Lec 33 Pulmonary Hypertension Flashcards

1
Q

What types of vessels in pulm vasculatrue have most resistance?

A

small vasculature = arterioles and venules

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2
Q

What is flow/resistance of pulm circulation?

A

high flow

low resistance

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3
Q

What is capacitance of pulm circulation?

A

high capacitance to accommodate increased CO

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4
Q

What are the major causes of pulmonary hypertension?

A
  • left heart disease
  • chronic thrombotic or embolic disease
  • parenchymal lung disease or chronic hypoxemia
  • miscellaneous –> sarcoidosis
  • pulmonary arterial hypertension
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5
Q

What is the most common cause of pulmonary hypertension?

A

left heart disease

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6
Q

What are the major causes of pulmonary arterial hypertension?

A
  • idiopathic = primary pulmonary hypertension
  • heritable
  • drug/toxin induced
  • associated w/ other disease
  • persistant plum HTN of newborn
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7
Q

What is general definition of pulmonary hypertension?

A

resting mean pulm arterial pressure > 25 mmHg

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8
Q

What is general definition of pulmonary arterial HTN?

A

resting mean PAP > 25 AND

  • PCWP/LAP/LVEDP < 15 mmHg
  • pulmonary arteriolar resistance > 3 woods units
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9
Q

What 3 vascular changes happen in PAH?

A
  • vasoconstriction
  • smooth muscle and endothelial cell proliferation
  • thrombosis in situ
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10
Q

What is effect of vascular remodeling in PAH?

A

decrease flow

increase resistance

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11
Q

What are plexiform lesions?

A

hallmark of advanced pulmonary HTN

proliferation of cells that compromise lumen of small pulmonary artery

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12
Q

What are the mechanisms leading to PAH?

A
  • homeostatic imbalance of vascular effectors [vasoconstors, prothrombotic, mitogenic factors]
  • environmental

genetic

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13
Q

What is role of prostacyclin and thromboxane A2 in PAH?

A

prostacyclin = vasodilator, inhibits platelet activation

TXA2 = vasoconstrictor, platelet agonist

in PAH levels of TXA2 > > prostacyclin

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14
Q

What is role of serotonin in PAH?

A
  • vasoconstrictor
  • promotes smooth muscle cell hypertrophy
  • elevated in PAH
  • impaired serotonin reuptake in PAH patients
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15
Q

What is role of Endothelin 1 in PAH?

A
  • potent vasoconstrictor, stimulates smooth muscle cell proliferation

increased in PAH

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16
Q

What is role of NO in PAH?

A
  • vasodilator, inhibits smooth cell proliferation

- endothelial isoform of NO synthase decreased in pts with PAH

17
Q

What is role of hypoxia in PAH?

A
  • in systemic circulation –> hypoxia induces vasodilation
  • in pulm beds –> induces vasoconstriction [to improve V/Q mismatch]

chronic hypoxia –> structural remodeling, SMC proliferation

18
Q

What is role of anorexigens in PAH?

A
  • linked to higher risk for PAH

- fen-phen

19
Q

What is role of CNS stimulants in PAH?

A

cocaine/methamphetamines cause medial hypertrophy of pulm arteries

20
Q

What is role of TFG-B in PAH?

A
  • bone morphogenetic protein receptor type 2 [BMPR2]

modulates growth of vascular cells
mutant –> lose control, extra growth

incomplete penetrance –> may be necessary but insufficient to cause disease

21
Q

What genetic pathways predispose to PAH?

A
  • TFG-B mutation

- serotonergic path mutation

22
Q

What is role of serotonergic path in PAH development?

A

variant gene expression of serotonin receptor –> increase serotonin dependent vascular remodeling

23
Q

WHat is theory of cause of PAH?

A

causes multiple hits to get the disease

risk factors + genetic predispostion + cell dysfunction + inflammation

24
Q

What happens in presymptomatic/compensated PAH?

A

higher PAP pressure but able to compensate

CO ok

25
What happens in symptomatic/decompensating PAH?
RV facing consequences of years of pressure --> weakens --> CO starts to drop and RAP rises manifests as fatigue, lower extremity edema, elevated neck veins
26
What happens in declining/decompensated PAH?
CO way down RAP way up pulm artery pressure reaches inflection point and starts to go down = sign of how poor your RV is function you can't even get fluid into pulm vasculature eventually get RV failure
27
What is prognosis of PAH?
3 year survival 60%
28
What are symptoms of PAH?
- dyspnea - fatigue - SOB - chest pain - syncope - palpitations - edma
29
What are physical findings in PAH?
- elevated jugular venous distension - tricuspid regurg due to increase pulm artery P - accentuated S2 - S3 gallop - peripheral edema - hepatomegaly, pulsatile liver, ascites
30
What doe you see on EKG with PAH?
- not sensitive enough to be a screening tool see - RV hypertrophy = big V1 QRS; big S in V5/6 - R axis deviation = I down; aVF up - R atrium enlarged = big P wave in II, III, aVF
31
What do you see on CT with PAH?
- enlarged central pulm arteries | - reduced caliber of peripheral vessels
32
What diagnostic workup for PAH?
- do clinical exam - look for sings of RV or RA enlargement or increased pressure primary screening = do echo rule out other common causes of pulm HTN if you see high pressure on pulm arteries --> confirm with invasive cath lab measurement of pressures
33
What should you screen for if you think PAH?
- screen for thromboembolic disease - work up for infectious disease or connective tissue disorder - screen for chronic lung disease
34
What is treatment for PAH?
- give ox --> maintain saO2 > 90% - routine vaccinations - give diuretics carefully for volume overload symptoms - maybe digitalis - warfarin b/c of prothrombotic state of PAH can give vasoactive agents
35
What should you avoid in PAH
- avoid vasoconstrictors --> nicotine, sympathomimetics - avoid deep valsalva - avoid pregnancy
36
How does phosphodiesterase 5 inhibitor help in pAH?
prevents breakdown of cGMP --> get vasodilitory effects of NO