Pulmonary Arterial HTN Flashcards
what is the normal PA pressure
PAP 8-20
what is used to dx PAH
right heart catheterization with a vasoreactive test using NO, epoprostenol or adenosine
what are the different causes of PAH
group 1: idiopathic or genetic
group 2: 2/2 left heart disease
group 3: 2/2 lung disease or hypoxia
group 4: 2/2 chronic thromboembolic pulmonary HTN (CTEPH)
what drugs cause PAH
cocaine
stimulants
SSRI in pregnancy –> PPHN
weight loss drugs (phentermine)
what is the overall patho behind PAH
increase vasoconstriction (TXA2, endothelin-1)
decreased vasodilation (prostacyclins)
non-pharm recommendations for PAH
salt <2.4g a day
no NSAIDs
influenza and pneumococcal vaccines
avoid high altitudes
O2 >90%
what can be used to treat volume overload in PAH
loops
what can be used to treat low CO and afib in PAH
digoxin
what can be used to treat clot risk in PAH
warfarin preferred
all patients with a PAH dx should be
referred to a PAH specialty care center
what is the first line tx option for patients whose R heart cath testing showed an mPAP decrease by 10mmHg to an absolute value of <40mmHg (|40mmHg|)
CCB!
LA nifedipine
amlodipine
diltiazem
why is verapamil not recommended in PAH treatment
contractility properties too strong
what are the treatment options for patients whose R heart cath testing showed an mPAP decrease that was still 40mmHg +
1-2 of the following…
prostacyclin analogues
endothelin-1 R antagonists
PDE5-i (sildenafil and tadalafil)
Riociguat
what are the prostacyclin analogues recommended in PAH non responsive to vasoreactive testing?
Epoprostenol (Flolan)
Treprostinil (Remodium)
prostacyclin analogues
SE
CI
SE - bleed risk due to plt aggregation inhibition, hypotension
CI in HFrEF!!!!!
endothelin-1 receptor antagonists used in PAH
end in - entan
bosentan (Tracleer)
ambrisentan (Letairis)
Macifentan (Opsumit)
which treatment options for PAH should not be used in pregnancy due to teratogenicity
endothelin-1 R antagonists (-entan)
riociguat
endothelin-1 R antagonists SE
hepatotoxicity
teratogenicity
flushing
hypotension
HA
URTIs
fluid retention
PDE5-is for PAH
sildenafil (Revatio)
tadalafil (Adcirca)
sildenafil and tadalafil are CI with
nitrates
riociguat
3A4- inhibitors (PDE5-i are major substrates)
59 yo male presents with PAH, non-responsive. PMH HFrEF, DM, afib, SIHD.
meds - entresto BID, metoprolol succinate 50mg QD, empagliflozin 10mg QAM, NTG PRN, lipitor 40mg, metformin 500 BID, aspirin 81mg PO QD
What options are not CI in this patient? SATA
A. prostacyclin analog
B. endothelin-1 R antagonist
C. PDE5-i
D. riociguat
B. endothelin-1 R antagonist
D. riociguat
prostacyclins CI in HFrEF
PDE5-i CI with nitrates (NTG)
what would be the concern of starting sildenafil (Revatio) 20mg PO TID for PAH in a patient on amiodarone for rhythm control in afib?
sildenafil is a major 3A4 substrate and amiodarone will therefore inhibit its metabolism
prostacyclin analogues are available in what dosage form
continuous IV
