CPRS 73: Pulmonary Hypertension

Question 1

What is the normal mean pulmonary artery pressure

Answer 1

Less than 20 mmHg

Question 2

What is the mean pulmonary artery pressure during pulmonary hypertension

Answer 2

mPAP > 25mmHg during rest

Question 3

What is Group 1 Pulmonary Hypertension

Answer 3

Pulmonary Arterial Hypertension

Question 4

What is Group 2 Pulmonary Hypertension

Answer 4

Pulmonary Venous Hypertension

Question 5

What is Group 3 Pulmonary Hypertension

Answer 5

Associated with respiratory disease or hypoxia

Question 6

What is Group 4 Pulmonary Hypertension

Answer 6

Embolic or Thromboembolic Occlusion

Question 7

What is Group 5 Pulmonary Hypertension

Answer 7

Unclear Mechanisms

Question 8

Is Idiopathic Group 1 PAH and Heritable Group 1 PAH clinically distinguishable from each other?

Answer 8

No

Question 9

What is the gene causing heritable Group 1 PAH

Answer 9

BMPR2 Mutation

Question 10

What diseases causes secondary Group 1 Pulmonary Hypertension

Answer 10

Congenital Heart Disease

Collagen Vascular Disease

Question 11

What is scleroderma

Answer 11

Harden and thickening of areas of the skin

Question 12

How does scleroderma cause PAH

Answer 12

Obliteration (destruction) of alveolar capillaries

Narrowing of small arteries

Question 13

What diseases lead to obliteration of the pulmonary vascular bed

Answer 13

Systemic Lupus Erythematosus
Rheumatoid Arthritis
Scleroderma

Question 14

What is Systemic Lupus Erythematosus

Answer 14

Immune System Attacking its own tissues

Question 15

What’s the most common, second most common and third most common cause of pulmonary hypertension, and their respective groups classified under pulmonary hypertension?

Answer 15

Left Heart Disease (most Common)- Group 2
Scleroderma (2nd most common)- Group 1
Obstructive Sleep Apnea (3rd most common)- Group 3

Question 16

What are the Symptoms of Pulmonary Hypertension

Answer 16

Dyspnea during activity (Shortness of Breath)
Syncope (Fainting/ Passing out)
Chest Pain
Leg Edema (Sign of right heart failure)

Question 17

What are the signs of Pulmonary Hypertension

Answer 17

Scleroderma
Systemic Lupus Erythematous 
Cyanosis
Obesity
Elevated Jugular Venous Pressure

Question 18

What are the three features in Pulmonary Hypertension of the Chest X-Ray

Answer 18

Elevated Cardiac Apex due to Right Ventricular Hypertrophy
Enlarged Right Atrium
Enlarged Pulmonary Arteries

Question 19

What are the ECG Abnormalities of Pulmonary Hypertension

Answer 19

ST Depression
T wave inversion
in V1 to V3

Question 20

What methods are used to screen pulmonary hypertension

Answer 20

Transthoracic echocardiography

Right Heart Catheterization

Question 21

How to treat Pulmonary Hypertension (Group 1)

Answer 21

Calcium Channel Blockers

Nifedipine Diltiazem Amlodipine

Question 22

How to treat Pulmonary Hypertension (Group 2)

Answer 22

Correct the left heart disease

Question 23

How to treat Pulmonary Hypertension (Group 3)

Answer 23

Supply with oxygen to reverse the hypoxemia

Question 24

How to treat Pulmonary Hypertension (Group 4)

Answer 24

Supply with anticoagulation therapy

Question 25

What is not needed for treatment of pulmonary hypertension for groups 2,3,4

Answer 25

Vasodilator Therapy

Question 26

What are some general treatments for treating pulmonary hypertension of all groups

Answer 26

``` Diuretics Oxygen Anticoagulation Digoxin Exercise ```

Question 27

How does endothelin receptor antagonists work

Answer 27

Endothelin-1 is a vasoconstrictor | Antagonists reduce the action of Endothelin-1 and leads to vasodilatation

Question 28

Name some endothelin receptor antagonists

Answer 28

Bosentan Sitaxsentan Ambrisentan Macitentan

Question 29

How does PDE-5 inhibitors work

Answer 29

PDE-5 breaks down cGMP Inhibits PDE-5 retains more cGMP that leads to vasodilation Increase nitric oxide pathway also simulates sGC, leading to increase in cGMP production, leading to vasodilation

Question 30

Name some PDE-5 inhibitors

Answer 30

Sildenafil | Tadalafil

Question 31

Name a soluble guanylate cyclase stimulator

Answer 31

Riociguat

Question 32

How does soluble guanylate cyclase stimulator work

Answer 32

Stimulates production of cGMP

Question 33

Name a prostacyclin analogue

Answer 33

Epoprostenol

Question 34

Name a prostacyclin receptor agonist

Answer 34

Selexipag

Question 35

How does prostacyclin analogue work?

Answer 35

Stimulation of prostacyclin increases cAMP | Leads to vasodilation