UW pulm physio 2

Question 1

Hereditary PAH is most often due to an …………………..

Answer 1

Inactivating mutation in BMPR2

Question 2

Patients with BMPR2 mutation have presdisposition for (2) ……………….

Answer 2

dysfunctional epithelium and smooth muscle cell proliferation

Question 3

What is thought activate disease process in pulmonary hypertension?

Answer 3

An insult as infection or drugs

Question 4

Insults, that activate pulmonary hypertension leads to decreased ………… (2) an increased …………….. (2)

Answer 4

decr. vasodilative, antiproliferative mediators eg NO, prostacyclin;
incr. vasoconstrictive, proliferative mediators eg endothelin

Question 5

Insults, that activate pulmonary hypertension leads to decreased ………… (2) an increased …………….. (2)

Answer 5

decr. vasodilative, antiproliferative mediators eg NO, prostacyclin;
incr. vasoconstrictive, proliferative mediators eg endothelin

Question 6

What is end result that cause pulmonary hypertension?

Answer 6

Vasoconstriction with vascular smooth muscle proliferation, intimal thickening and fibrosis + incr. pulm. vasc. resist and progresive pulmonary hypertension

Question 7

What complication results due to PAH and causes right axis deviation on ECG?

Answer 7

PH –> right ventricular hyperthrophy as compensation –> right axis deviation on ECG.

Question 8

How can be detected pulmonary artery hypertension? (physical examination)

Answer 8

Loud pulmonic component of S2

Question 9

How can be detected pulmonary artery hypertension? (physical examination)

Answer 9

Loud pulmonic component of S2

Question 10

2 steps of hereditary PAH development?

Answer 10

Abnormal BMPR2 predisposes to excessive smooth muscle and endotheliai proliferation. Insult such infection of drugs then activate the disease process

Question 11

What form s in the last stage of PAH pathogenesis in arteries?

Answer 11

Capillary tufts (pexiform lesions)

Question 12

What form s in the last stage of PAH pathogenesis in arteries?

Answer 12

Capillary tufts (pexiform lesions)

Question 13

What triggers remodeling in systemic sclerosis in arteries?

Answer 13

Increased proliferation of T cells with secretion of variety cytokines (eg TGF-beta) –> stimulate fibroblasts –> increased collagen and extracellular matrix proteins.
Also, endothelial dysfunction due to inc. endothelin and TXA2 versus NO and prostacyclin

Question 14

What other changes apart PAH can cause systemic sclerosis in lungs?

Answer 14

Interstitial fibrosis –> PAH due to hypoxia-induced vasoconstriction

Question 15

What disease in chest cavicty also manifest in systemic sclerosis and induce RHF?

Answer 15

Pericardial fibrosis –> impaired diastolic dysfunction of RV

Question 16

What causes loud pulmonic component in PAH?

Answer 16

forceful pulmonic valve closure in the setting of high pulmonary arterial pressure

Question 17

What can create accenuated impulse palpated at the left sternal border in PAH?

Answer 17

RV enlargement due to inc. load (ie concentric RV hypertrophy)

Question 18

What can create accenuated impulse palpated at the left sternal border in PAH?

Answer 18

RV enlargement due to inc. load (ie concentric RV hypertrophy)

Question 19

How is described accenuated impulse felt on left sternal border in PAH?

Answer 19

Left parasternal lift due to right ventricular heave

Question 20

PAH relatively more often afftects what population?

Answer 20

young women

Question 21

What causes fatigue and exertional dyspnea in PAH?

Answer 21

Decreased CO

Question 22

Where happens congestion due to HF in lungs?

Answer 22

Pulmonary venous congestion

Question 23

Where happens congestion due to HF in lungs?

Answer 23

Pulmonary venous congestion

Question 24

Smooth muscle cell proliferation in pulmonary arteries leads to ………… (1)

Answer 24

medial hypertrophy

Question 25

Collagen depositions in pulmonary arteries leads to ............ (2)

Answer 25

Intimal thickening and fibrosis

Question 26

The remodeling in pulmonary arteries is less extensive in PH due to LH or due to primary PAH?

Answer 26

in LV induced PAH. primary is due to genetic mutations

Question 27

Congenital heart disease that causes ..............shunting (eg, ventricular septal defect, atrial septal defect) can lead to ...... via an increase in pulmonary arterial blood flow

Answer 27

left-to-right; pulmonary hypertension

Question 28

Why occurs hypoxic vasoconstriction in pulmonary hypertension?

Answer 28

Hypoxic vasoconstriction is a physiologic mechanism unique to lung tissue that helps minimize ventilation-perfusion mismatch and increase overall gas exchange efficiency.

Question 29

An area of ventilation defect without perfusion defect is suggestive of conditions that lead to .......................

Answer 29

acute alveolar filling (pneumonia, pulmonary edema)

Question 30

Pulmonary arterial compliance in pulmonary hypertension?

