Therapeutics

Question 1

what is the definition of pulmonary arterial hypertension?

Answer 1

Having a mPAP > or = to 25 mmHg at rest, normal PCWP

Question 2

Pathophysiology of PAH

Answer 2

Caused by:
Pulmonary vasoconstriction
Fibrosis
Hypertrophy
In situ thrombosis

Question 3

WHO groups of Pulmonary Hypertension:

Group 1

Answer 3

Pulmonary arterial hypertension

Question 4

Group 2

Answer 4

Pulmonary hypertension due to left heart disease

Question 5

Group 3

Answer 5

Pulmonary hypertension due to lung diseases and/or hypoxia/vasoconstriction

Question 6

Group 4

Answer 6

Chronic thromboembolic pulmonary hypertension

Question 7

Group 5

Answer 7

Pulmonary hypertension with unclear multifactorial mechanisms

Question 8

WHO Functional Classification of PAH:

Class I

Answer 8

Patients with PAH in whom there is NO limitation of usual physical activity; ordinary physical activity does NOT cause increased dyspnea, fatigue, chest pain, or presyncope

Question 9

Class II

Answer 9

Patients with PAH who have mild limitation of physical activity; there is NO discomfort at rest, but normal physical activity causes increases dyspnea, fatigue, chest pain, or presyncope

Question 10

Class III

Answer 10

Patients with PAH who have marked limitation of physical activity; there is NO discomfort at rest, but less than normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope

Question 11

Class IV

Answer 11

Patients with PAH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure; dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity

Question 12

What is the gold standard for diagnosis of PAH?

Answer 12

right heart catheterization is the gold standard for evaluating pulmonary pressures and measurement of acute vasoreactivity testing

Question 13

Evaluating Pulmonary Vasoreactivity

Answer 13

uses fast-acting, short-duration vasodilators to determine: extent of smooth muscle constriction and vasodilator response

Question 14

Agents used to test vasoreactivity

Answer 14

Epoprostenol
Adenosine
Nitric oxide 99% of the time this is used

Question 15

If they have a Positive Response to vasoreactivity they must meet all requirements

Answer 15

1. decrease mPAP by at least 10 mmHg
2. decrease mPAP to value
3. Increased or unchanged CO

Question 16

Patient returns to clinic with increasing symptoms and is now WHO functional class III. She is currently receiving sildenafil 80 mg PO TID, warfarin 5 mg PO daily, furosemide 40 mg PO BID, and lisinopril 5 mg PO daily. She is very hesitant about starting a parenteral therapy and asks to try another oral option first. Which of the following would you recommend?

Answer 16

a. increase her sildenafil to 200 mg PO TID
b. add riociguat 1 mg PO TID
c. add treprostinil SC @ 2 ng/kg/min
d. add bosentan 62.5 mg PO BID
e. add macitentan 10 mg PO daily

An: D or E