Survival in Primary Pulmonary Hypertension

Abstract

Background-Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration-approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined. Methods and Results-One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure.

Noninvasive cardiac output measurements in patients with pulmonary hypertension

“…Moreover, NICOM demonstrated superior precision to TD and reliably detected dynamic, directional changes in CO following vasodilator challenge. Among the many variables obtained during an invasive haemodynamic assessment, the CO has arguably the most prognostic relevance and affects clinical decision strategies more than any other value in patients with PH [2,[13][14][15]. Accordingly, having the ability to easily measure and detect changes in CO noninvasively may have importance in the clinical care and assessment of treatments in patients with PH.…”

“…Because CO, as measured by either TD or indirect Fick, is an important predictor of outcomes in PH [2,14,15], clinical PH specialists often track changes in CO over time to assess response to therapy or the need to escalate care. For example, MCLAUGHLIN et al [13] showed that an improvement in cardiac index after initiation of epoprostenol therapy in patients with PAH predicted improved survival.…”

Guidelines for the diagnosis and treatment of pulmonary hypertension

“…Epoprostenol improves symptoms, exercise capacity and haemodynamics in both clinical conditions, and is the only treatment shown to improve survival in IPAH in a randomised study [118]. Long-term persistence of efficacy has also been shown [79,97] can be accomplished by a microinfusion pump and a small subcutaneous catheter. The effects of treprostinil in PAH were studied in the largest worldwide RCT performed in this condition, and showed improvements in exercise capacity, haemodynamics and symptoms [128].…”

Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids

“…Although it is undisputed that this treatment is highly effective, there are significant risks associated with the permanent central venous access and delivery system required for an uninterrupted supply of epoprostenol [5,6]. These problems have led to the development of novel prostacyclin derivatives, which can be administered subcutaneously, orally or by means of inhalation.…”