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 Conclusion: These results support the relevance of ESC/ERS guidelines risk stratification to define treatment goals in patients with PAH.