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Lec 19 Pulmonary Vascular Disease Flashcards

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1
Q

What is ohms version of equation of Pulm artery pressure?

KNOW THIS!!

A

Ppa = (CO * PVR) + Pla

Pla = left atrial pressure
b/c change in P = CO * R

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2
Q

What is ohm’s version of equation of pulm artery pressure?

A

Psa = (CO*PVR) + Pra

Pra = right atrial pressure b/c changes in P = CO*R

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3
Q

How do pulm vessels differ from systemic vessels?

A

less muscle and elastin

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4
Q

What is definition of pulmonary hypertension?

A

mean Pa > 25

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5
Q

What are 3 mechs of what will cause increased pulm artery pressure?

A
  • increased CO
  • increase left atrial P
  • increased PVR
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6
Q

What are some things that cause increased CO and thus lead to high pulm artery pressure?

A
  • congenital heart defects w/ L to R shunt
  • cirrhosis
  • anemia
  • A-V malformations
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7
Q

What are some things that cause increased left atrial P and thus lead to high pulm artery pressure?

A
  • systolic/diastolic LV failure
  • mitral valve disease
  • restrictive cardiomyopathy
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8
Q

What are some things that cause incraesed PVR and thus lead to high pulm artery pressure?

A
  • destruction pulm vascular bed in ILD, emphysema, PE
  • hypoxic vasoconstriction in COPD, high altitude
  • small pulm artery vasculopathy [PAH]
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9
Q

What is group 1 pulmonary hypertension?

A

pulmonary arterial hypertension

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10
Q

What is group 2 pulm HTN?

A

due to left heart disease

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11
Q

What is group 3 pulm HTN?

A

due to lung disease, hypoxia

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12
Q

What is group 4 pulm HTN?

A

due to thromboembolic disease

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13
Q

What is group 5 pulm HTN?

A

miscellaneous “other”

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14
Q

What are some pathological signs seen in pulmonary arterial hypertension?

A
  • medial hypertrophy
  • intimal thickening
  • in situ thrombosis
  • plexiform lesions
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15
Q

What is pathogenesis of pulmonary hypertension?

A
  • increased endothelin pathway –> vasoconstriction and proliferation of smooth muscle
  • decrease NO path –> less vasodilation/antiproliferation
  • decrease prostacycline path –> less vasodilation and antiproliferation
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16
Q

What are 4 possible etiological categories of PAH?

A
  • idiopathic
  • genetic
  • drug/toxin induced
  • associated w/ other disease
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17
Q

What is genetic cause of pulmonary arterial hypertension?

A
  • mutation in BMPR2
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18
Q

What drugs/toxins can cause pulmonary arterial htn?

A
  • anorexigens [fen phen]
  • grapeseed oil
  • illicits
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19
Q

What are some diseases associated with PAH?

A
  • connective tissue [SLE, scleroderma]
  • congenital heart disease –> increased pulm pressure/flow
  • portal htn
  • HIV
  • schistosomiasis
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20
Q

WHat is prevalence of PAH?

A

5-15 / 1 million adults

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21
Q

How prevalent is PAH in pts with CHD?

A

30% of pts who do not have CHD repaired will develop PAH

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22
Q

What is survival of untreated idiopathic PAH?

A
  • median survial 2.8 years

34% survival at 5 years

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23
Q

What is difference RV and LV?

A
  • LV concentric shape; thick free wall

- RV crescent shape; thin free wall; lacks circumferential constrictor fibers; more compliant

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24
Q

What is definition preload?

