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P173 Prognostic factors for survival in idiopathic pulmonary arterial hypertension

Introduction Idiopathic pulmonary arterial hypertension (IPAH) is rare with an estimated prevalence of 5.9 cases/million population.1 Untreated, prognosis is poor with one year survival 68%.2 The ESC/ERS guidelines’ risk assessment tool estimates one year mortality.1 This tool was used to assess the number of green prognostic factors and observe survival. Methods Patients were identified retrospectively from a cohort of incident IPAH patients. Prognostic factors of WHO functional class, 6MWT and NT-proBNP were selected at 0 (baseline), 6 and 12 months. The number of green prognostic factors at baseline was compared with 6 and 12 months and survival observed. Results 28 patients were identified; 11 male (39%), 17 female (61%), mean age at diagnosis 65 years (range 42–85). At baseline 19 (68%) patients had no green prognostic factors compared with 12 (43%) at 6 months (p=0.0045) and 11 (39%) at 12 months (p=0.002). At baseline 8 (29%) had 1 green prognostic factor compared with 5 (18%) at 6 months (p=0.08) and 10 (36%) at 12 months (p=0.17). At baseline 1 (3%) had 2 green prognostic factors compared with 11 (39%) at 6 months (p=<0.0001) and 7 (25%) at 12 months (p=0.006). No patients had 3 green prognostic factors. The number of patients with 0 red prognostic factors increased from 13 (46%) at baseline to 20 (71%) at 12 months (p=0.005). At one year all patients survived. Conclusions There was a statistically significant increase (p=<0.05) in the number of patients with two green (low risk of mortality) prognostic factors at 6 and 12 months and patients with zero red (high risk) prognostic factors at 12 months, compared with baseline. A concurrent statistically significant reduction in number of patients with no green prognostic factors was seen. These suggest decreased one year mortality. One year survival between patients with 0, 1 or 2 green prognostic factors was identical. Patients are being followed for long term survival. References ESC/ERS. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016;37:67–119. D’Alonzo GE. Survival in patients with primary pulmonary hypertension: Results from a National Prospective Registry. Ann Intern Med 1991;115(5):343–9.