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Flashcards in Pulmonary Hypertension Deck (19):
1

WHO clinical classifiation of PH

group 1 - pulmonary artery hypertension

group 2 - PH owing to left heart disease

group 3 - PH owing to lung diseases or hypoxia

group 4 - chronic thromboembolic PH

group 5 - PH with unclear multifactorial mechanisms

2

drivers of group 1 PAH rise in PVR

vasoconstriction

remodeling of pulmonary vessel wall

in situ thrombosis

inflammation

3

drivers of WHO group 2 PH

passive oxygen congestion of pulmonary vasculature by back up of blood from left heart disease

out of proportion PH or persistent PH even after diuresis and decreasing left heart filling pressures - fixed vascular remodeling

4

drivers of WHO group 3 PH

hypoxic vasoconstriction

endothelial cell dysfunction with imbalance of vasodilators and vasoconstrictors

destruction of capillary bed

5

drivers of WHO group 4 CTEPH

chronic clot in pulmonary arterial bed

6

common pathologic findings of WHO group 1 PAH

smooth muscle hypertrophy

neointima formation and neovascularization

in situ thrombosis inflammation

distal extension of smooth muscle into non-muscular PA

endothelial cell proliferation (monoclonality)

7

common pathologic findings of WHO group 2 and 3

pulmonary arterial side medial thickening of muscular arteries

no endothelial proliferation

smooth muscle hypertrophy

also see changes of the venous side

occlusive venopathy with fibrous intimal thickening

lymphatic dilation and congested alveolar capillaries

8

clinical signs of PAH

accentuated second heart sound

notable heart murmur - tricuspid valve regurgitation

RV lift

RV failure - right sided third heart sound, JVD, hepatomegaly, peripheral edema, ascites

9

hemodynamic and clinical course of PAH

constant incrase in PVR

PAP increases and then falls off with CO

CO falls off first

BNP starts being released as CO begins to fall

10

What is the best method of diagnosis for evaluating Chronic Thromboembolic Pulmonary Hypertension?

V/Q scan

normal scan makes CTEPH unlikely

greater than one segmental sized or larger mismatched perfusion defect sween with CTEPH

11

What is the gold standard test for pulmonary hypertension?

right heart catheterization

definiteive diagnosis - direct measurement of RAP, PAP, PCWP, CO

calculation of PVR, CI

12

Why is characterizing the type of PH important?

therapies differ drastically between groups

incorrect treatment can worsen a patient's clinical status

PAH treatments are costly

13

treatment of group 1 PAH

endothlin blockers

nitric oxide promoters

prostacyclin pathway blockers

14

endothelin pathway blockers

ambrisentan

bosentan

macitentan

15

nitroc oxide promoters

sildenafil

tadalafil

riociguat

16

prostacyclin pathway blockers

epoprostenol

treprostinil

iloprost

17

treatment of WHO Group 2 PH

optimize heart failure medications

diuresis

maybe PDE-5 inhibitors

18

treatment of WHO group 3 PH

optimize lung disease medications

reverse hypoxia

ihnaled prostacyclines

PDE-5 inhibitors

19

treatment of WHO group 4 PH