Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

850 > Pulmonary Arterial Hypertension > Flashcards

Pulmonary Arterial Hypertension Flashcards

1
Q

Pulmonary Arterial Hypertension

A
  • PAH: sustained elevated pulmonary arterial pressure (>= 25 mmHg) with a mean PCWP or LVEDP =< 15 mmHg
  • Ultimately leads to right ventricular failure and death
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

PAH Symptoms

A
  • Fatigue
  • Dyspnea
  • Weakness
  • DOE
  • Less common symptoms: chest pain, near-syncope/syncope, peripheral edema, and palitations
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

WHO Group 1 PAH

A
  • Idiopathic PAH
  • Familial PAH
  • Associated with APAH: connective tissue disease, portal HTN, HIV, drugs
  • Associated with significant venous or capillary involvement
  • Persistent PAH of newborn
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

WHO Group 2 PAH

A
  • PAH with left heart disease
  • Left-sided atrial or ventricular heart disease
  • Left-sided valvular heart disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

WHO Group 3 PAH

A
  • PAH associated lung disease and/or hypoxemia
  • COPD
  • Interstitial lung disease
  • Sleep-disordered breathing
  • Chronic exposure to high altitude
  • Developmental abnormalities
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

WHO Group 4 PAH

A
  • Due to chronic thombotic disease or embolic disease (CTEPH)
  • Thromboembolic obstruction or proximal or distal pulmonary arteries
  • Nonthrombotic pulmonary embolism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

WHO Group 5 PAH

A
  • Sarcoidosis
  • Pulmonary Langerhans
  • Lymphangiomatosis
  • Compression of pulmonary vessels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

PAP

A
  • Pulmonary Arterial Pressure
  • PAP is generated by right ventricle, ejecting blood into pulmonary circulation
  • Acts as a resistance to output from RV
  • Pulmonary vasculature is low resistance system
  • PAP = (CO * PVResistance) + PVPressure
  • PAP ~ 15, PVP ~8
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Pulmonary Vascular Changes

A
  1. Vasoconstriction - imbalance between vasodilators and vasoconstrictors
  2. Smooth muscle cell and endothelial cell proliferation: imbalance between growth inhibitors and mitogenic factors
  3. Thrombosis: imbalance between antithrombotic and prothrombotic determinants
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

PAH Diagnostic Evaluation

A
  1. History: family and physical examination
  2. Electrocardiography, chest radiography, pulmonary-fxn test
  3. Echocardiography
  4. Ventilation/perfusion scan, chest CT
  5. CBC, HIV-1 antibody, LFTs, thyrotropin, antinuclear antibody
  6. Exclude other secondary causes
  7. Right heart catheterisation with vasodilator testing: NEEDED to confirm PAH
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

WHO Functional Classifications

A
  • I: No limitation of physical activity
  • II: Mild limitation: no discomfort at rest, normal activity causes increased dyspnea, fatigue, chest pain presyncope
  • III: Marked limitation of physical activity, no discomfort at rest, less than ordinary activity causes dyspnea, fatigue, chest pain, presyncope
  • IV: unable to perform any physical activity, signs of RV failure, dyspnea/fatigue present and rest and symptoms increases by most activity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

PAH Markers for Disease Severity

A
  • BDI (Borg Dyspnea Index): 0 = no impairment, 10 = severe impairment
  • 6-MWT (6 minute walk test): measure of exercise level reflective of everyday life
  • Cardiopulmonary hemodynamic variables: mPAP, PVR, CO via right heart catheterization
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

NonPharm General Treatment Measures

A
  • Oxygen: want to maintain O2 saturations >90%, helps fight hypoxic vasoconstriction
  • Diet: Na (<2500 mg/day) strongly encouraged and fluid restriction when necessary
  • Immunizations: influenza, pneumococcal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pharm General Treatments

A
  • Diuretics: indicated in patients with evidence of RV failure (edema, ascites, elevated JVD), maintain normal volume status is the goal
  • Monitor: vitals, renal fxn, and serum electrolytes

-Digoxin: may be beneficial to patients with refractor right ventricular failure and/or atrial dysrrhythmias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Oral Anticoagulants

