PAH Flashcards

1
Q

What is PH

A

Vascular disease with structural changes

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2
Q

PAH 3 Hemodynamic levels

A

mPAP > 20 mmHg
PAWP ≤15 mmHg
PVR > 2 woods unit

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3
Q

Why is diagnosis of PAH difficult

A

Fatigue
SOB
Edema
sounds like HF

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4
Q

Goal of treatment for PAH

A
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5
Q

When can we use CCB

A

Positive vasodilator test

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6
Q

what are the 3 CC for PAH

A

amlodipine
diltiazem
Nifedipine

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7
Q

Amlodipine Dose

A
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8
Q

Diltiazem Dose

A
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9
Q

Nifedipine (XL) Dose

A
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10
Q

CCB is the 4 AD most concerning

A
  1. Peripheral edema
  2. Flushing
  3. Muscle cramps
  4. Gingival hyperplasia
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11
Q

What do we have to consider before giving ERA

A

Rems

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12
Q

What are the warnings of ERA

A
  1. All ERAs are teratogenic
  2. Require monthly pregnancy test – 2 methods of contraception
  3. Liver complications
  4. Drug interactions
  5. Edema
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13
Q

Bosentan

A
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14
Q

issue with Bosentan

A

Never recommend

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15
Q

Ambrisentan

A
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16
Q

Why Ambrisentan

A

generic
less DDI
no UTI like macitentan

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17
Q

Macitentan

A
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18
Q

why Macitentan

A

less edema

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19
Q

ERA class effec

A

Edema
nasal congestion

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20
Q

Warning of NO pathway durgs

A
  1. Hypotension
  2. nitrates
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21
Q

Sildenafil

A
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22
Q

Tadalafil

A
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23
Q

Riociguat

24
Q

Riociguat warnings

A
  • CYP inhibitors / inducers * GERD
  • REMS – teratogenic
25
Q

Biggest thing to monitor with Riociguat

26
Q

smoking and Riociguat

27
Q

Opsynvi

28
Q

What best NO i to poick

29
Q

when would i use Riociguat

A

when they need more power

30
Q

Warning of Prostacyclins

A

Serious side effects
central line infections
Risk of abrupt discontinuations

31
Q

Epoprostenol

32
Q

issues with Epoprostenol

33
Q

Treprostinil

34
Q

Treprostinil formulation

35
Q

Why Treprostinil

A

live far from hospital
pumps are smaller

36
Q

Treprostinil Sq good?

A

dont want IV access
but pain for a week then get better
Try to use the same site

37
Q

why nebulizer

A

group 1 and group 3

38
Q

why dry powder

A

group 1 and group 3
easier

39
Q

know the pump rate

40
Q

Iloprost

41
Q

Selexipag

42
Q

bonus of Selexipag

44
Q

Flolan® and Veletri®

45
Q

prost class AD

A

Headache
* Jaw pain
* Flushing
* Nausea/diarrhea * Skin rash
* Musculoskeletalpain
* Infections
* Interruptionininfusionlifethreatening

46
Q

sotatercept

47
Q

sotatercept need to happen

A

need to be monitored

48
Q

sotatercept hemoglobin paramenters

A

Delay treatment for at least 3 weeks if any of the following occur:
* Hgb increases more than 2 g/dL from the previous value and is above the upper limit of normal (ULN)
* Hgb increases more than 4 g/dL from baseline
* Hgb increases more 2 g/dL above ULN
* Platelet count decreases to less than 50,000/mm3

49
Q

what happens have to hold more than 9 weeks?

50
Q

game changer drug sotatercept

51
Q

biggest thing to counsle on sotatercept

A

nose bleeds

52
Q

how do we treat patietns

A

1-4 drugs depending on patient

53
Q

treatment pathway

54
Q

non-pharm treatments

A

surgery ( CTEPH, BPA)
lung transplant

54
Q

PAH and pregnancy

A

must be avoided bc it messes up everything
can do it but must be planned

55
Q

supportive thearpies

A

Dietsodium restriction
* Exercisenot heavy physical, low level aerobic
* Appropriate vaccinations (influenza ; pneumococcal pneumonia, COVID19)
* Avoidance of pregnancy (hemodynamic changes)
* Anticoagulation (if not contraindicated)
* Diuretics (RV overload, peripheral edema)
* Oxygen (keep O2 sat>90%)
* Caution in high altitude
* Psychological support