Risk Categories from European Guidelines Applied to the French Pulmonary Hypertension (PH) Registry

Background: Current European (ERS/ESC) PH guidelines recommend risk assessment in patients with pulmonary arterial hypertension (PAH). The aim of our study were to analyze the association between the number of determinants of low-risk criteria achieved and long-term outcome. Methods: All patients with idiopathic, heritable and drug-induced PAH entered into the French Registry from 2006 and reassessed within a year were analyzed. The Cox model was used to determine the weight of 4 low-risk criteria achieved at first follow-up visit: NYHA FC, 6MWD, right atrial pressure (RAP) and cardiac index (CI). Results: 1017 patients were analyzed (mean age 57 yrs, 59% female, 75% iPAH). After a median follow-up of 34 months, 23% had died. In univariate analysis, HR (95%CI) for NYHA FC I-II, 6MWD>440 m, RAP<8 mmHg and CI≥2.5 L/min/m2 were 0.28 (0.21-0.36), 0.18 (0.12-0.27), 0.51 (0.39-0.65) and 0.50 (0.39-0.65), respectively. At first follow-up visit (5.6±3.4 mo.), the proportion of patients achieving 1, 2, 3 and 4 low-risk criteria were 22%, 27%, 24.5% and 17%, respectively. Figure 1 shows KM survival curves according to the number of low-risk criteria achieved. Three-year survival was 97%, 93%, 81%, 68% and 40% in patients who achieved 4, 3, 2, 1 and no low-risk criteria, respectively (p<0.00001). Conclusion: These results support the relevance of ESC/ERS guidelines risk stratification to define treatment goals in patients with PAH.