Pulmonary artery hypertension

Points to remember about pulmonary artery hypertension

1. Pulmonary arterial hypertension (PAH) - mean pulmonary arterial pressure > 20 mm Hg and a pulmonary vascular resistance > 3 WU, with a normal capillary wedge pressure of < 15 mm Hg

2. Increased pulmonary resistance results in right ventricular overload and right heart failure

3. Mortality of at least 50% in 7 years

4. Pregnancy in women with PAH is associated with high maternal and neonatal mortality rates

5. The only therapy with a direct survival benefit is intravenous epoprostenol, a prostanoid vasodilator

6. A positive acute vasodilator response is defined by a decrease in mean PAP ≥ 10 mm Hg to reach a mean PAP < 40 mm Hg with an improved or stable cardiac output. In these patients, high-dose calcium channel blockers significantly lower PAP and vascular resistance.

7. Most common cause of death- right sided heart failure

8. Agents within the same class (including PDE-5 inhibitors and guanylate cyclase stimulators) should not be used together.

9. Riociguat, a soluble guanylate cyclase stimulator, is approved for the treatment of chronic thromboembolic pulmonary hypertension.