A

wall stress at end of diastole

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25
What is definition afterload?
wall stress during ventricular ejection
26
What is contractility?
intrinsic ability of myocardium to contract independent of load
27
What happens to LV if dildate RV?
compress LV
28
Why do you get such rapid decompensation in PAH?
high pulm pressure means increased RV afterload RV afterload --> increased RV wall stress --> ischemia and decreased output leading to decreased CO RV afterload --> decreased RV output --> also decreases CO RV afterload --> increase RV dilatation --> leftward septal shift and tricuspid regurg --> also decrease RV output and LV preload leading to more decreased CO significant decrease LV ---> leading to cardiogenic shock
29
What are symptoms of PAH?
from low perfusion - dyspnea - fatigue - chest pain - palpitation from congestion --> ab pain and fulness, peripheral edema
30
What do you seen on physical exam in PAH?
- hypoxemia, tachycardia, hypotension - JVP distension - RV head, split S2, loud P2, systolic tricuspid murmur - RV S3/S4 - hepatomegaly, pulsatile liver, ascites - lower extremity edema
31
Do you see rales in pure PAH?
nope! no pulmonary edema in pure PAH
32
WHat do you see on chest xray in PAH?
dilated pulmonary arteries | restro-sternal space disappears in lateral view
33
What do you see on EKG in PAH?
- RV hypertrophy/strain - RBBB - right atrial enlargement
34
What is use of right heart cath? What does it tell you?
- confirms diagnosis of pulmonary hypertension | - tells you hemodynamic profile --> RAP, PAP, PCWP, CI, PVR
35
What does PCWP tell you about etiology of pulmonary hypertension?
- if PCWP > 15 suggests PH type 2 [due to left heart disease] - if PCWP < 15 consistent w/ pulm HTN that occurs before capillaries = type I [PAH]; III [lung disease; or IV [thromboembolic]
36
What is natural history of pulmonary hypertension?
in asymptomatic/compensated: CO still pretty high and RAP low; PAP/PVR rising in symptomatic/decompensated: CO declining; PAP/PVR really high in overt R HF: high PVR but PAP starts to decrease as CO drops and RAP increase = signs of low perfusion and congestion
37
What are the names of the 4 Ca channel blockers?
amlodipine/nifedipine | diltiazem/verapamil
38
What is use of Ca channel blockers in pulm htn?
- result in pulmonary arterial vasodilation | - beneficial if positive vasoreactivity test [vasoresponder]
39
What is test to see if patient is vasoresponder?
- give short-acting vasodilator like epoprostenol/adenosine/inhaled NO - a vasoresponder will have decreased PAP by > 10 to absolute < 40 without concurrent drop in CO only makes up 13% of population of pts with idiopathic PAH
40
What is mech of prostanoids?
- stimulate adenylate cyclase --> incrase cAMP | - cause vasodilation, antiproliferation, platelet aggregation inhibition
41
What is effect of prostanoids?
- improve hymodynamics; functional capacity
42
What is the one prostanoid that increases survival?
epoprostenol
43
What are the 3 types of prostanoids and their mech of administratiON?
``` epoprostenol = IV treprostinil = SC, IV, PO, inhale iloprost = inhaled ```
44
How is eoprostenol administered? half life?
1/2 life = 3-5 min | continuous IV admin
45
Which prostanoid is just synthetic prostacyclin?
epoprostenol
46
How is treprostinil administered?
IV and subcutanous = bioequivalent | 1/2 life is 2-4 hrs
47
Where are the two types of endothelin receptor antagonists located? action?
- ETA/ETB on smooth muscle cells and cardiac myocytes - ETB also on endothelial cells both cause vasoconstriction/ proliferation smooth muscle ETB also mediates pulmonary clearance and induces production of local mediators
48
What are the 3 endothelin receptor antagonists?
- bosentan - ambrisentan - macitentan
49
What is order of highest affinity for ETA/ETB among the 3 endothilin receptor antagonists?
ambrisentan > macitentan > bosentan affinity ETA: ETB
50
WHat is effect of endothelin receptor antagonists?
improve hemodynamics and functional capacity
51
WHat is mech/effect of phosphodiesterase 5 inhibitors?
- inhibits cGMP specific phosphoidesterase cause vasodilation/ antiproliferation/ platelet aggregation inhibition improve hemodynamics and functional capacity
52
What are the 2 phosphodiesterase 5 inhibitors?
sildenafil [PO/IV] | tadalafil [PO
53
What is mech of action of riociguat?
stimulates solumble guanylate cyclase [sGC] --> converts GTP to cGMP
54
What is effect of riociguat?
vasodilation, antiproliferation, platelet aggregation inhibition improves hemodynamics and functional capacity
55
What are some things you need to consider when you are giving vasodilators?
- vasodilation will decrease SVR not just PVR --> caution in hypotensive patients - abrupt medication withdrawal causes rebound pulmonary HTN --> need to wean gradually - can worsen V/Q match leading to hypoxemia --> caution in intrinsic lung disease
56
What are side effects common of pulmonary vasodilators?
vasodilation --> headache, dizziness, flushing, nasal congestion
57
What is a side effect of bosentan?
liver tox
58
What are side effects common to endothelin receptor antagonists?
- teratogenic, peripheral edema, anemia
59
What are side effects of prostanoids?
jaw pain
60
What are general treatment measures of pulm HTN?
- give supplemental O2 to maintain saturation > 90% - anticoagulation - digoxin to improve contractility - cardiopulmonary rehab to improve functional capacity
61
What surgical treatments available for pulm HTN?
- atrial septostomy --> unloads RV at cost of hypoxemia - pulm thromboendarectomy in case of chronic thromboembolic pulm htn - lung transplant

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