Pulmonary Hypertension (General)

Question 1

What is Pulmonary Hypertension

Answer 1

Elevated Pulmonary Pressure

Question 2

Pulmonary Arterial Hypertension

Answer 2

Specific type of PH
–> Elevated Pulmonary Artery Pressure

Question 3

What does persistent elevated pulmonary vascular resistance cause

Answer 3

High Right Ventricular Systolic Pressure
–> Right Ventricular Growth/Remodeling

*Right ventricle must pump harder against the elevated pulmonary vascular resistance

Question 4

Idiopathic PAH

Answer 4

Unknown Cause of PAH

Rare and Severe

Question 5

Familial PAH

Answer 5

PAH is caused by Mutations in Bmpr2 Gene

Question 6

Secondary PAH

Answer 6

PAH is associated with other medical conditions

Question 7

Endothelial Cell Explanation of PAH

Answer 7

Endothelial Cell damaged
–> Endothelial Cell undergoes Apoptosis
–> Imbalance in Endothelial Cell Function
–> Imbalance in Vasodilating and Vasoconstricting Endothelial Cells
–> Increase in Vasoconstriction
–> Increases Pulmonary Vascular Resistance
–> Pulmonary Arterial Hypertension

Question 8

Gene Explanation of PAH

Answer 8

Bmpr2 (Bone Morphogenic Protein 2) Mutation
–> Smooth Muscle Cell Overgrowth
(Hyperplasia of SMC)
–> Increased Pulmonary Vascular Resistance
–> Pulmonary Arterial Hypertension

Question 9

Endothelin Receptor Antagonists (Mechanism)

Answer 9

Block Smooth Muscle Cell’s ET(A) Receptors
–> Vasodilation

Question 10

Endothelin Receptor Antagonists (Adverse Effects)

Answer 10

Headache
Flushing
Edema
Birth Defect (Don’t take if pregnant)

Question 11

Endothelin-1 (ET-1) Effects

Answer 11

Binds to ET(A) receptors leading to an increase of calcium
–> Vasoconstriction

Question 12

Prostacyclin (Mechanism)

Answer 12

Binds to smooth muscle cells IP receptors
–> Increase G-protein activity
–> Increase Adenylate Cyclase
–> Increase cAMP signaling
–> Vasodilation

Question 13

Prostacyclin (Adverse Effects)

Answer 13

Headache
Diarrhea
Birth Defect: Beraprost (Do not use if pregnant)

Question 14

What causes the Largest Reduction in Pulmonary Artery Pressure

Answer 14

Prostacyclin (Ex. Epoprostenol)

Question 15

What PAH Treatments are contraindicated in pregnancy

Answer 15

Endothelin Receptor Antagonists
–> Bosentan
Prostacyclin
–> Epoprostenol

Question 16

sGC Activator (Mechanism)

Answer 16

Potentiate action of Nitric Oxide by:

Activates sGC (More sGC converts GTP into cGMP)
–> Vasodilation

Question 17

sGC Activator (Adverse Effects)

Answer 17

Hypotension

Question 18

PDE5i (Mechanism)

Answer 18

Phosphodiesterase 5 usually breakdown cGMP

PDE5i inhibits this breakdown of cGMP
–> Increases cGMP signaling
–> Vasodilation

Question 19

PDE5i (Adverse Effect)

Answer 19

Headache
Do not use with nitrovasodilators
–> Will cause severe hypotension if used together

Question 20

Calcium Channel Antagonists (Mechanism)

Answer 20

Reduces entry of calcium into smooth muscle cells
–> Generalized arterial/arteriolar dilation
–> Reduces Blood Pressure

Question 21

Calcium Channel Antagonists (Adverse Effects)

Answer 21

Flushing
Headache
Swelling

Question 22

Inhaled NO (Uses)

Answer 22

Used as acute vasodilator testing of PAH

No therapeutic uses except:
–> Intensive care for PH crisis in newborn babies
–> All other use if for diagnosis and testing

Question 23

PH in Newborn (What Fails?)

Answer 23

Normally:
–> Pulmonary Vasculature goes from HIGH resistance state to a LOW resistance state at birth

Failure:
–> Disease in fetal development can cause HIGH resistance state to persist
–> Inhaled NO is used to treat this and cause a transition into a LOW resistance state

Question 24

What is Shock

Answer 24

Inappropriate Vasodilation that can lead to very low arterial blood pressure

Question 25

What kind of metabolism does Shock cause

Answer 25

Anaerobic Metabolism within Vital Organs

Question 26

Where does Shock commonly occur

Answer 26

In intensive care settings

Question 27

What can cause Shock

Answer 27

Hemorrhage (Loss of Circulating Volume) Burns Bacterial Infections (Septic Shock) Anaphylaxis (Histamine Release) Myocardial Infarction (Lowers Cardiac Output)

Question 28

Epidural

Answer 28

Local Anesthesia, can produce a state of low blood pressure leading to shock

Question 29

How is Shock Treated

Answer 29

Fluid Replacement Adrenergic Vasopressors Vasopressin

Question 30

Shock (Fluid Replacement)

Answer 30

Transfusion is used to treat Hemorrhage --> Increases Circulating Volume to replace volume lost --> Increases Cardiac Output --> Increases Blood Pressure to normal

Question 31

Shock (Adrenergic Vasopressors)

Answer 31

Either causes vasoconstriction (alpha 1) or increases cardiac output (beta 1) --> Increases Blood Pressure back to normal

Question 32

Shock (Vasopressin)

Answer 32

Vasopressin (ADH) is used along with noradrenaline to treat septic shock --> Promotes water reabsorption --> Increases Blood Pressure

Question 33

How does Shock Kill a person

Answer 33

Not enough arterial pressure leads to a decrease in renal blood flow --> Renal Failure --> Death

Question 34

Group 1 PAH

Answer 34

PAH caused by Pulmonary Arterial Hypertension

Question 35

Group 2 PAH

Answer 35

PAH caused by Left Heart Disease

Question 36

Group 3 PAH

Answer 36

PAH caused by Lung Disease

Question 37

Group 4 PAH

Answer 37

PAH caused by Chronic Blood Clots in Lungs

Question 38

Group 5 PAH

Answer 38

Unknown Cause