PAH Flashcards

(71 cards)

1
Q

What is PAH?

A

Increased BP in pulmonary vasculature
1’ elevation of pulmonary arterial system not 2’

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2
Q

PAH can lead to?

A

Right heart failure- it is a progressive disease

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3
Q

What the clinical markers of PAH?

A

MPAP: >25 mmhg @ rest with PCWP < /= 15 mmHG and PVR >3

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4
Q

What are the initial symptoms?

A

Exertional dyspnea, fatigue, exercise intolerance

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5
Q

What are the progressive symptoms?

A

Dyspnea @ rest, chest pain, syncope, peripheral edema, ascites, pleural effusions, right heart failure

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6
Q

Pah diagnosing risk factors?

A

Female, younger <65 years old, no significant heart/ lung disease
Stimulant use, HIV, cirrhosis, connective tissue disease, heart defects, appetite suppressants

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7
Q

What should you assess for diagnosis?

A

Right ventricular systolic pressure, and residual volume to rule out left heart disease
Pulmonary disease and sleep disorders
Chronic thromboembolic disease

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8
Q

What tests should be done?

A

HIV, LFTs, ANA, TSH, drug screen
ECG, PFT, PSG, RHC

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9
Q

What is the gold standard?

A

Right heart catheterization
Required for insurance approval

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10
Q

How do you manage group 1:

A

PAH- highly specialized and specific meds

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11
Q

How do you manage group 2:

A

Left Heart(venous HTN): treat HFpEF, HFrEF

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12
Q

How do you manage group 3:

A

Lung disease- treat COPD, sleep apnea

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13
Q

How do you manage group 4:

A

Chronic thrombolic embolic PH: surgical care

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14
Q

If it’s precapillary (right), what are the clinical markers?

A

MPAP > 25
PAWP<15

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15
Q

If it’s post capillary (left), what are the clinical markers?

A

MPAP>25
PAWP >15

Regular PH due to LHD

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16
Q

Class 1?

A

Without limitation of physical activity

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17
Q

Class 2?

A

Slight limitation of physical activity but comfortable at rest
Ordinary physical activity causes dyspnea and fatigue and chest pain

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18
Q

Class 3?

A

Marked limitation of physical activity but comfortable at rest, less than ordinary activity causes dyspnea and fatigue and chest pain

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19
Q

Class 4?

A

limitation of ANY physical activity without symptoms- dyspnea and fatigue at rest

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20
Q

If patient is in group 2 or 3 due to left heart disease or hypoxemic, what do you do?

A

Do NOT treat with vasoactive agents
No benefit- worsening fluid
Group 3- no therapies but inhaled treprostinil some improvement of 6MWD and interstial lung disease

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21
Q

1’ PAH

A

Idiopathic, heritable-BMPR2 mutation

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22
Q

2’ PAH

A

Drug and toxin induced, HIV, heart disease, connective tissue, lupus

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23
Q

What drugs are risk factors for PAH?

A

SSRI, amphetamines, methaphetamines, cocaine and St. John’s wort

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24
Q

What is the patho of PAH?

A

Vasoconstriction and cell proliferation
Inflammation
Thrombosis
Decreased PGI2, NO, VIP
Increased ET-1, TxA2, 5-HT

