Pulm HTN + CF Flashcards by Maya Birchler

mean pulm arterial pressure >/20 at rest is

pulmonary HTN

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pulmonary HTN is measured by

right heart cath

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normal right heart cath pressures

8-20

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DOE, fatigue, chest pain, syncope, palpitations, swelling in abdomen/legs

pulm HTN

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Group 1 of pulmonary HTN

idiopathic, inherited mutations, drug/toxin induced, ass w/ connective tissue disease, HIV, portal HTN, CHD

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Group 2

left sided heart disease

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group 3

due to chronic lung disease

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group 4

from a PE

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group 5

unclear multifactor mechanisms

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WHO class I

no limitation on activity

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WHO class 2

slight limitation

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WHO class 3

marked limitation

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WHO class 4

inability to carry out any physical activity

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use CCBs when

patient responds to vasoactive testing

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CCBs to use

nifedipine and amlodipine (smooth + cardiac)

MC ADR: reflex tachy and peripheral edema

dialitazem (cardiac)

MC ADR: bradycardia and peripheral edema

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block endothelin receptors, preventing vasoconstriction + promoting vasodilation

endothelial receptor antagonists

ambrisentan, bosentan, macitentan

(preferentially type A receptors)
B + M = dual agents

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ambrisentan, bosentan, macitentan are first line for

WHO class II-III, second line in WHO class IV

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ambrisentan, bosentan, macitentan can/cannot be used in pregnancy

cannot – women of child bearing age must take monthly pregnancy test

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What are ADRs of ambrisentan?

peripheral edema, increased liver enzymes, significant drops in hemoglobin, decreased spermatogenesis

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bosentan also has warnings for

hepatotoxicity
- must monitor AST/ALT, dosage adjustments, avoid in elevations

** DIs/ CI in combo with strong 3A4 and 2C9 inhibitors (inducer of those) **

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macitentan has much lower incidence of — —

reported hepatotoxicity

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macintentan is a — of 3a4

substrate – do not use with strong 3a4 inhibitors or inducers

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inhibition of PDE V –> increase in no signlaing –> vasodilation through NO/cGMP –> slow degredation of cGMP –> vasodilation

Phosphodiesterase V inhibitors – sildenafil, tadalafil, vardenafil

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sildanefil, taladafil are first line for

WHO Class II-III, second line in WHO class IV

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sildenafil caution in patients who have had

MI or stroke

ADRs of sildenafil/tadalafil include

flushing, HA, dyspepsia, visual disturbances, sudden cessation

DIs of sildenafil/tadalafil include

major substrate of 3a4, may enhance hypotensive effects of other BP lowering agents like vasodilators (nitrates) and alpha blockers

Tadalafil needs dosage considerations in

hepatic and renal impairment

Vardenafil has more/less efficacy in class II-IV patients

less but same ADRs

stimulate NO-cGMP pathway to enhance cGMP production + promote vasodilation

guanylate cyclase stimulator

guanylate cyclase stimulator ADRs

tetratogenic increased mortality if pulm HTN was ass w/ idiopathic interstitial pneumonias ADRS: significant HOTN, dizziness, dyspepsia, HA 3A4 substrate smoking can reduce levels!

guanylate cyclase stimulators cannot be combined with what due to significant HOTN?

PDE-V inhibitors

direct vasodilation of pulmonary arteries and inhibition of platelet aggregation

prostacyclin analogs: epoprostenol, iloprost, treprostinil

prostacyclin analogs are used in

WHO III-IV idiopathic PAH

prostacyclin analogs ADRs

half life 3-5 minutes only stable for 8 hours -- performed in ICU setting tachy, flushing, HOTN, dizziness, HA, injection site reaction, anxiety, agitation, N/V, diarrhea, anorexia, myalgia, bone pain

abrupt interruption with prostacyclin analogs can cause

deterioration and death

caution with prostacyclin analogs and drugs that have antiplatelet or blood pressure lowering effects because

Ilioprost is an

inhaled prostacyclin, used for WHO class III-IV immediate access to medication is always necessary

