B4-067 Pulmonary Vascular Disease

Question 1

mean pulmonary artery pressure > 20mm Hg by right heart cath

Answer 1

pulmonary hypertension

Question 2

• mean pulmonary arterial pressure > 20 mm Hg
• PCWP less than 15 mmHg
• PVR greater than 3 wood units

Answer 2

pulmonary arterial hypertension

Question 3

3 prostaglandin pathways

Answer 3

1. endothelin
2. NO pathway
3. prostacyclin

Question 4

• activity through cAMP
• vasodilator
• inhibits proliferation of vascular smooth muscle
• decreases platelet aggregation

Answer 4

prostacyclin

PGI2

Question 5

in PAH, prostacyclin synthase is […]

Answer 5

decreased

Question 6

in PAH, endothelin is […]

Answer 6

increased

Question 7

• arterial vasoconstrictor
• increases sympathetic activity
• increases systemic vascular resistance

Answer 7

endothelin

Question 8

• vasodilator
• inhibits smooth muscle hypertrophy

Answer 8

NO

Question 9

in PAH, NO is

Answer 9

decreased

Question 10

• decreased NO and prostacylin
• increased endothelin 1

lead to vascular injury via

2

Answer 10

• smooth muscle hypertrophy
• vasocontriction

early intimal proliferation

Question 11

PAH eventually causes right ventricular overload, leading to

Answer 11

RV failure

cause of death

Question 12

symptoms of right sided heart failure

Answer 12

• hepatosplenomegaly
• JVD
• ascites
• weight gain
• peripheral edema

Question 13

oral therapies for NO pathway

3

Answer 13

• sildenafil
• tadalafil
• riociguat

inhibit PDE-5

Question 14

oral therapies for endothelin pathway

3

Answer 14

• bosentan
• ambrisentan
• macitentan

endothelin receptor antagonists

Question 15

oral therapies for prostacylin pathway

2

Answer 15

• treprostinil
• selexipag

Question 16

inhaled therapy

1

other one she said no longer used

Answer 16

treprostinil

prostacylin pathway

Question 17

infusion therapies

2

Answer 17

• epoprostenol
• treprostinil

prostacyclin pathway

Question 18

• pressure related phenomenon
• LV problems, left sided valve problems, pulmonary vein obstruction

Answer 18

cardiogenic pulmonary edema

Question 19

• leaky capillaries
• ARDS, HAPE, Opiate OD, etc

Answer 19

non cardiogenic pulmonary edema

Question 20

causes of PAH

7

Answer 20

• idiopathic
• drugs
• connective tissue disease
• HIV
• portal hypertension
• CHD
• schistosomiasis

Question 21

the biggest factor in RV adaptation is

Answer 21

time

Question 22

• related to increased left side pressures
• may be exacerbated by volume overload

Answer 22

cardiogenic edema

Question 23

what is used to make the definitive diagnosis of pulmonary artery hypertension?

Answer 23

right heart cath

Question 24

most common cause of PH in the world

Answer 24

schistosomiasis

Question 25

most common cause of PH in US

Answer 25

heart disease

Question 26

most common cause of death in patients with PAH

Answer 26

progressive RV failure and collapse

Question 27

lung transplantation in PAH is reserved for those

Answer 27

failing maximal medical therapy

Question 28

treatment for severe PAH at urgent risk of HF

Answer 28

• parenteral prostacyclin
• diurectics
• oxygen

Question 29

• mean pulmonary pressure >20 mmHg
• wedge pressure <15 mm Hg
• pulmonary vascular resistance greater than 3 wood units

Answer 29

PAH group 1

Question 30

what 3 pathways are implicated in the development of PAH

Answer 30

1. nitric oxide
2. prostacyclin
3. endothelin

Question 31

• abdominal distention
• hepatomegaly
• lower extremity edema

indicate..

Answer 31

right sided heart failure

Question 32

bilateral rales would indicate

Answer 32

pulmonary edema/left sided HF

Question 33

what measures correspond to risk assessment in PAH?

2

Answer 33

• decreased CO
• syncope