Answer 30

decreases due to vascular remodeeling and stiffening of the walls

Question 31

The main mechanism of pulmonary hypertension in chronic hypoxic diseases?

Answer 31

hypoxia induced vasoconstriction

Question 32

Massive PE can lead to sudden occlusion of .....% of the pulmonary arterial circulation

Answer 32

>50proc

Question 33

If CT is contraindicated for PE diagnostics, what method we would use?

Answer 33

V/Q scan

Question 34

Low tidal volume ............ dead-space ventilation

Answer 34

increases

Question 35

Increases in physiologic dead space occur in many lung diseases, including (3)

Answer 35

PE, emphysema, ARDS

Question 36

lower tidal volumes increase the proportion of ....................

Answer 36

each breath composed of dead space

Question 37

minute ventilation formula?

Answer 37

tidal volume x RR

Question 38

Why patients with weakened respiratory muscles tend to breath at low tidal volumes?

Answer 38

To minimize the work of breathing

Question 39

Patients with weakened respiratory muscles tend to breath at low tidal volume. What is a compensatory response?

Answer 39

hypoventilation triggers increase in respiratory drive --> incr. RR to maintain ventilation

Question 40

What is rapid shallow breathing index (RSBI)?

Answer 40

Ratio of respiratory rate/tidal volume

Question 41

How is called the ratio of respiratory rate/tidal volume?

Answer 41

Rapid shallow breathing index (RSBI)

Question 42

What indicates low RSBI? (3)

Answer 42

relatively high tidal volume, relatively efficient breathing and lower likelihood of recurrent respiratory failure once ventilatory support is discontinued.

Question 43

What indicates low RSBI? (3)

Answer 43

relatively high tidal volume, relatively efficient breathing and lower likelihood of recurrent respiratory failure once ventilatory support is discontinued.

Question 44

Increases in both respiratory rate and tidal volume result in increased .................

Answer 44

minute ventilation

Question 45

A decrease in respiratory rate and an increase in tidal volume would ...............

Answer 45

keep minute ventilation

Question 46

Decr. RR and inc. tidal volume. Dead space ventilation each breath?

Answer 46

decrease

Question 47

Because at low tidal volumes a higher proportion of each breath is composed ..............., it leads to..................

Answer 47

Of dead space; leads to an increase in wasted ventilation (inefficient breathing)

Question 48

Because at low tidal volumes a higher proportion of each breath is composed ..............., it leads to..................

Answer 48

Of dead space; leads to an increase in wasted ventilation (inefficient breathing)

Question 49

How astma changes compliance of the lungs?

Answer 49

doesnt change

Question 50

What method is used if patient cannot tolerate CT angiography in PE with IV contrast?

Answer 50

V/Q scan - compares regional ventilation and perfussion.

Question 51

V/Q scan in PE. 1 stage - what is used?

Answer 51

Radiolabeled aerosol - inhaled and delivered throughout the tracheobronchial tree.

Question 52

V/Q scan in PE. 2 stage - what is used?

Answer 52

IV tracer is distributed throughout the pulmonary vasculature.

Question 53

V/Q scan in spontaneous pneumotorax?

Answer 53

normal perfusion but impaired ventilation due to compressed lung

Question 54

what is normal thick of the RV wall?

Answer 54

3-4mm

Question 55

Long-standing pulmonary hypertension eventually leads to .................................right ventricle (cor pulmonale)

Answer 55

hypertrophy and/or dilation of the right ventricle

Question 56

What is the most common cause of death in PAH?

Answer 56

RHF with circulatory collapse and respiratory failure

Question 57

What is the difference between dilated cardiomyopathy and cor pulmonale due to PAH?

Answer 57

dilated cardiomyopathy - all 4 chambers are dilated RV hypertrophy due due PAH - thick RV wall

Question 58

Gross changes in WPW?

Answer 58

none, because it is electrophysical abnormality.

Question 59

Endothelin pathway and result?

Answer 59

Proendothelin --> endothelin-1 --> activates endothelin receptor on smooth mucle --> vasoconstriction+proliferation

Question 60

NO pathway and result?

Answer 60

L-arginine --> forms NO and L-citruline --> NO converts GTP to cGMP --> vasodilation and decreased proliferation

Question 61

Prostacyclin pathway and result?

Answer 61

Arachidonic acid --> prostacyclin --> cAMP --> vasodilation and decr. proliferation

Question 62

What part of vessel proliferates in PAH?

Answer 62

Intima

Question 63

What targets PAH therapy?

Answer 63

Targets the mediator imbalance created by endothelial dysfunction (inc. vasoconstrictive mediators and decr. vasodilative mediators)

Question 64

What is endothelin receptor antagonist?

Answer 64

Bosentan

Question 65

What is prostacyclin analogue?

Answer 65

Epoprostenol

Question 66

NO oxide enhancing agent?

Answer 66

Sildenafil

Question 67

What is the same effect of all medications used for PAH therapy?

Answer 67

All help to reduce tissue proliferation regardless their mechanism of action