Abstract
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<0.001). The discriminatory function was particularly high in the non-Es patients (figure).
Conclusion: The number of low-risk prognostic factors predicts outcome in ACHD-PAH. Further work to assess utility in follow-up is required.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, OA498.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019