CPRS 73: Pulmonary Hypertension Flashcards

1
Q

What is the normal mean pulmonary artery pressure

A

Less than 20 mmHg

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2
Q

What is the mean pulmonary artery pressure during pulmonary hypertension

A

mPAP > 25mmHg during rest

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3
Q

What is Group 1 Pulmonary Hypertension

A

Pulmonary Arterial Hypertension

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4
Q

What is Group 2 Pulmonary Hypertension

A

Pulmonary Venous Hypertension

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5
Q

What is Group 3 Pulmonary Hypertension

A

Associated with respiratory disease or hypoxia

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6
Q

What is Group 4 Pulmonary Hypertension

A

Embolic or Thromboembolic Occlusion

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7
Q

What is Group 5 Pulmonary Hypertension

A

Unclear Mechanisms

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8
Q

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

A

No

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9
Q

What is the gene causing heritable Group 1 PAH

A

BMPR2 Mutation

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10
Q

What diseases causes secondary Group 1 Pulmonary Hypertension

A

Congenital Heart Disease

Collagen Vascular Disease

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11
Q

What is scleroderma

A

Harden and thickening of areas of the skin

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12
Q

How does scleroderma cause PAH

A

Obliteration (destruction) of alveolar capillaries

Narrowing of small arteries

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13
Q

What diseases lead to obliteration of the pulmonary vascular bed

A

Systemic Lupus Erythematosus
Rheumatoid Arthritis
Scleroderma

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14
Q

What is Systemic Lupus Erythematosus

A

Immune System Attacking its own tissues

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15
Q

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

A

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

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16
Q

What are the Symptoms of Pulmonary Hypertension

A

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

17
Q

What are the signs of Pulmonary Hypertension

A
Scleroderma
Systemic Lupus Erythematous 
Cyanosis
Obesity
Elevated Jugular Venous Pressure
18
Q

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

A

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

19
Q

What are the ECG Abnormalities of Pulmonary Hypertension

A

ST Depression
T wave inversion
in V1 to V3

20
Q

What methods are used to screen pulmonary hypertension

A

Transthoracic echocardiography

Right Heart Catheterization

21
Q

How to treat Pulmonary Hypertension (Group 1)

A

Calcium Channel Blockers

Nifedipine Diltiazem Amlodipine

22
Q

How to treat Pulmonary Hypertension (Group 2)

A

Correct the left heart disease

23
Q

How to treat Pulmonary Hypertension (Group 3)

A

Supply with oxygen to reverse the hypoxemia

24
Q

How to treat Pulmonary Hypertension (Group 4)

A

Supply with anticoagulation therapy

25
What is not needed for treatment of pulmonary hypertension for groups 2,3,4
Vasodilator Therapy
26
What are some general treatments for treating pulmonary hypertension of all groups
``` Diuretics Oxygen Anticoagulation Digoxin Exercise ```
27
How does endothelin receptor antagonists work
Endothelin-1 is a vasoconstrictor | Antagonists reduce the action of Endothelin-1 and leads to vasodilatation
28
Name some endothelin receptor antagonists
Bosentan Sitaxsentan Ambrisentan Macitentan
29
How does PDE-5 inhibitors work
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
30
Name some PDE-5 inhibitors
Sildenafil | Tadalafil
31
Name a soluble guanylate cyclase stimulator
Riociguat
32
How does soluble guanylate cyclase stimulator work
Stimulates production of cGMP
33
Name a prostacyclin analogue
Epoprostenol
34
Name a prostacyclin receptor agonist
Selexipag
35
How does prostacyclin analogue work?
Stimulation of prostacyclin increases cAMP | Leads to vasodilation