Pulmonary Arterial Hypertension

Question 1

normal pulmonary artery pressure

Answer 1

8-20 mmHg

Question 2

Define pulmonary hypertension

Answer 2

pulmonary artery pressure > 25 mmHg

Question 3

Classification of PAH: group 1

Answer 3

pulmonary arterial hypertension-includes idiopathic, heritable, drug and toxin-induced, disease-associated (connective tissue disease, HIV, portal HTN), and persistent pulmonary hypertension of a newborn

Question 4

Classification of PAH: group 2

Answer 4

PH due to left heart disease

Question 5

Classification of PAH: group 3

Answer 5

PH due to lung diseases/hypoxia

Question 6

Classification of PAH: group 4

Answer 6

chronic thromboembolic pulmonary hypertension (CTEPH)

Question 7

Classification of PAH: group 5

Answer 7

pulmonary HTN with unclear or multifactorial mechanisms

Question 8

drugs that can cause PAH

Answer 8

cocaine

SSRi use during pregnancy (in newborns)

Question 9

Treatment for PAH

Answer 9

warfarin-INR 1.5-2.5
loop diuretics
digoxin
CCB
prostacyclin analogs and receptor agonists
endothelin receptor antagonists
phosphodiesterase-5 inhbitors

Question 10

Prostacyclin analogues MOA

Answer 10

potent vasodilators of both pulmonary and systemic vascular beds and inhibitors of platelet aggregation

Question 11

Name a prostacyclin analogue

Answer 11

Epoprostenol

Question 12

MOA of PDE-5 inhibitors

Answer 12

PDE-5 is responsible for the degradation of cyclic guanosine monophosphate (cGMP); increased cGMP concentration lead to pulmonary vasculature relaxation and vasodilation

Question 13

List a soluble guanylate cyclase stimulator

Answer 13

Riociguat