PAH Flashcards
What is PH
Vascular disease with structural changes
PAH 3 Hemodynamic levels
mPAP > 20 mmHg
PAWP ≤15 mmHg
PVR > 2 woods unit
Why is diagnosis of PAH difficult
Fatigue
SOB
Edema
sounds like HF
Goal of treatment for PAH
When can we use CCB
Positive vasodilator test
what are the 3 CC for PAH
amlodipine
diltiazem
Nifedipine
Amlodipine Dose
Diltiazem Dose
Nifedipine (XL) Dose
CCB is the 4 AD most concerning
- Peripheral edema
- Flushing
- Muscle cramps
- Gingival hyperplasia
What do we have to consider before giving ERA
Rems
What are the warnings of ERA
- All ERAs are teratogenic
- Require monthly pregnancy test – 2 methods of contraception
- Liver complications
- Drug interactions
- Edema
Bosentan
issue with Bosentan
Never recommend
Ambrisentan
Why Ambrisentan
generic
less DDI
no UTI like macitentan
Macitentan
why Macitentan
less edema
ERA class effec
Edema
nasal congestion
Warning of NO pathway durgs
- Hypotension
- nitrates
Sildenafil
Tadalafil
Riociguat
Riociguat warnings
- CYP inhibitors / inducers * GERD
- REMS – teratogenic
Biggest thing to monitor with Riociguat
BP
smoking and Riociguat
Opsynvi
What best NO i to poick
tadalifil
when would i use Riociguat
when they need more power
Warning of Prostacyclins
Serious side effects
central line infections
Risk of abrupt discontinuations
Epoprostenol
issues with Epoprostenol
Treprostinil
Treprostinil formulation
Why Treprostinil
live far from hospital
pumps are smaller
Treprostinil Sq good?
dont want IV access
but pain for a week then get better
Try to use the same site
why nebulizer
group 1 and group 3
why dry powder
group 1 and group 3
easier
know the pump rate
Iloprost
Selexipag
bonus of Selexipag
selective
Flolan® and Veletri®
prost class AD
Headache
* Jaw pain
* Flushing
* Nausea/diarrhea * Skin rash
* Musculoskeletalpain
* Infections
* Interruptionininfusionlifethreatening
sotatercept
sotatercept need to happen
need to be monitored
sotatercept hemoglobin paramenters
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
what happens have to hold more than 9 weeks?
game changer drug sotatercept
biggest thing to counsle on sotatercept
nose bleeds
how do we treat patietns
1-4 drugs depending on patient
treatment pathway
non-pharm treatments
surgery ( CTEPH, BPA)
lung transplant
PAH and pregnancy
must be avoided bc it messes up everything
can do it but must be planned
supportive thearpies
Dietsodium restriction
* Exercisenot 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