Abstract 2263: Outcomes in Adults With Eisenmenger Physiology Receiving Endothelin Antagonist Therapy
Eisenmenger physiology in adults with congenital heart disease (ACHD) causes significant limitation of exercise tolerance and associated morbidity. We studied the outcomes in patients treated with the non selective endothelin antagonist bosentan. Data from 23 ACHD patients, including 5 with Down’s syndrome who received bosentan were prospectively collected. Parameters included baseline demographics, 6-minute walk tests (6MWT), haematology and liver function tests, and major adverse events. Due to the difficulties in assessing exercise tolerance accurately in patients with Down’s syndrome, 6MWT data were analysed only in non-Down’s patients. Wilcoxon signed-rank tests were used for analysis. Mean age was 36.3 ± 13.6 years. There were 14 females (61%) and 17 (74%) were Caucasian. All except 1 with Down’s Syndrome were commenced on bosentan 62.5 mg b.i.d., titrated to 125 mg b.i.d. after 4 weeks. Mean follow-up for all patients was 50.4 ± 35.8 weeks (range, 2–116 weeks). Mean baseline 6MWT distance was 309.1 ± 106.5 m. and at 1, 3, 6, 9 and 12 months were 354.0 ± 93.4, 401.0 ± 54.0, 376.6 ± 97.3, 415.6 ± 82.2 and 403.2 ± 97.2 respectively (p<0.05 vs. baseline for all values). At patients’ last available visit the mean increase of 6MWT distance from baseline was 82.2 ± 70.6 m (p<0.001). Changes in oxygen saturation at rest, aspartate aminotransferase (AST), alanine aminotransferase (ALT) and haemoglobin are given in Table 1⇓. None of these changes were of major clinical relevance. No patient had bosentan discontinued due to adverse events. Two patients with Down’s syndrome died during follow up due to non cardiac causes. Treatment with bosentan treatment resulted in significant improvement in exercise capacity in ACHD patients with Eisenmenger physiology, sustained at 1 year. There was no evidence of significant hepatic dysfunction in this study and the drug appears to be well tolerated in patients with Down’s syndrome.