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what is the definition of pulmonary arterial hypertension?

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


Pathophysiology of PAH

Caused by:
Pulmonary vasoconstriction
In situ thrombosis


WHO groups of Pulmonary Hypertension:
Group 1

Pulmonary arterial hypertension


Group 2

Pulmonary hypertension due to left heart disease


Group 3

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


Group 4

Chronic thromboembolic pulmonary hypertension


Group 5

Pulmonary hypertension with unclear multifactorial mechanisms


WHO Functional Classification of PAH:
Class I

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


Class II

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


Class III

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


Class IV

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


What is the gold standard for diagnosis of PAH?

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


Evaluating Pulmonary Vasoreactivity

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


Agents used to test vasoreactivity

Nitric oxide 99% of the time this is used


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

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


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?

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