Pulmonary Hypertension Flashcards Preview

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Flashcards in Pulmonary Hypertension Deck (34):
1

Key features of pulmonary circulation

High flow, low resistance, high capacitance

2

Pulmonary VR is what fraction of SVR

1/15 due to large cross-sectional area

3

What are some causes of pulmonary hypertension

LV dysfuction, left atrial disease, valvular disease
COPD
Chronic thromboembolic disease

4

What is Pulmonary arterial hypertension

It's exactly what it sounds like and is not accounted for by any of the previous causes

5

Primary pulmonary arterial hypertension

Is idiopathic in nature

6

Some broad categories of things that cause PAH

Heritable, drug and toxin induced, associated with HIV/schisto/connective tissue disease

7

General definition of pulmonary hypertension

Resting mean pulmonary artery pressure >25

8

Definition of pulmonary arterial hypertension

Resting mean pulmonary artery pressure > 25, Left sided pressures 3 wood units

9

Some vascular changes associated with PAH

Vasoconstriction. Smooth muscle and endothelial cell proliferation. Thrombosis

10

Vascular remodeling associated with PAH

High flow low resistance system becomes low flow high resistance due to smooth muscle hypertrophy, in situ thromboses, intimal proliferation. Plexiform lesions

11

Pathology of pulmonary hypertension reveals

Medial thickening and compressed lumen. Intimal fibrosis, , and plexiform lesion formation

12

Mechanisms of PAH

Homeostatic imbalance of vascular effectors that favor vasocontriction, thrombosis and mitogenesis (growth).

Environmental and genetic factors too.

13

Prostacyclin

A vasodilator, inhibitor of platelet activation and is antiproliferative. Reduced in PAH in comparison to TXA2

14

Thromboxane A2

Released by platelets, vasoconstrictor. Levels are increased in PAH.

15

Serotonin in PAH

Acts as a vasoconstrictor, promotes smooth muscle cell hypertrophy. Elevated plasma levels in PAH patients

16

Endothelin 1

Potent vasoconstrictor that promotes smooth muscle cell proliferation. Levels increased in PAH. Levels are inversely proportional to magnitude of pulmonary blood flow.

17

NO

Vasodilator and inhibitor of platelet activation and smooth muscle cell proliferation. Endothelial NOsynthase is decreased in PAH

18

Hypoxia effect on systemic circulation and on pulmonary circulation

In systemic, hypoxia induces vasodilation.

In pulmonary, hypoxia induces vasoconstriction.

19

How does hypoxia contribute to PAH

Can lead to PA vascular remodeling

20

What drugs are associated w/PAH?

Fen-phen. Cocaine/methamphetamines (associated with medial hypertrophy of pulmonary arteries)

21

What genetic defects are associated with PAH

Mutations in the TGFß receptor pathway. Bone Morphogenic protein receptor (BMPR2) type 2 has been shown to modulate the growth of vascular cells. Incomplete penetrance though.

Also in serotonergic pathway. Depending on receptorq

22

Hemodynamic progression of PAH during presymptomatic/compensated period

Cardiac output still normal. Pulmonary artery pressure increasing, PVR increasing, right atrial pressure still low.

23

Progression during symptomatic/decompensating period

CO dropping due to increased PAP and PVR. RAP increasing because it can't handle increased volume backing up from PA

24

Progresion during the declining/decompensated period

CO drops dramatically and causes right heart failure.
PVR increases still. RAP increases dramatically. PAP actually drops because RV can't pump into pulmonary artery.

25

What is an ominous sign that PAH has gotten really bad

If pulmonary artery pressure drops during severe disease. This signals that the right heart cannot pump into the severely stenotic pulmonary arteries.

26

prognosis of PAH

Not good, 3 year survival is 58%

27

Symptoms of PAH

Dyspnea, Fatigue, Chest pain, near syncope, edema,

28

Signs of PAH

Elevated JVD, Tricuspid regurg due to increased PA pressures, grahm-steel murmur, Accentuated p2, S3 gallop on the right side, peripheral edema, hepatomegaly

29

EKG findings

Not sensitive enough to be a screening tool. However, frequently RVH -- > big R wave in anterior prevordials (V1 v2 v3)

30

Roentgenogram findings for PAH

Enlargement of central pulmonary arteries, reduced caliber of peripheral vessels. Cardiomegaly w/ RV enlargement, enlargement of R and L PAs.

31

Best diagnostic test for PAH?

TTE

32

How to do the workup for PAh

Make sure that PAH isn't just pulmonary hypertension. Look for chronic lung disease, look for infection, look for thrombi.

33

What should patients avoid doing with PAH?

Keep SaO2 high. Avoid vasoconstrictors, avoid valsalva, avoid pregnancy.

34

Treatment for PAH

PDE5 inhibitors, Prostacyclin derivatives to increase cAMP. Endothelin receptor antagonists.