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Pulmonary arterial hypertension
P31 Disease targeted therapies and effect on survival in idiopathic, heritable and anorexigen-associated pulmonary arterial hypertension (PAH)
  1. Y Ling,
  2. M Johnson,
  3. A Peacock
  1. Scottish Pulmonary Vascular Unit, Glasgow, UK

Abstract

Introduction The median survival of idiopathic PAH was 2.8 years before the availability of modern therapies. Substantial progress has been made over the past 20 years. Currently available disease targeted therapies have been shown in clinical trials to improve symptoms, exercise capacity and survival. Combination therapies should also be considered in patients with inadequate clinical response to monotherapy (ESC/ERS 2009). We aim to assess the use of modern advance treatments in ‘real-life’ patients and their impact on survival.

Methods A retrospective review of all incident cases of idiopathic, heritable and anorexigen-associated PAH diagnosed between 1st January 2001 and 31st December 2009 in our unit. Baseline and follow-up data were collected from case notes and local pulmonary hypertension database.

Results 94 incident cases were diagnosed (62% female) with median age of 63 years. 36% (n=34) of patients were on combination therapy. All except one patient were started on monotherapy, with second agent added at later stage. The median time from initiation of monotherapy to introduction of the second agent was 244 days. 25% had prostanoids; 51% endothelin receptor antagonists and 81% phosphodiesterase type-5 inhibitor at some point of their treatment. The median survival is 5.63 years. We performed further analysis on the 28 patients who have died by censor date. Only seven patients were on prostanoids and eight patients on combination therapy. Possible explanations for low proportions of dead patients on prostanoids and/or combination therapy are outlined in Abstract P31 Table 1.

Abstract P31 Table 1

Possible explanations why prostanoids or combination therapies were not started

Conclusion Our results demonstrate improved survival compared to pre-advance therapy era. Apart from proven efficacy of various PAH specific treatment, several other factors may also influence the treatment decision for individual patient.

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