The most appropriate management is sildenafil. In patients with pulmonary arterial hypertension (PAH) and respiratory symptoms occurring during exertion but not at rest, oral medications are the preferred initial therapy. Examples of oral agents include phosphodiesterase-5 inhibitors (sildenafil, tadalafil) and endothelin receptor antagonists (bosentan, ambrisentan). Prostanoids are available only as parenteral (epoprostenol) or inhaled (iloprost) forms and are typically reserved for patients with more advanced disease and symptoms and for those in whom disease progresses despite oral therapy. Additional treatments that are provided for most patients with PAH include supplemental oxygen for documented hypoxemia and chronic warfarin therapy. Pregnancy can worsen pulmonary hemodynamics and increase the risk of mortality in patients with PAH, so women of childbearing age should be counseled to avoid pregnancy.

In patients who have a positive response to vasoreactivity testing (a decrease in mean pulmonary artery pressure of at least 10 mm Hg without significant reductions in cardiac output or systemic blood pressure), oral calcium channel blockers such as nifedipine are a reasonable initial therapy. In those with a negative vasoreactivity test, as in this patient, calcium channel blockers are not beneficial.

Recent clinical trials have shown supervised exercise programs to be safe and to improve walking distances and quality of life in patients with PAH. Excessive exertion resulting in respiratory distress should be avoided.