English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

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.Material 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: 159 patient were included (94 females: 59%), aged 27.7±14.8years 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%), 7 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 (1 agent in 20%, 2 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: Survival rates of adult patients with Eisenmenger Syndrome seem to improve up to 50-year of age with target therapy agents. These results have to be confirmed by larger scale multicentre studies.

Long-term survival and functional status of adult patient with Eisenmenger syndrome