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PAH Flashcards

1
Q

What is the life expectancy of untreated PAH?

A

3 Y

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2
Q

What is the PAH?

A

HTN in pulmonary artery

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3
Q

What is the normal PAP? During exercise?

A

N: 8-20 mag at rest
PH: mPAP > at rest, >30 during excersise

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4
Q

Pathophysiology of PAH?

A
  1. Imbalance in vasoconstrictors and dilators
  2. Imbalance in cell proliferation and apoptosis
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5
Q

How are vasoconstrictors affected in PAH?

A

ET1 and TXA2 increase

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6
Q

How are vasodilators affected in PAH?

A

Prostocyclins increase inhibits platelet aggregation and antiproliferation

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7
Q

How can PAH cause imbalances in cell proliferation and apoptosis?

A

Increased smooth muscle cells in pulmonary artery → further artery narrowing → more difficult for right ventricle (RV) to pump blood into lungs → enlarged RV and right heart failure (HF)

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8
Q

What are risk factors of PAH?

A
  1. Anorexigens
  2. HIV infection
  3. increased pulmonary flow
  4. Portal HTN
  5. Connective tissue destruction
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9
Q

What are the diagnosis tests for PAH?

A
  1. Doppler Echo
  2. Echocardiography
  3. Right heart catheterization (RHC)
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10
Q

What is Doppler Echo?

A
  1. Noninvasive
  2. Detects increase pulmonary pressures
  3. Can’t definitively diagnose
  4. First if PH is suspected
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11
Q

What is echocardiography?

A
  1. Can detect specific cardiac causes
  2. Used to assess treatment effects and progression
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12
Q

What is RHC?

A
  1. Definitive
  2. Used to evaluate clinical worsening
  3. Asses vasoreactivity
  4. mPAP≥25 with PCWP≤15 and PVR≥3
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13
Q

What are the classification systems of PAH?

A

Clinical and functional

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14
Q

What is the difference between clinical and functional classifications?

A

Clinical: classifies etiologies (Groups 1-5)
Functional: classifies symptoms and severity (Class1-IV), increases as disease worsens

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15
Q

What falls under group 1 PAH?

A

Drug induced PAH

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16
Q

What is class 1?

A

Symptom free when physically active or resting

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17
Q

What is class 2?

A

No symptoms at rest but some discomfort and SOB

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18
Q

What is class 3?

A

Resting may be symptom free but limitations due to SOB or feeling tired

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19
Q

What is class 4?

A

Symptoms at rest and severe symptoms with activity

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20
Q

What is the purpose for monitoring parameters?

A

Determine baseline, functional classification, and monitor severity, response to therapy, risk level, prognosis, progression

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21
Q

How would we monitor parameters?

A
  1. Pulmonary unction tests
  2. ABGs
  3. 6MWD
  4. Serial biomarkers (bnp, LFTs)
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22
Q

What are the non-pharms for PAH?

A
  1. Sodium restriction <2.4 g/day
  2. Influenza and pneumonia vaccinations
  3. Oxygen to maintain sats >90%: Particularly if PaO2 <60
  4. Cardiopulmonary rehab
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23
Q

What are the types of pharm therapies?

A
  1. Supportive therapy
  2. Targeted therapy
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24
Q

What are examples of supportive therapies?

A
  1. Warfarin
  2. Loop diuretics for volume overload
  3. Digoxin to improve HR and CO
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25
Q

Characteristics of warfarin supportive therapy?

A
  1. Controversial (varies by MD)
  2. INR goal 1.5-2.5
  3. May consider if on long-term IV prostaglandins due to risk of catheter thrombosis
  4. NOT recommended if portal HTN or HIV is the etiology
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26
Q

What is the rationale behind warfarin?

A

HF, immobility, thrombotic changes in pulmonary microcirculation

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27
Q

Characteristics of digoxin supportive therapy?

A
  1. Adjunct with diuretics in R HF
  2. Arrhythmias
  3. Goal level 0.5-0.8 ng/mL
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28
Q

What are the classes of targeted therapy?

A
  1. Prostacyclin Analogues and Agonists
  2. Endothelin Receptor Antagonists
  3. Phosphodiesterase-5 Inhibitors
  4. Soluble Guanylate Cyclase Inhibitors
  5. (supplement endogenous vasodilators, inhibit endogenous vasoconstrictors, reduce endothelial platelet interactions)
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29
Q

What is the purpose for targeted therapy? What do they include?

A

Addresses endothelial abnormalities seen in PH

  1. Endogenous vasodilators
  2. Inhibitors of vasoconstriction
  3. Reducers of endothelial platelet interaction and thrombosis
30
Q

What are loop diuretic used for?

A

Volume overload

31
Q

What are therapeutic pathways affected by PAH?

A
  1. Prostacyclin
  2. Endothelian
  3. Nitric oxide
  4. guanylyl cyclase pathway
32
Q

Mechanism of prostacyclin pathway?

33
Q

Mechanism of endothelium pathway?

34
Q

Mechanism of nitric oxide pathway?

35
Q

Mechanism of guanylyl cyclase pathway?

36
Q

What is the purpose for vasoreactivity testing?

A

Determine if patient will respond to treatment with calcium channel blockers

37
Q

How do you administer vasoreactivity testing? Responders? Nonresponders?

A

Administer short acting VDs

Responders: mPAP falls by at least 10 mm Hg to absolute value <40 mm Hg (treatable with CCB)
Non: must treat with targeted therapies

38
Q

What drugs are used for the vasoreactivity test?

A
  1. inhaled NO
  2. IV epoprostenol
  3. IV adenosine
39
Q

What are the preferred drugs for PAH? What class are they?

