Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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
Tadalafil
- 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
Riociguat
- 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
Riociguat AE/Monitoring
- 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
Prostanoids
- Prostaglandin I2: prostacyclin induces relaxation of vascularsmooth muscle by stimulating cAMP production - Powerful inhibitor of platelet aggregation - [PGI2] are decreased in PAH
29
Epoprostenol
- 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
Epoprostenol Administration
- 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
Epoprostenol DI/CI/AE
- 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
Treprostinil
- 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
Treprostinil AE/Monitoring
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
Inhaled Trepostinil
- 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
Oral Treprostinil
- 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
Orenitram DI/Counseling
-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
Iloprost
- 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
Selexipag
- 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
Combination Therapy
- Ambrisentan + Tadalafil - Trial studied its use in naive PAH patients - Improved outcomes versus monotherapy of either medication on its own
40
PAH Longitudinal Eval
- 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.
© 2025 Bold Learning Solutions. Terms and Conditions