Ilioprost ADRs

flushing, HOTN, HA, trismus, jaw pain

iloprost could induce what in hyperactive airways

bronchospasm

treprostinil must be administered with what

a high fat meal -- use with caution with underlying respiratory disorders and abrupt withdrawal

alcohol increases/decreases absorption of oral dosage of treprostinil

increases

selexipag

prostacyclin IP receptor agonists -- selectively agonizes receptors

Selexipag is used in class

II or II patients -- avoid use with hepatic impairment ADRS: flushing, HA, N/V/D, myalgia, jaw pain

greater experience to use

combo therapy

ambition trial:

ambrisentan + tadalafil

Compass-2:

bosentan + sildanefil

Griphon

selexipag + ERA +/- PDE-5i

43% reduction in all cause mortality at 3 years from

initial combo therapy

seraphin

+ macitentan

patent

riociguat added to ERA

respite

riociguat added to PDE-5i

replace

PDE-5i --> riociguat

sequential add on therapy improves outcomes when

monotherapy is insufficient

supportive pulm HTN therapy

diuretics (RV failure/fluid retention) oxygen oral anticoagulants anemia + iron status other CV agents

chronic cough, frequent lung infections, wheezing and shortness of breath, progressive obstruction, pancreatic insufficiency, steatorrhea, cirrhosis, infertility, digital clubbing, and sinus infections

elevated immunoreactive trypsinogen (IRT) levels on newborn screening

What are dx tips for CF

genetic testing, sweat test, pulmonary function tests

airway clearance techniques

chest physiotherapy high frequency chest wall oscillation positive expiratory pressure devices

inhaled treatments for CF

1) bronchodilators = albuterol (relieve bronchospasm + improve airflow) 2) Mucolytics = dornase alfa, hypertonic saline (breaks down mucus) 3) inhaled antibiotics = tobramycin, aztreonam (treat chronic, especially pseudomonas) 4) inhaled steroids

interferes with bacterial protein synthesis, utilized in patients > 6 and p. aeruginosa consistently present, dosed 2x daily in 28 day doses

tobramycin

tobramycin ADRs

bronchospasm, productive cough, rhinitis, oto/nephrotoxicity

monobactam that inhibits bacterial cell wall synthesis by binding penicillin-binding proteins which inhibit peptidoglycan syntehsis in cell walls -- > 6 yo patients + p aeruginosa present in culture 3x daily in cycles of 28 days ** pre-treat with bronchodilator **

aztreonam

What are ADRs of aztreonam?

throat pain, cough, nasal congestion, bronchospasm

Oral and IV antibotics for CF

tailored to specific pathogens -- combo of beta-lactams, AGs, FQs

cleaves DNA reducing mucous viscosity

dornase alfa 1x daily , > 6 years caution with FVC <40% ADRS: cough, pharyngitis, rhinitis, CP, voice disorder

managing CF exacerbations

- abx (2+) - steroids - contact precautions - clean/disinfect nebulizers - UTD vax

maintaining --- ---- leads to better FEV1 and survival

normal weight 2-20 = BMI at or above 50th percentile >20 female >22, males >23

pancreatic enzyme replacement therapy

combo lipase, amylase, protease (Creon, pancraze) dissolve in basic pH of duodenal high doses = colonic stricture ADRs = HA, abdominal pain, LAD, congestion

CFTR modulators

target underlyng defect in CFTR protein, improving chloride transport correctors = ivacaftor, lumacaftor potentiators = ivacfactor, increase function combo (trikafta) specific genetic mutations

potentiates epitheleal cell chloride ion transport of defective cell-surface, 2x daily oral therapy, major substrate of 3a4

ivacaftor

ivacaftor administration with --- --- will increase abdorption

high fat

ivacaftor warnings

risk for hepatotoxicity, increased risk for cataracts in pediatrics others: HA, diarrhea, abdominal pain, nausea, upper RTI, nasal congestion, oropharyngeal pain

improves conformational stability resulting in increased processing and trafficking, 2x daily oral therapy, with fat foot

lumacaftor

lumacaftor warnings

discontinue if AST/ALT >5x normal limit or >3x with increases in bilirubin - increases in BP - menstrual irregularitiy - increased CK

Also to consider in CF --

gene editing aproaches -- CFTR gene RNA therapies future directions

other CF meds

beta agonists oral NSAIDs for <18 azithromycin inhaled ICS oral steroids leukotrine modifiers inhaled anticholinergics

most CF patients live into their

40s and 50s