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25
Low risk?
Classes 1 and 2 6MWD>440 CI> 2 O2> 65%
26
Intermediate risk?
Class 3
27
High risk? >10%
Class 4 6MWD<165 CI<2 O2<60% RHF, repeated syncope, pericardial effusion
28
What are some supportive therapies?
Exercise, diuretics, anticoagulation, oxygen, vaccinations, avoidance of pregnancy
29
Should we use anticoagulants?
No- trials indicate no benefit and no assessment of risks vs benefit
30
Should you use CCB?
No- low number of patients have a positive vasoreactivity response Would have to use higher than standard dose
31
What are the prostacyclins?
Epoprosterol, illprost, tetroprostinil, selexipag
32
What do prostacyclins do?
Increase CaMP= vasodilation and anti proliferation- inhibits platelet aggregation
33
What are the endothelins?
Ambrisentan: bosentan + macitentan
34
What do endothelins do?
Vasoconstriction and proliferation
35
Which are the NO?
Tadalafil, sildenafil, riociguat
36
What does NO do?
Vasodilation and anti proliferation
37
What happens to prostacyclin levels in PAH?
It goes down - so need to give
38
What is clinical marker of just pulmonary hypertension?
MPAP>25 at rest
39
What is the response of vasoreactivity testing?
Defined as MPAP decrease by >10 to <40 AND maintain/ improve cardiac output
40
What SE are possible with prostacyclin?
Flushing, headache, nausea, vomiting, anxiety, hypotension and chest pain Later- muscoskeletal pain and neuropathic pain Long term
41
How do you deal with SE of prostacyclins?
Titrate drug to se and keep titrating till patient develops then stop and start again once feels better
42
Epoprostenol is the only prostacyclin to show a ——
Decrease in mortality
43
What happens if you abruptly DC prostacyclins?
Rebound pulmonary HTN
44
Epoprostenol has a ——- half life so,
Short, the moment you stop you’ll get side effects within minutes
45
What is the prostacyclin dosing?
Ng/ kg/ min 1 mg= 1,000,000ng Dosing weight should never change without consulting provider
46
When giving prostacyclins what should you know?
Back up pump and tubing need to be available Dedicated central line - do not flush other meds through line Home pump not good Do not draw labs from the line
47
Epoprostenol comes in 2 forms:
Veletri- more stable Flolan
48
Which are the inhaler prostacyclins?
Iloprost and treprostinil Too many dosing, 6-9 in a day
49
What are the oral prostacyclins?
Treprostinil and selexipag
50
Treprostinil
Less invasive and simpler Monotherapy for class 2-3
51
Selexipag
More subjective improvements of symptoms
52
In pah endothelins is ——-
High- we don’t want so we antagonize to prevent vasoconstriction and cell proliferation
53
Endothelin patho,
Causes vasoconstriction and mitogenic effects by binding to A/B receptors found in pulmonary smooth muscle and endothelial cells
54
What drugs are the endothelins antagonist?
Bosentan, ambrisentan, macitentan
55
What are the se of endothelins Antagon.?
Hepatotoxicity, REMs pregnancy-need 2 forms of BC, edema
56
Which of the endothelin drugs have more hepatotoxicity?
Bosentan , so do baseline LFT
57
Endothelins are metabolized by?
2C19 -PPI metabolized by this and 3A4
58
If bosentan is a 3a4 inducer then
Concentration of sildenafil, warfarin, plavix, and protease inhibitors will go down.
59
Macitentan benefit?
Enhanced tissue penetration and receptor binding- reason for less liver issues Minimal drug interactions
60
Patho of phosphodiesters?
Break down camp so no vasodilation- so need to inhibit
61
Phosphodiesterase inhibitors do what?
Vasodilator and increase gmp to Camp and more NO
62
Which are the phosphodiesterase I?
Sildenafil -20mg tid Tadalafil- 40 mg Guanylate Cyclase stimulator Riociguat
63
Major se of pde5I?
Flushing, headaches, syncope, visual changes, diarrhea Do not use with riociguat and nitrates-increased HTN Do not use with Bocentan-3A4 inducer
64
Pde5I get metabolized how?
By 3A4- inhibitors will increase drug concentrations
65
Riociguat contraindicated and se?
Syncope Do not use with nitrates and pde5I or crcl <15 Smokers decrease concentration since works as inducer
66
Treatment for class 1?
No theory recommended Monitor for development of symptoms
67
Treatment for class 2?
Monotherapy or combo- ambrisentan and tadalafil
68
Treatment for class 3?
Monotherapy or combo- ambrisentan and tadalafil Can use Epoprostenol or treprostinil if rapid disease progression
69
Treatment for class 4?
Epoprostenol
70
Combo therapy: ambrisentan and tadalafil- benefits and negs?
Significant improvement in functional outcomes but more adverse effects- peripheral edema, nasal congestion and headache
71
How do you use sequential combinations?
Upfront combo- start with one then add another (2-3 drugs) Never remove drugs