Therapy for primary pulmonary hypertension

Abstract

In summary, when considering therapy in a patient with PPH or pulmonary hypertension secondary to the CREST syndrome (with minimal interstitial lung disease), it is first appropriate to perform an acute vasodilator challenge with a Swan-Ganz catheter in place. If there is an acceptable acute vasodilator response (see above), the patient should be started on long term calcium channel blocker therapy. If there is not a significant acute vasodilator response, the therapeutic choice is between continous intravenous eposprostenol and oral bosentan. Despite the fact that eposprostenol is the only therapy with a demonstrated survival benefit, many specialist begin therapy with bosentan, and closely monitor patients for three to six months for clinical, functional and hemodynamic improvements. If no improvements are noted, therapy is often changed to epoprostenol in patients who are able to tolerate this very arduous therapy. In the absence of a contraindication, all patients with PPH should be anticoagulated for life. In patients that fail medical therapy, the only remaining option is lung transplantation. Although therapy for this disease is complicated, expensive and often onerous, we have come a long way in the past 10 to 15 years. Previously, the prognosis was appalling; there are now therapeutic options available that provide these patients with hope.