08.25 - Pulmonary Hypertension (Muthiah) - Questions Flashcards

1
Q

Test critical to confirming and quantifying PAH

A

Right Heart Catheterization

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2
Q

Commonest cause of pulmonary HTN

A

Pulmonary Venous HTN (post capillary)

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3
Q

Definition of PAH

A

Mean PAP > 25mmHg at rest (Mean PCWP&LVEDP <15)

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4
Q

Alveolar hypoxemia causes what remodeling change

A

Vasoconstriction –> Chronic thickens vessels

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5
Q

Group 1 PAH

A

iPAH, Heritable (BMPR2), HIV-related, CTDz

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6
Q

Group 4 PAH

A

Chronic Thromboembolic

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7
Q

Group 5 PAH

A

Multifactorial etiology

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8
Q

Most important vasoconstrictor

A

Endothelin 1

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9
Q

Prostacyclin vs TXA2

A

Anti-coagulant vs Coagulant

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10
Q

Which Collagen Vascular Disease is most often associated with PAH

A

Systemic Sclerosis

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11
Q

Importance of Raynaud’s

A

Conditions with Raynauds often have PAH

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12
Q

Thrombocytosis and PAH

A

Increased 5-HT

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13
Q

10% of what hemoglobinopathy is associated with PAH

A

Sickle Cell - Free Hb and Iron implicated

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14
Q

Gene and protein to remember for Familial PPH

A

PPH1 –> BMPR2

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15
Q

History of what emphasized class of medications is important to obtain in patient with suspected PAH?

A

Stimulants, such as Fen-Phen, Cocaine, etc

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16
Q

TXA and Prostacyclin in PPH

A

Increased TXA, Decr Prostacyclin

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17
Q

Nitric Oxide promotes vasodilation and

A

inhibits SM growth

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18
Q

Endothelin 1 in PAH (and 5-HT)

A

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

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19
Q

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

A

Endothelin 1 in PAH

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20
Q

Role of Serotonin in PAH

A

Vasoconstriction and SM Cell proliferation; Plasma concentrations higher in PAH

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21
Q

Role of V-gated K Channels in PAH

A

Lower mRNA in PAH - Low channel current - Incr in intracellular Ca2+ - Vasoconstriction

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22
Q

Why is intracellular Ca2+ incr in PAH

A

Low v-gated K channels

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23
Q

Appetite suppressant drugs may generate PAH by

A

blocking v-gated K channels

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24
Q

Role of appetite suppressant drugs

A

May generate PAH by blocking v-gated K channels

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25
Mean age at dx for PPH
36
26
Good screening test for PPH
Echocardiography
27
In an RHC report, the parameter that distinguishes PAH from PVH:
Pulmonary Capillary Wedge Pressure
28
Relevance of Pulmonary Capillary Wedge Pressure
Proportional to LAP and LVEDP
29
Prognosis and percent of patients that are "vasoreactive"
5-6%; Good prognosis
30
During RHC, which is the agent of choice for vasoreactivity testing?
Nitric Oxide
31
PFT findings in PAH
Normal FEV1, FVC, TLC; Reduced DLCO
32
PFT findings in Emphysema
Reduced FEV1/FVC; Incr TLC; Reduced DLCO
33
Reduced FEV1/FVC; Incr TLC; Reduced DLCO
PFT findings in Emphysema
34
Reduced FVC, TLC, DLCO
Interstitial Lung Disease
35
PFT findings in Interstitial Lung Disease (FVC, TLC, DLCO)
Reduced FVC, TLC, DLCO
36
PFT findings in PE
Normal FEV1, FVC, TLC; Reduced DLCO
37
PFT findings in Anemia
Normal FEV1, FVC, TLC; Reduced DLCO
38
Normal FEV1, FVC, TLC; Reduced DLCO (name 3 conditions)
PAH, PE, Anemia
39
Drug for PAH Stage 4
Epoprostenol (Flolan)
40
How does Pulmonary Artery Pressure change through PAH progression
Increases, Levels off, Decreases as heart fails
41
How does CO change throughout PAH progression
Constant, Decreases, Drops as heart fails
42
How does PVR change throughout PAH progression
Constantly increases
43
EKG findings in PAH
Tall p wave in 2; Tall R in aVR and V1; RV string pattern
44
RV String Pattern
ST depression with U wave inversion
45
What defines a "response"?
>20% reduction in PAP and PVR
46
Fall in PAP in response to inhaled NO indicates
Patient will also respond to CCB's
47
MOA of Riocuguat
Incr cGMP by stimulating Guanylate Cyclase
48
When is Epoprostenol used
Functional class 4 patients
49
3 major effects of Epoprostenol
Vasodilation; Inhibit platelet aggregation and SM proliferation (unique)
50
Which drug can reverse the remodeling in PAH
Epoprostenol
51
Which PPH patients can benefit from Epoprostenol
Both responders and non-responders
52
How does Epoprostenol cause vasodilation
Incr intracellular cAMP
53
3 side effects of Epoprostenol
Jaw pain, Diarrhea, Arthralgias
54
2 adverse effects of Bosentan
Elevated aminotransferase; Teratogenic
55
Anticoagulant to use in PAH
Warfarin
56
Mean survival for PAH
3 years from dx
57
2 proven therapies to improve survival in PAH
Epoprostenol, Bosentan, Maybe Anticoagulation (warfarin)
58
MOA of Bosentan
Endothelin Receptor Antagonist
59
MOA of Macitentan
Endothelin Receptor Antagonist
60
MOA of Sildenafil/Tadalafil
PDE 5 inhibitor
61
MOA of Epoprostenol
Prostacyclin Analog
62
MOA of Ilopros
Prostacyclin Analog