Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study

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TitreOutcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study
Type de publicationJournal Article
Year of Publication2017
AuteursHascoet S, Fournier E, Jais X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M
JournalARCHIVES OF CARDIOVASCULAR DISEASES
Volume110
Pagination303-316
Date PublishedMAY
Type of ArticleArticle
ISSN1875-2136
Mots-clésCongenital heart diseases, Drug therapy, Eisenmenger syndrome, outcome, Pulmonary arterial hypertension
Résumé

{Background. - The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. Aims. - To investigate outcomes in patients with ES, and their relationship with PAH-SDT. Methods. - Retrospective, observational, nationwide, multicentre cohort study. Results. - We included 340 patients with ES: genetic syndrome (n =119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE51]) 46.7%; dual therapy (ERA + PDE51) 40.9%; triple therapy (ERA+PDE51+prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0-9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4-47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time, scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P=0.0001 and P=0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P< 0.001 and P< 0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P=0.002

DOI10.1016/j.acvd.2017.01.006