Pulmonary Arterial HTN

Question 1

what is the normal PA pressure

Answer 1

PAP 8-20

Question 2

what is used to dx PAH

Answer 2

right heart catheterization with a vasoreactive test using NO, epoprostenol or adenosine

Question 3

what are the different causes of PAH

Answer 3

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)

Question 4

what drugs cause PAH

Answer 4

cocaine
stimulants
SSRI in pregnancy –> PPHN
weight loss drugs (phentermine)

Question 5

what is the overall patho behind PAH

Answer 5

increase vasoconstriction (TXA2, endothelin-1)

decreased vasodilation (prostacyclins)

Question 6

non-pharm recommendations for PAH

Answer 6

salt <2.4g a day
no NSAIDs
influenza and pneumococcal vaccines
avoid high altitudes
O2 >90%

Question 7

what can be used to treat volume overload in PAH

Answer 7

loops

Question 8

what can be used to treat low CO and afib in PAH

Answer 8

digoxin

Question 9

what can be used to treat clot risk in PAH

Answer 9

warfarin preferred

Question 10

all patients with a PAH dx should be

Answer 10

referred to a PAH specialty care center

Question 11

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|)

Answer 11

CCB!

LA nifedipine
amlodipine
diltiazem

Question 12

why is verapamil not recommended in PAH treatment

Answer 12

contractility properties too strong

Question 13

what are the treatment options for patients whose R heart cath testing showed an mPAP decrease that was still 40mmHg +

Answer 13

1-2 of the following…
prostacyclin analogues
endothelin-1 R antagonists
PDE5-i (sildenafil and tadalafil)
Riociguat

Question 14

what are the prostacyclin analogues recommended in PAH non responsive to vasoreactive testing?

Answer 14

Epoprostenol (Flolan)
Treprostinil (Remodium)

Question 15

prostacyclin analogues
SE
CI

Answer 15

SE - bleed risk due to plt aggregation inhibition, hypotension
CI in HFrEF!!!!!

Question 16

endothelin-1 receptor antagonists used in PAH

Answer 16

end in - entan

bosentan (Tracleer)
ambrisentan (Letairis)
Macifentan (Opsumit)

Question 17

which treatment options for PAH should not be used in pregnancy due to teratogenicity

Answer 17

endothelin-1 R antagonists (-entan)
riociguat

Question 18

endothelin-1 R antagonists SE

Answer 18

hepatotoxicity
teratogenicity
flushing
hypotension
HA
URTIs
fluid retention

Question 19

PDE5-is for PAH

Answer 19

sildenafil (Revatio)
tadalafil (Adcirca)

Question 20

sildenafil and tadalafil are CI with

Answer 20

nitrates
riociguat
3A4- inhibitors (PDE5-i are major substrates)

Question 21

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

Answer 21

B. endothelin-1 R antagonist

D. riociguat

prostacyclins CI in HFrEF
PDE5-i CI with nitrates (NTG)

Question 22

what would be the concern of starting sildenafil (Revatio) 20mg PO TID for PAH in a patient on amiodarone for rhythm control in afib?

Answer 22

sildenafil is a major 3A4 substrate and amiodarone will therefore inhibit its metabolism

Question 23

prostacyclin analogues are available in what dosage form

Answer 23

continuous IV