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
Q

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

A

Vasodilator Therapy

26
Q

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

A
Diuretics
Oxygen
Anticoagulation
Digoxin
Exercise
27
Q

How does endothelin receptor antagonists work

A

Endothelin-1 is a vasoconstrictor

Antagonists reduce the action of Endothelin-1 and leads to vasodilatation

28
Q

Name some endothelin receptor antagonists

A

Bosentan
Sitaxsentan
Ambrisentan
Macitentan

29
Q

How does PDE-5 inhibitors work

A

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
Q

Name some PDE-5 inhibitors

A

Sildenafil

Tadalafil

31
Q

Name a soluble guanylate cyclase stimulator

A

Riociguat

32
Q

How does soluble guanylate cyclase stimulator work

A

Stimulates production of cGMP

33
Q

Name a prostacyclin analogue

A

Epoprostenol

34
Q

Name a prostacyclin receptor agonist

A

Selexipag

35
Q

How does prostacyclin analogue work?

A

Stimulation of prostacyclin increases cAMP

Leads to vasodilation