ACCSAP Pulmonary Circulation

Question 1

What is the preferred initial treatment for patients with idiopathic PAH?

Answer 1

ambrisentan and tadalafil.
(Combination of PDE5 inhibitor and either endothelin receptor antagonist or a prostanoid should be considered).

Sildenafil alone improves 6-minute walk distance and hemodynamics, but not the secondary endpoint of time to clinical worsening.

Question 2

What other PAH meds are contraindicated with guanylate cyclase inhibitors (riociguat)?

Answer 2

PDE5 inhibitors

Question 3

What are some endothelin receptor antagonists?

Answer 3

Bosentan, ambrisentan

Question 4

What is a generally accepted goal of therapy for patients with PAH?

Answer 4

Achieving 6-minute walk distances above ~380-440 feet is a generally accepted goal for patients on therapies for pulmonary artery hypertension

Question 5

What are some prostacyclin drugs for PAH?

Answer 5

Inhaled epoprostenol, SQ or IV treprostinil (remodulin)

Question 6

What are the 5 WHO groups of pulmonary HTN?

Answer 6

Group 1 PAH is either idiopathic, heritable, drug- and toxin-related, or associated with connective tissue disease, portal hypertension, HIV, or congenital disease. Group 2 is secondary to left-heart disease.
Group 3 is secondary to lung disease or hypoxia.
Group 4 is due to thromboembolic and other pulmonary artery obstructive disease.
Group 5 is unclear and/or multifactorial etiology, such as hematologic or systemic inflammatory disorders such as sarcoid.

Question 7

What is the initial therapy for a patient with high-risk precapillary pulmonary hypertension (high RA pressure, low CI) and World Health Organization (WHO) functional class 4 status?

Answer 7

Combination of intravenous prostacyclin and an oral endothelin antagonist

Question 8

What is a positive response to vasodilator testing for PH?

Answer 8

Decrease in mean PAP of at least 10 mm Hg to an absolute mean PAP <40 mm Hg without a decrease in cardiac output.

Acute vasodilator testing is not indicated, and may be potentially harmful in patients with significantly elevated left heart filling pressures, because the acute reduction in the pulmonary resistance with high downstream pressures may result in pulmonary edema.