PAH Flashcards

1
Q

How does PAH work?

A
  1. pulmonary arterioles narrow
  2. RV dilates
  3. pulmonary edema and damage
  4. thrombi and/or plexiform lesion formation
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2
Q

PH vs. PAH (differences)

A

PH: MPAP > equal to 20 at rest; more common
PAH: progressive with endothelial dysfunction –> elevated pulmonary arterial pressure and pulmonary vascular resistance; rare

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3
Q

what are the WHO PH classifications?

A

Group 1: PAH
Group 2: LFH
Group 3: LD
Group 4: Chronic thromboembolic pulmonary HTN
Group 5: Pulmonary HTN resulting from unclear mechanisms

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4
Q

what are the signs and symptoms of PAH?

A

early –> present as non-specific resulting in a large differential diagnosis
late –> signs of right-sided HF

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5
Q

what is the diagnosis of PAH?

A
  1. echocardiogram
    –> evaluate potential causes, RV function, estimate PAP and PVR
  2. right heart catheterization
    –> assess response to pulmonary vasodilators before starting therapy
  3. exercise testing
    –> distance walked in 6 min
  4. biomarkers
    –> BP & NTproBNP
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6
Q

what are the hemodynamic definitions of PAH?

A

mPAP > 20
PAWP < or equal to 15
PVR > 2 wood units

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7
Q

what is the PAWP?

A
  1. estimates left arterial pressure
  2. normal 4-12
  3. elevated #s signal LV failure or mitral stenosis
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8
Q

what is the PVR?

A

calculated using formula based on mPAP and PAWP

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9
Q

what are the WHO functional classifications?

A
  1. WHO FC1
  2. WHO FC2
  3. WHO FC3
  4. WHO FC 4
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10
Q

What is the WHO FC1 classifications?

A
  1. no limit of activity
  2. ordinary activity does NOT cause any chest pain, dyspnea, ect.
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11
Q

What is the WHO FC2 classifications?

A
  1. slight limit of activity
  2. comfortable at rest but cause dyspnea, chest pain, ect
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12
Q

What is the WHO FC3 classifications?

A
  1. marked limitation of activity
  2. comfortable at rest and less than ordinary dyspnea, chest pain, ect.
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13
Q

What is the WHO FC4 classifications?

A
  1. cant do anything
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14
Q

what are the guidelines of treatment with PAH pts without comorbidities?

A

RISK 3
if low or interm risk
– initial ERA + PDE5i therapy
if high risk
– initial ERA + PDE5i + PCA
RISK 4
if low risk
– continue initial therapy
if interm-low risk
– add PRA OR
– switch from PDE5i to sGCs
if high or interm-high risk
– add iv or sc PCA and/or evaluate lung transplant

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15
Q

what are the guidelines of treatment with PAH pts with comorbidities?

A
  1. initial oral monotherapy with PDE5i or ERA
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16
Q

what is a vasoreactivity test?

A

positive test = drop in mPAP > 10 w/PAP < 40 w/stable improved cardiac output

17
Q

when can you use a CCB and does it guarantee a response?

A
  1. positive test does not guarantee response to CCB therapy and patents should be continually assess (symptoms and RHC) for response
  2. use only when vasoreactive +
18
Q

what CCBs do you use?

A

-LA nifedipine or diltiazem
- amlodipine
- NO verapamil due to inotropic effects

19
Q

what are the adverse reactions for PDE-5 inhibitors?

A

hearing loss, sudden vision loss, hypotension

20
Q

what is important to know about a soluble guanylate cyclase stimulator?

A

RIOCIGUAT
- cannot be used with PDE5 due to risk of hypotension

21
Q

what are the ERAs?

A

bosentan (mixed)
ambrisentan (ETa selective)
macitentan (mixed)

22
Q

how long does it take ERA to work?

A

8-10 weeks

23
Q

what are the prostacyclin medications?

A

epoprostenol IV
treprostinol (all the above)
selexipag ( po agonist)

24
Q

what are the ADRs for prostacyclins?

A

thrombocytopenia and hypotension

25
Q

what are the inhaled prostacyclins?

A

treprostinol (tyvaso and tyvaso DPI)

26
Q

what is special about treprostinil?

A

dosing always based of weight when initiating the drug

27
Q

what is special and important to know about epoprostenol IV?

A

t1/2 of 3-5 minutes
must ALWAYS have back up cassette prepared
dosing ALWAYS based off of weight when initiating drug

28
Q

what was the conclusion of the ambition trial?

A

if patient candidate for initial combo therapy and is WHO class 2 or 3, then ERA + PDE5i as initial therapy will result in less clinical feature

29
Q

what are some cardiopulmonary comorbidities?

A

CV: obesity, HTN, diabetes, CAD
Pulmonary: lung disease (fibrotic lungs)

30
Q

what is sotatercept-csrk (winrevair)? what is its action and pk note?

A

ACTION:
–> activin signaling inhibitor
–> fusion protein with ActR2a and human IgG Fc
–> acts as ligand trap for TGF-b superfamily
PK NOTE:
–> given SQ q24d and require reconstitution
–> avg. peak 7 day and HL of 24h

31
Q

what is sotatercept-csrk (winrevair)? what is its AEs and use?

A

USE:
–> PAH to inc. excersie capacity, inc. functional class and dec. risk of clinical worsening events
AEs:
–> thrombocytopenia, HA, serious bleeding, emryo-fetal harm

32
Q

what are some general considerations in terms of physical activity?

A

be active within symptom limits

33
Q

what are some general considerations in terms of anticoag?

A
  • no recom against
  • inc. risk of bleeding but could be beneficial
34
Q

what are some general considerations in terms of diuretics?

A

may be used once HF
- monitor kidney and weight

35
Q

what are some general considerations in terms of O2?

A

may be used when symptomatic and desaturation on exercise

36
Q

what are some general considerations in terms of anemia and iron?

A

IV iron needed Hgb <7 and ferritin < 100 or 100-299 ferritin and transferrin sat < 20%

37
Q

what are some general considerations in terms of vaccines?

A

NO flu, strep, pneomonia, covid

38
Q

what are some special considerations for prego and birth control?

A
  1. no in prego
  2. safe: CCBs, PDE5, prostacyclins
  3. NO: ERA, riociguat, selexipag
  4. contraception safe
39
Q
A