Risk stratification of pulmonary arterial hypertension (PAH) associated with adult congenital heart disease (ACHD)

Introduction: The role of risk stratification is an established part of the management of most forms of PAH.‎ There are, however, few data regarding risk stratification in ACHD-PAH. Methods: Consecutive ACHD-PAH patients diagnosed at a pulmonary hypertension referral centre during 1995-2014 were identified from the ASPIRE database. Survival according to the number of low-risk previously-identified prognostic factors (ISWD > 420m, DLco > 60%, WHO FC ≤ II and post-tricuspid defect) was assessed. Results: 240 ACHD-PAH patients were identified:138 Eisenmenger (Es), 56 moderate-large defects with left-to-right shunts, 16 small defects and 30 with closed defects. 69 patients had post-tricuspid defects. At diagnosis, 30%, 41%, 18%, 8%, 3% of patients had 0, 1, 2, 3 or 4 low-risk criteria. There was a significant difference in transplant-free survival at baseline between the risk groups (p Conclusion: The number of low-risk prognostic factors predicts outcome in ACHD-PAH. Further work to assess utility in follow-up is required.