A

DHP (amlodipine and nifedipine)

40
Q

What are the disadvantages of diltiazem? CIs?

A
  1. Can use with concomitant tachycardia
  2. Verapamil not recommended due to higher negative inotropic effects
  3. Avoid all non-DHPs if LV systolic dysfunction present
41
Q

What are the doses of amlodipine? Diltiazem? Nifedipine?

A

Amlodipine: 20-30 mg/d
Diltiazem: 240-720 mg/d
Nifedipine: 120-140 mg/d

42
Q

What is the most common ADR for CCB?

A

Peripheral edema

43
Q

What happens is CCBs stop working for PAH?

A

DC and switch to another agent
Can add on another drug to enhance its effects

44
Q

Contraindications of CCBs?

A
  1. if RV dysfunction, reduced CO, or FC IV symptoms
  2. if vasoreactivity testing not performed
  3. Increase morbidity
45
Q

Epoprostenol

A

Flolan, Veletri
Class: III-IV
Routes: IV

46
Q

Treprostinil

A

Remodulin
Class II-IV: IV, SC
Class II-III: PO
Class III: Inhaled

47
Q

Iloprost

A

Ventavis
Class: III-IV
Form: Inhaled

48
Q

Dosing of Selexipag?

A

Start with 200 mcg po BID; increase by 200 mcg BID weekly; max 1600 mcg BID

49
Q

Effects of prostacyclin analogues?

A

vasodilations and platelet inhibiton
2. ↓ PGI2 synthase and ↓ excretion of PGI2 metabolites

50
Q

Effects of prostacyclin analogues?

A

vasodilations and platelet inhibiton
2. ↓ PGI2 synthase and ↓ excretion of PGI2 metabolites

50
Q

Advantages of inhaled prostacyclin analogues?

A

selective pulmonary vasodilation with fewer systemic effects

51
Q

Class warnings with prostacyclin analogues?

A

Rebound PH with abrupt discontinuation, Increased risk of bleeding, CYP interactions

51
Q

Class warnings with prostacyclin analogues?

A

Rebound PH with abrupt discontinuation, Increased risk of bleeding, CYP interactions

51
Q

ADRs with prostacyclin analogues? Inhalation? Oral?

A

Flushing, HA, N/V/D, jaw pain, thrombocytopenia, hypotension

Inh: cough, throat irritation, bronchospasm
Oral: abdominal discomfort

51
Q

Selexipag

A

Uptravi
Class II-III
ADRs: Anemia, hyperthyroidism
Interruptions >3 days requires retitration

52
Q

Endothelin receptor antagonist MOA? ADRs? BBW? Monitoring?

A

Prevents vasoconstriction
HA, flushing, hypotension, increased LFTs, anemia, edema
Teratogenicity
(Greater dose=greater improvement)
Monitoring: CBC, LFTs, bilirubin, pregnancy test

53
Q

ERA types?

A

Bosentan
Macitentan
Ambrisentan

54
Q

Bosentan

A

Tracleer
Class II-IV
BBW: hepatotoxicity
CIs: glyburide or cyclosporine
DDIs: CYP3A4 an 2C9 inhibitors/inducers

55
Q

Macitentan

A

Opsumit
Class II-IV
DDIs: CYP3A4 an 2C19 inhibitors/inducers

56
Q

Ambrisentan

A

Letairis
Class II-III
CIs: Idiopathic pulmonary fibrosis
DIs: Cyp3A4, CYP2C19, Pgp

57
Q

PDE5i MOA? CIs? Warnings? ADRs? DDIs?

A

Relaxation and vasodialtion
CI: Nitrates, riociguat
Warnings: hearing loss, vision loss, priapism, hypotension
ADRs: HA, flushing, epistaxis, dyspepsia, diarrhea, myalgia
DDIs: nitrates, CYP3A4 drugs, ED drugs

58
Q

Sildenafil

A

Revatio
Forms: PO, IV
Class: II-III
CIs: nitrates riociguat

59
Q

Tadalafil

A

Adcirca
Forms: PO
Class: II-III
CIs: nitrates riociguat

60
Q

sGCS example?

A

Riociguat (Adempas)

61
Q

Riociguat dosing

A

Adempas
Dosing: Start with 0.5-1 mg TID; increase Q2 weeks by 0.5 mg TID if SBP >95
Max dose 2.5 mg TID with higher doses used in smokers

62
Q

Riociguat

A

Adempas
BBW: teratogenicity
CI: pregnancy, nitrates, PDE5i
Warnings: Hypotension, bleeding
ADRs: HA, hypotension, hemoptysis, dizziness, dyspepsia, N/V/D, anemia
DDIs: smoking, antacids, CYP3A4, 2C8, Pap drugs

63
Q

How does Adempas differ from other PAH meds?

A

Only drug approved for Group 4 PH (CTEPH) with residual CTEPH after surgical treatment or inoperable CTEPH

64
Q

What are PAH goals of therapy?

A
  1. improve exercise tolerance
  2. Improve symptoms
  3. Improve QOL
  4. Prevent progression
  5. Improve survival
  6. Preserve RV size and function
  7. Normalize bnp
65
Q

How do you improve exercise tolerance?

A
  1. Preserve 6MWD to 380 m or more
  2. Cardiopulmonary exercise testing goals: peak O2 consumption >15 ml/kg/min and ventilator equivalent for CO2 <45 L/min
66
Q

How do you improve symptoms?

A

Achieve and maintain FC I or II

67
Q

What is PAH therapeutic selection according to CHEST?

A

Severity should be evaluated using a combination of WHO FC

68
Q

Med determination algorithm?

Therapeutics I (32 decks)

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