment recommendation for a patient with WHO FC IV?

A

The same as WHO FC III High

*See if they are a candidate for Parenteral Prostanoids
-Yes: SC Treprostinil
-No: Oral Prostanoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the therapeutic pathways for treating PAH?

A

Prostacyclin Pathway, NO Pathway, Endothelim Pathway

17
Q

What is the MOA for the PDE-5i?

A

They will block the conversion of cGMP to GMP
-The increase of the cGMP will cause pulmonary dilation.

18
Q

What are the PDE5i?

A

Sildenifil, Tadalafil

19
Q

How is Sildenifil and Tadalafil dosed?

A

Sildenifil is BID
Tadalafil is ONCE DAILY

20
Q

What are the Drug Interactions with the PDE5i?

A

Avoid use with Riocigant or Nitrates & CYP3A4

21
Q

What are the side effects of the PDE5i?

A

Headache, Blue Vision, Flushing, Hypotension, Loss of vision and hearing

22
Q

What is the MOA for the Endothelin Receptor Antagonist?

A

ET found within the vasocular smooth muscle with cause vasoconstriction; so ET-1 will cause some bad remodeling. So, blocking ET will result in vasodilation

23
Q

What are the ERAs?

A

Ambrisetan, Bosetan, Machitentan

24
Q

How are the ERAs dosed?

A

Ambrisetan and Macitentan are ONCE DAILY
Bosetan is BID

25
Q

What are the two receptor subtypes for Endothelin Recepters?

A

ETa and ETb
-ETa: found on endothelium and will cause vasodilation [Increased NO & Prostacyclin]
-ETb: found on vascular smooth muscle and will cause vasoconstriction

26
Q

What is the Soluble Guanylate Cyclase Stimulator?

A

Riocigant [the only one in the class]

27
Q

What is important to know about Riocigant?

A

-It can be used as an alternative for PDE5i
-CANNOT be used with Sildenafil & Tadalafil because of the increase risk of Hypotension

28
Q

What was the importance of the AMBITION Trail?

A

It showed the effectiveness of Ambrisetan + Tadalafil [patients with FC II and FC III Low]

29
Q

What was the importance of the TRITON Trial?

A

It showed that there was NO significance in using a TRIPLE therapy over using DUAL therapy
-Triple was 54% decrease while Dual was 52% decrease
-Used Macitentan + Tadalafil + Selexipag

30
Q

What is the MOA of the Prostacyclins?

A

They stimulate the cAMP pathway to increase pulmonary vasodilation
-Used in FC III High and FC IV

31
Q

What are the side effects of Prostacyclins?

A

Headache, jaw pain, limb pain, nausea/vomiting, thrombocytopenia, hypotension

32
Q

What are the Oral Prostacyclins?

A

Treprostanil and Selexipag
-Used for patients that refuse parental therapy

33
Q

What are the side effects for the Oral Prostacyclins?

A

Diarrhea and Anemia

34
Q

What are the Inhaled Prostacyclins?

A

Iloporst and Treprostanil
-Used in patients that refuse parental therapy

35
Q

What are the side effects of the Inhaled Prostacyclins?

A

Cough, Throat Irritation

36
Q

What is the importance about Epoprostenol IV?

A

-It has a very fast half-life [3 to 5 mins]
-Must ALWAYS have a back up ready
-would NEVER start with this

37
Q

What are the pregnancy considerations for PAH medicaitons?

A

Want to AVOID in pregnancy
-Estrogen-containing contraceptives increase VTE
-Bosentan will decrease effectiveness
-ERA & Riocigant are Cat X