CPRS 73: Pulmonary Hypertension Flashcards
What is the normal mean pulmonary artery pressure
Less than 20 mmHg
What is the mean pulmonary artery pressure during pulmonary hypertension
mPAP > 25mmHg during rest
What is Group 1 Pulmonary Hypertension
Pulmonary Arterial Hypertension
What is Group 2 Pulmonary Hypertension
Pulmonary Venous Hypertension
What is Group 3 Pulmonary Hypertension
Associated with respiratory disease or hypoxia
What is Group 4 Pulmonary Hypertension
Embolic or Thromboembolic Occlusion
What is Group 5 Pulmonary Hypertension
Unclear Mechanisms
Is Idiopathic Group 1 PAH and Heritable Group 1 PAH clinically distinguishable from each other?
No
What is the gene causing heritable Group 1 PAH
BMPR2 Mutation
What diseases causes secondary Group 1 Pulmonary Hypertension
Congenital Heart Disease
Collagen Vascular Disease
What is scleroderma
Harden and thickening of areas of the skin
How does scleroderma cause PAH
Obliteration (destruction) of alveolar capillaries
Narrowing of small arteries
What diseases lead to obliteration of the pulmonary vascular bed
Systemic Lupus Erythematosus
Rheumatoid Arthritis
Scleroderma
What is Systemic Lupus Erythematosus
Immune System Attacking its own tissues
What’s the most common, second most common and third most common cause of pulmonary hypertension, and their respective groups classified under pulmonary hypertension?
Left Heart Disease (most Common)- Group 2
Scleroderma (2nd most common)- Group 1
Obstructive Sleep Apnea (3rd most common)- Group 3
What are the Symptoms of Pulmonary Hypertension
Dyspnea during activity (Shortness of Breath)
Syncope (Fainting/ Passing out)
Chest Pain
Leg Edema (Sign of right heart failure)
What are the signs of Pulmonary Hypertension
Scleroderma Systemic Lupus Erythematous Cyanosis Obesity Elevated Jugular Venous Pressure
What are the three features in Pulmonary Hypertension of the Chest X-Ray
Elevated Cardiac Apex due to Right Ventricular Hypertrophy
Enlarged Right Atrium
Enlarged Pulmonary Arteries
What are the ECG Abnormalities of Pulmonary Hypertension
ST Depression
T wave inversion
in V1 to V3
What methods are used to screen pulmonary hypertension
Transthoracic echocardiography
Right Heart Catheterization
How to treat Pulmonary Hypertension (Group 1)
Calcium Channel Blockers
Nifedipine Diltiazem Amlodipine
How to treat Pulmonary Hypertension (Group 2)
Correct the left heart disease
How to treat Pulmonary Hypertension (Group 3)
Supply with oxygen to reverse the hypoxemia
How to treat Pulmonary Hypertension (Group 4)
Supply with anticoagulation therapy
What is not needed for treatment of pulmonary hypertension for groups 2,3,4
Vasodilator Therapy
What are some general treatments for treating pulmonary hypertension of all groups
Diuretics Oxygen Anticoagulation Digoxin Exercise
How does endothelin receptor antagonists work
Endothelin-1 is a vasoconstrictor
Antagonists reduce the action of Endothelin-1 and leads to vasodilatation
Name some endothelin receptor antagonists
Bosentan
Sitaxsentan
Ambrisentan
Macitentan
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
Name some PDE-5 inhibitors
Sildenafil
Tadalafil
Name a soluble guanylate cyclase stimulator
Riociguat
How does soluble guanylate cyclase stimulator work
Stimulates production of cGMP
Name a prostacyclin analogue
Epoprostenol
Name a prostacyclin receptor agonist
Selexipag
How does prostacyclin analogue work?
Stimulation of prostacyclin increases cAMP
Leads to vasodilation