A
  • Microscopic thrombosis has been documented with IPAH and right-sided HF increases risk of PE
  • Patients with IPAH should receive anticoagulation with warfarin
  • Still consider anticoagulation if PAH is related to other conditions like scleroderma or congenital HD
  • Use other anticoagulants if PAH patient has an indwelling catheter
  • Target INR: 1.5-2.5
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Acute vascular Testing

A
  • Vasodilator testing to determine response to vasodilators
  • If responsive, better survival with long-term CCB use
  • Acute vasodilator testing must be done during a right heart catheterization
  • Positive response defined as a drop in PAP >= 10 mmHg to =< 40 mmHg with unchanged to increased CO
  • Patients with IPAH should undergo this testing as well
  • Use short-acting agents like IV epoprostenol, IV adenosine, or inhaled NO for testing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

CCB

A
  • ~10% have favorable vasodilator response
  • Nifedipine XL, diltiazem, or amlodipine used the most (avoid verapamil, (-) inotrope)
  • Start low and titrate dose as tolerated
  • Monitor: @ 3 months for symptoms/vitals/edema,
  • Sustained response to CCB therapy is defined as being FC I or II
18
Q

ERAs

A
  • Endothelin-1 Receptor Antagonists
  • ET-1 is a potent, endogenous vasoconstrictor and smooth-muscle mitogen that is overexpressed in plasma and lung tissue of PAH patients
  • Two receptors: ETa and ETb
  • Eta: activation facilitates vasoconstriction and proliferation of vascular smooth-muscle cells
  • ETb: involve clearance of ET-1, may cause vasodilation and NO release
19
Q

Bosentan

A
  • Tracleer
  • Block Type A and B receptors
  • CI: pregnancy (test monthly), hepatotoxicity, glyburide and cyclosporin
  • Check LFTs every month
  • CYP2C9 and 3A4 substrate
20
Q

Ambrisentan

A
  • Letairis
  • Selective for ETa receptor
  • CI: pregnancy (test montly)
  • CYP3A4 (major), 2C19 (minor), and P-gp substrate
21
Q

Macitentan

A
  • Opsumit
  • Non-selective A/B receptor antagonist
  • CI: pregnancy (test monthly)
  • CYP3A4 (major) and 2C19 (minor) substrate
22
Q

ERA Warnings/AE/Monitorign

A
  • Warnings: DEcrease Hgb/HCT, fluid retention, decreased sperm counts
  • AE: headache, URI, flushing, hypotension
  • Monitoring: LFTs, Hgb/Hct, pregnancy tests
23
Q

PDE5-I + PAH Treatment

A
  • NO is a pulmonary vasodilator which is mediated trough cGMP and rapidly degraded by PDE
  • PDE5 is a predominate isoform in the lung that metabolizes cGMP (upregulated in PAH)
  • By inhibiting PDE5, increases of cGMP occur and enhance NO vasodilation
24
Q

Sildenafil

A
  • FDA-indicated for PAH WHO Group I to improve exercise ability
  • Monitor: BP, symptoms, vision, hearing
  • Interacts with nitrates, CYP3A4 inhibitors/inducers, and alpha-blockers/other vasodilators (hypotensive effects)
25
Q

Tadalafil

A
  • Indicated for treatment of PAH (WHO Group I) to improve exercise ability
  • Monitor: BP, symptoms, vision, hearing
  • DI: nitrates, CUP3A4 inhibitors/inducers
  • Reduce dose by 1/2 in renal insufficiency (CrCl 31-80)
26
Q

Riociguat

A
  • Adempas
  • Guanylate cyclase (sGC) stimulator
  • Acts in synergy with and independently of endogenous NO to stimulate sGC
  • Indicated for those with CTEPH (WHO Group 4) and WHO Group 1 to increase exercise capacity
  • Available through REMS program
  • Start at lower dose if hypotensive
27
Q

Riociguat AE/Monitoring

A
  • AE: Headache, dyspnea, dizzy, N/V, diarrhea, anemia, GERD, Pregnancy Category X
  • Monitoring: BP, bleeding, symptoms, preggo test monthly
  • PK: Metabolized y CYP 1A1, 3a, 2C8, and 2J2
  • DI: CYP inhibitors and inducers, P-gp/BCRP inhibits, antacids, tobacco smoking
  • CI: nitrates, specific or non-specific PDE5I
28
Q

