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Cardio > PAH > Flashcards

PAH Flashcards

1
Q

drug interactions with PDE5i

A

nitrates, alpha blockers, alcohol, strong CYP3A4 inhibitors/inducers

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2
Q

drug interactions with riociguat

A

strong CYP and p-gp inhibitors/inducers, PDE5i, non-specific PDEi (theophylline, dipyridamole), smoking, nitrates, antacids

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3
Q

drug interactions with ERAs

A

strong CYP3A4 and CYP2C19 inhibitors/inducers, warfarin, oral conteraceptives

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4
Q

drug interactions with bosentan

A

glyburide, cyclosporine

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5
Q

drug interactions with prostacyclins

A

vasodilators, antiplatelets, anticoagulants

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6
Q

drug interactions with treprostinil

A

gemfibrozil, rifampin

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7
Q

drug interactions with selexipag

A

CYP2C8 inhibitors (clopidogrel), inducers (rifampin)

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8
Q

which PDE5 inhibitor has a special renal/hepatic dose or taking ritonavir

A

tadalafil: half dose

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9
Q

which agents have a REMS program for teratogenicity

A

bosentan, ambrisentan, macitentin, riociguat

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10
Q

what do you monitor for REMS with the ERAs

A

pregnancy, LFTs, Hb

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11
Q

which ERA is selective and what is it selective for

A

ambrisentan (ET-1)

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12
Q

which ERA is lipophilic and tissue-selective

A

macitentan

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13
Q

which of the newer oral therapies should you avoid if CrCL<15 mL/min or on hemodialysis

A

riociguat

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14
Q

which of the newer oral therapies should you administer with a high calorie, high fat meal to improve bioavailability

A

treprostinil diolamine

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15
Q

what are some line-related complications with SQ prostacyclins

A

injection-site pain and swelling (use topical analgesics)

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16
Q

what are some line-related complications with IV prostacyclins

A

catheter-related infection, bacteremia, thrombosis (increased risk of gram-negative infection treprostinil>epoprostenol)

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17
Q

what are some adjunctive treatments for PAH

A

diuretics, digoxin, oxygen, warfarin (out of favor)

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18
Q

explain the difference between pulmonary hypertension and pulmonary arterial hypertension

A

PH is high blood pressure in the lungs, a general term with many possible underlying causes. PAH is a specific subset of PH, formerly referred to as primary pulmonary hypertension

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19
Q

hemodynamic classification of PH

A

mPAP >20 mmHg

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20
Q

hemodynamic classification of PAH

A

mPAP>20 mmHg, PAWP <15 mmHg, PVR>3 wood units

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21
Q

name some definite drug-induced causes of PAH

A

aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil, benfluorex, methamphetamines, dasatinib (tyrosine kinase inhibitors for cancer)

22
Q

name some possible drug induced causes of PAH

A

cocaine, phenylpropanolamine, st john’s wort, chemo, interferon alpha and beta, amphetamines, l-tryptophan, alkylating agents, bosutinib, direct-acting antiviral agents against hepatitis C virus, leflunomide, indirubin

23
Q

WHO Class I

A

symptoms at levels of exertion that would limit normal individuals

24
Q

WHO Class II

A

symptoms on ordinary exertion

25
Q

WHO Class III

A

symptoms on less-than-ordinary exertion

26
Q

WHO Class IV

A

symptoms at rest

27
Q

common symptoms of PAH

A

dyspnea, fatigue, syncope, palpitations, chest pain, leg edema

28
Q

describe the pathophysiology of PAH

A

Increased vascular resistance: vasoconstriction, vascular wall remodeling, in situ thrombosis. Increased vasoconstriction: ET1, TXA2. Decreased vasodilation: PGI2, NO

29
Q

what are the three pathways for therapeutic targets

A

endothelin pathway, nitric oxide pathway, prostacyclin pathway

30
Q

which agents work on the endothelin pathway

A

ERAs

31
Q

which agents work on the nitric oxide pathway

A

PDE5i, riociguat

32
Q

which agents work on the prostacyclin pathway

A

prostacyclins and selexipag

33
Q

explain right ventricular remodeling in PAH

A

the right heart is strained–> right chambers thicken and enlarge–> blood is forced backwards (regurgitation) through tricuspid valve–> compromises the heart’s pumping capacity

34
Q

parameters for low european risk assessment

A

WHO class 1-2, >440 m 6 min walk, NT-proBNP <300, BNP<50, CI>2.5, SvO2 >65%, RAP <8 mmHg

35
Q

parameters for intermediate european risk assessment

A

WHO class 3, 165-440 m 6 min walk, NT-proBNP 300-1400 ng/L, BNP 50-200 ng/L, SVO2 60-65%, CI 2-2.4, RAP 8-14 mmHg

36
Q

parameters for high european risk assessment

A

WHO class 4, <165 m 6 min walk, NT-proBNP >1400, BNP >300, RAP >14, CI<2, SvO2 <60%

37
Q

what drugs can you consider for positive response/vasoreactive

A

CCBs: amlodipine, nifedipine, diltiazem

38
Q

adverse reactions for PDE5i

A

headache, dyspepsia, flushing, epistaxis, insomnia, hypotension, visual changes

39
Q

adverse reactions for riociguat

A

headache, dizziness, dyspepsia, gastroesophageal reflux, nausea, diarrhea, vomiting, hypotension, anemia, constipation, teratogenicity

40
Q

adverse reactions for ERAs

A

headache, flushing, peripheral edema, nasal congestion, sinusitis, transaminitis, liver injury, anemia, teratogenicity

41
Q

adverse reactions for prostacyclins

A

nausea, vomiting, diarrhea, flushing, jaw pain, headache, rash, erythema, hypotension, leg pain (inhaled: cough, throat irritation)

42
Q

adverse reactions for selexipag

A

headache, diarrhea, jaw pain, nausea, myalgia, vomiting, extremity pain, flushing

43
Q

which drug has increased risk of gram-negative infection

A

treprostinil>epoprostenol

44
Q

give a PDE5i dose

A

tadalafil 40 mg po daily (20 if renal/hepatic impairment or on ritonavir)

45
Q

give a ERA dose

A

ambrisentan 10 mg po daily

46
Q

give a parenteral prostacyclin dose

A

epoprostenol 2 ng/kg/min titrated to dose-limiting adverse effects (usual range 20-40 ng/kg/min)

47
Q

give an inhaled prostacyclin dose

A

treprostinil 3 breaths QID, titrate by 3 breaths every 1-2 weeks up to 9 breaths QID

48
Q

give the riociguat dose

A

1 mg PO tid, titrate in 0.5 mg increments every 2 weeks up to 2.5 mg PO tid (start at 0.5 if hypotension risk or strong CYP and p-gp inhibitors)

49
Q

when to avoid riociguat

A

CrCL<15 mL/min

50
Q

give the treprostinil diolamine extended release dose

A

initiate 0.25 mg PO bid or 0.125 mg tid, titrate by 0.125-0.5 mg PO increments bid to tid every 3-4 days or longer

51
Q

give the selexipag dose

A

200 mcg PO bid and uptitrate weekly as tolerated to maximum of 1600 mcg po bid

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