Continuous intravenous
epoprostenol improves exercise capacity, haemodynamics, and survival in severe
primary pulmonary hypertension.
Pulmonary hypertension can also be life-threatening in patients with
connective tissue diseases. In a prospective open monocentre uncontrolled study, the effects of
epoprostenol were evaluated in patients with severe
pulmonary hypertension secondary to
connective tissue diseases who were unresponsive to oral
vasodilators (including
calcium channel blockers) and continued to be in the New York Heart Association (NYHA) functional class III or IV despite conventional medical
therapy. Seventeen patients received
epoprostenol administered by a portable
infusion pump associated with conventional
therapy (oral
anticoagulants,
diuretics, supplemental
oxygen). During the first six weeks of
therapy, two (12%) patients died, of pulmonary oedema (n = 1) and
severe sepsis (n = 1). In the fifteen remaining subjects, clinical and haemodynamic parameters improved significantly at six weeks. These 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 successfully 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
connective tissue diseases as demonstrated by better clinical status and haemodynamics at six weeks. However, this study reports several cases of early and late major complications including
severe sepsis and pulmonary oedema. Additional information is needed to evaluate the benefit: risk ratio of long-term
epoprostenol therapy in
pulmonary hypertension secondary to
connective tissue diseases.