Background.– In the context of new target therapies, this study aimed to assess the functional status and long-term outcomes of patients with Eisenmenger Syndrome reaching adulthood.

Patients and methods.– This is a single-centre retrospective review of all patients diagnosed with Eisenmenger Syndrome. Demographics, clinical data, underlying cardiac disease, functional status, therapeutics and outcomes were collected.

Results.– One hundred and fifty-nine patients were included (94 females: 59%), aged 27.7 ± 14.8 years at end-follow up, and 60 with Down syndrome (38%). Underlying cardiac disease was: AVSD in 30%, VSD in 35%, ASD in 9%, PDA in 5%, associated shunts in 5%, complex CHD in 10%, left heart obstruction in 2.5%, pulmonary veins anomaly in 2.5% and TGA in 1%. CHD was native in 122 cases (77%), seven had palliation (4%) and 30 complete repair (19%). Pulse oxygen saturation was 84 ± 12% (range 44 to 98%), lower in non-operated or palliated cases (81%) than in repaired cases (92%, P = 0.002). Patients were in NYHA class I (18%), class II (42%), class III (37%) or IV (3%), not different with previous repair or not. Target therapy agents were given in 35% of the cases (one agent in 20%, two associated in 13%, intravenous epoprostenol in 1.5%). Death occurred in 26 patients (16%) at the age of 29.3 ± 17.8years. Complications occurred in most of the cases (64%) including: hemorrhages events, syncopes, thrombo-embolia, cerebral abscess, infective endocarditis, heart failure or arrhythmias. NYHA class did not differ between patients with or without target therapy. SpO2 was 82% in untreated cases compared to 86% in treated cases (NS). Survival rates were: 98% at 10-years, 93% at 20-years, 87% at 30-years, 83% at 40-years, 73% at 50-years and 53% at 60-years of follow-up. Survival was lower in Down patients ( P = 0.0023), in males ( P = 0.04) and tends to be higher up to 50-years of age in patients under target therapy ( P = 0.05).

Conclusion.– The survival rate of adult patients with Eisenmenger Syndrome seems to improve up to 50 years of age with target therapy agents. These results have to be confirmed by larger scale multicentre studies.