Prostanoids

A
  • Prostaglandin I2: prostacyclin induces relaxation of vascularsmooth muscle by stimulating cAMP production
  • Powerful inhibitor of platelet aggregation
  • [PGI2] are decreased in PAH
29
Q

Epoprostenol

A
  • Flolan
  • Naturally occuring prostaglandin with potent vasodilatory activity and inhibitory activity of platelet aggregation
  • Indicated for long-term IV treatment of IPAH and PH associated scleroderma in Class III-IV patients who don’t respond to conventional therapy
30
Q

Epoprostenol Administration

A
  • Given continuously IV through central venous catheter
  • Keep cold during infusion and protectfromlight
  • Veletri formulation is more stable and can last 2-3 days at times, protect from light still
  • Avoid abrupt D/C, symptom deterioration and perhaps death
31
Q

Epoprostenol DI/CI/AE

A
  • Start lose with dosing and slowly titrate up based on SE/tolerability (usually dose max between 20-40 ng/kg/min)
  • DI: risk of hypotension with other HTN drugs, antiplatelets/anticoagulants increase bleed risk, increases [digoxin]
  • CI: HFrEF
  • AE: HA, jaw pain while chewing, flushing, diarrhea, nausea, rash, aches/pains
  • Rare Delivery AE: sepsis, cellulitis, hemorrhage, and pneumothorax
32
Q

Treprostinil

A
  • Remodulin
  • Synthetic prostacyclin analog
  • Indicated for PAH patients with Class II-IV symptoms
  • Can be continuous IV or SC
  • Start slow and increase doses over first for weeks
33
Q

Treprostinil AE/Monitoring

A

AE

  • SC Infusion: infusion site pain/rxn, often severe and could need narcotics/D/C
  • IV infusion: line infections, sepsis, arm swelling, hematoma, and pain
  • General: diarrhea, jaw pain, vasodilation, and edema
  • Monitor: AEs, vital signs, and PAH symptoms
34
Q

Inhaled Trepostinil

A
  • Tyvaso
  • Indicated to PAH WHO Group I to improve exercise (mainly studied in FCIII group with bosentan or sildenafil use)
  • Use only with Tyvaso inhalation system
  • QID, separated 4 hours
  • Target maintenance dose: 9 breathes per session
  • AE: Cough, HA, throat irritation, nausea, flushing, syncope, bleeding, hypotension
35
Q

Oral Treprostinil

A
  • Orenitram
  • ER tablet who WHO Group 1 to improve exercise
  • Available in several dosing strengths
  • Increase dose every 3-4 days to max tolerability/symptom response
36
Q

Orenitram DI/Counseling

A

-DI: increased bleeding with anticoag, increased hypotension with anti-HTN, strong CYP2C8 inhibitors

Counseling

  • Avoid abrupt D/C (worsens symptoms)
  • Take with food
  • Swallow tablets whiole, don’t split/crush/chew
  • Tablet shell remains intact during GI transmit and will be eliminated in feces
  • Don’t take with alcohol
37
Q

Iloprost

A
  • Inhaled, Ventacis
  • Synthetic analog of prostacyclin’
  • For WHO Group I with Class III or IV symptoms
  • Given 6-9 times per day and slowly titrate to max tolerated dose
  • AE: vasodilation (Flushing), cough, HA, trismus (lockjaw), and insomnia
  • Monitoring: AE, vital signs, and PAH symptoms
38
Q

Selexipag

A
  • Uptravi
  • Non-prostanoid, IP prostacyclin receptor agonist
  • Different structure than prostacyclin, metabolized by CYP2C8 and 3A4
  • Oral tablet for WHO Group I to delay disease progression and reduce hospitalization risk
  • DI: Strong CYP2C8 inhibitors (gemfibrozil)
  • AE: HA, flushing, nausea, diarrhea, myalgia, jaw/extremity pain
39
Q

Combination Therapy

A
  • Ambrisentan + Tadalafil
  • Trial studied its use in naive PAH patients
  • Improved outcomes versus monotherapy of either medication on its own
40
Q

PAH Longitudinal Eval

A
  • Follow up based on therapy
  • IV: by phone in 1-3 weeks then qweek until symptoms subside
  • Oral: F/U every 1-2 months
  • Combination: F/U every 2-4 weeks
  • Assess: WHO-FC,, 6-MWT, Chem-7, AST/ALT if on ERAs INR if on warfarin, NT-proBNP

850 (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions