Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study

Continuous intravenous epoprostenol improves exercise capacity, haemodynami cs, and survival in severe primary pulmonary hypertension. Pulmonary hypert ension can also be life-threatening in patients with connective tissue dise ases, In a prospective open monocentre uncontrolled study, the effects of epopros tenol were evaluated in patients with severe pulmonary hypertension seconda ry to connective tissue diseases who were unresponsive to oral vasodilators (including calcium channel blockers) and continued to be in the New York H eart Association (NYHA) functional class III or IV despite conventional med ical therapy. Seventeen patients received epoprostenol administered by a po rtable infusion pump associated with conventional therapy (oral anticoagula nts, diuretics, supplemental oxygen), During the first six weeks of therapy, two (12%) patients died, of pulmonar y oedema (n=1) and severe sepsis (n=1). In the fifteen remaining subjects, clinical and haemodynamic parameters improved significantly at six weeks, T hese patients were subsequently monitored for 80+/-48 (range 14-154) weeks after initiation of epoprostenol. Five (33%) patients died, of right heart failure (n=2), severe sepsis (n=2) or syncope (n=1) and two patients were s uccessfully transplanted 24 and 52 weeks after initiation of epoprostenol, Seven of the remaining eight patients had a persistent clinical improvement . Short-term epoprostenol therapy is effective in some patients with connecti ve tissue diseases as demonstrated by better clinical status and haemodynam ics at six weeks. However, this study reports several cases of early and la te major complications including severe sepsis and pulmonary oedema, Additi onal information is needed to evaluate the benefit: risk ratio of long-term epoprostenol therapy in pulmonary hypertension secondary to connective tis sue diseases.