Pulm Vasc Dz

Question 1

pulmonary circulation is ___ pressure and ____ volume

Answer 1

low; low

Question 2

how does pulm circulation accommodate increased cardiac output?

Answer 2

recruitment of additional blood vessels (rather than distension)

Question 3

what is an avg pulm blood pressure (syst/diast)

Answer 3

25/8

Question 4

blood flow is greatest in this region of the lung

Answer 4

zone 3 (lower region)

Question 5

alveolar pressure is greatest in this region of the lung

Answer 5

zone 1 (upper)

Question 6

an intrinsic pulm vacular disease implies that the etiology of the dz is?

Answer 6

related to injury of the blood vessels themselves

Question 7

the ultimate cause/precurser of pulm HTN is

Answer 7

decreased cross-sectional area

Question 8

what vascular changes (4 steps) lead to thromboembolism and/or a decrease in cross-sectional area of pulm blood vessels?

Answer 8

medial hypertrophy –> intimal proliferation –> angiomatoid transformation –> fibrinoid necrosis (flexiform lesions)

Question 9

definition of pulm HTN

Answer 9

mean pulm artery pressure (PAP) > 25mmHg at rest (average is 15)

Question 10

definition of pulm arterial HTN (PAH)

Answer 10

mean PAP >/= 25, pulm wedge pressure =/< 15, PVR >/= 3 wood units

Question 11

what is the equation of pulm vascular resistance?

Answer 11

mPAP - PCWP/CO

Question 12

the ultimate negative consequence of pulm vascular disease is?

Answer 12

cor pulmonale

Question 13

passive PH is due to?

Answer 13

left heart dysfunction leading to increase LA pressure

Question 14

hyperkinetic PH is due to?

Answer 14

intracardiac shunt (high flow), hemolytic anemia, dialysis

Question 15

occlusive PH is due to?

Answer 15

chronic PE, tumor emboli

Question 16

obliterative PH is due to?

Answer 16

emphysema, ILD, vasculitis, sarcoidosis

Question 17

vasoconstrictive PH is due to?

Answer 17

hypoxia, scleroderma

Question 18

group 1 PH is defined as?

Answer 18

pulm arterial hypertension (intrinsic PH) = idiopathic, heritable, drug/toxin-induced, etc.

Question 19

intrinsic PH is assoc with what diseases?

Answer 19

connective tissue disease, HIV infection, portal HTN, congenital heart dz, schistosomiasis, sickle cell anemia

Question 20

group 2 PH is due to?

Answer 20

heart disease

Question 21

group 3 PH is due to?

Answer 21

lung disease and/or hypoxia (leading to vasoconstriction)

Question 22

group 4 PH is due to?

Answer 22

chronic thromboembolic pulm HTN (CTEPH)

Question 23

name 4 risk factors for group 4 PH

Answer 23

younger age, female, chronic inflamm conditions, large central PE

Question 24

name 3 causes of group 5 PH

Answer 24

myeloproliferative disorders, sarcoidosis, chronic renal failure on dialysis, metabolic disorders

Question 25

when pulm HTN progresses, there is loss of response to what drug?

Answer 25

calcium-channel blockers (short-acting vasodilators)

Question 26

what is the go to screening tool for PH?

Answer 26

echocardiogram - assess RH size, estimate PAP, evaluate LV function

Question 27

labs are collected in a patient with PH in order to?

Answer 27

rule out secondary causes

Question 28

name several labs that would be collected to evaluate PH

Answer 28

HIV, liver fnc, ANA, tox screen, polysomnogram (apnea), PFTs

Question 29

is a CXR diagnostic for PH?

Answer 29

NO, but can rule out other disease

Question 30

what is the gold standard eval of PH

Answer 30

right heart catheterization to measure PAP, PCWP, CO

Question 31

the WHO defines how many functional classifications of PH

Answer 31

IV, with Class IV being complete inability to perform any physical activity without sx

Question 32

where do plexiform lesions form?

Answer 32

around obliterated arteries

Question 33

in addition to the three major pathways for PAH tx, what adjunct tx can be added?

Answer 33

conservative anticoag, oxygen, atrial septostomy, lung transplant (digoxin questionable)

Question 34

death beyond the first few hours post-emboism is generally due to?

Answer 34

recurrence (and thus it is preventable)

Question 35

pulmonary consequences of PE

Answer 35

deadspace, hypoxemia (shunt, mismatch), hyperventilation, depletion of surfactant(?), pulm infarct

Question 36

hemodynamic consequences of PE

Answer 36

decreased cross-sectional area (leading to PH, RHF), humoral reflex mechanisms (hypoxic vasoconstriction, mediator release)

Question 37

dx of PE

Answer 37

elevate D-Dimers, doppler US, V/P scan, pulm angiogram

Question 38

sx of acute PE

Answer 38

dyspnea, pleuritic pain, sense of doom, cough, hemoptysis, syncope

Question 39

signs of acute PE

Answer 39

tachy, increased P2, thrombophlebitis, S3/S4, sweating, edema, murmer, cyanosis

Question 40

tx options for PE

Answer 40

1. anticoag, 2. thrombolytics (massive or RVD), IVC filter (if possibility of fatal), acute embolectomy

Question 41

how to screen for chronic thromboembolic PH

Answer 41

V/Q scan, followed by pulm angio to confirm

Question 42

clinical signs of pulmonary vasculitis

Answer 42

diffuse alveolar hemorrhage, cavitation, nodular dz, or nothing

Question 43

name three diseases that cause secondary pulm vasculitis

Answer 43

wegener's granulomatosis, goodpasture's syndrome, SLE

Question 44

this autoimmune dz results in antibodies against basement membrane and affects the lungs and kidneys

Answer 44

goodpasture's syndrome

Question 45

this autoimmune dz causes pulm vasculitis and is assoc with upper airway/oral lesions

Answer 45

wegener's granulomatosis