Factors influencing outcomes in patients with Eisenmenger syndrome: a nine-year follow-up study

Autor(es)

Clave, Mariana M. ; Maeda, Nair Y. ; Castro, Claudia R. P. ; Bydlowski, Sergio P. ; Lopes, Antonio A.

• Tipo de documento: Artigo Científico
• Fonte: PULMONARY CIRCULATION; v. 7, n. 3, p. 635-642, JUL-SEP 2017.
• Citações Web of Science: 4

Resumo

In patients with Eisenmenger syndrome, life expectancy is usually longer than in patients with other forms of pulmonary arterial hypertension (PAH). We conducted a cohort study in which patients were followed over a long period of time in an attempt to identify potential predictors of clinical outcomes. Sixty-seven treatment-naive patients were enrolled (age range = 12-60 years; median age = 33 years). Baseline demographic, diagnostic, and functional parameters, plasma levels of endothelial dysfunction markers, and treatment-related data were tested for possible correlations with event-free survival. Patients were started on oral PAH drugs at the beginning of follow-up (n = 23), during follow-up (n = 33), or remained untreated (n = 11). The duration of follow-up was 0.54-9.89 years (median = 7.13 years), with an overall survival rate of 82% and an event-free survival rate of 70%. The estimated mean for event-free survival time was 7.71 years (95% confidence interval {[}CI] = 6.86-8.55 years). Of the 16 variables that were analyzed, the duration of exposure to PAH drugs was identified as an independent protective factor (hazard ratio {[}HR] = 0.25 for quartiles, 95% CI = 0.14-0.47, P < 0.001). The initial functional class (HR = 3.07; 95% CI = 1.01-9.34; P = 0.048), the severity of right ventricular dysfunction (HR = 2.51 {[}mild, moderate or severe dysfunction]; 95% CI = 1.22-5.19; P = 0.013) and plasma von Willebrand factor concentration (HR = 1.74 for quartiles; 95% CI = 1.07-2.83; P = 0.026) were identified as risk factors. The length of exposure to oral PAH therapies influences survival favorably in Eisenmenger patients. This may be of interest for communities where access to medications is restricted. (AU)

• Processo FAPESP: 12/10739-0 - Estudo dos efeitos do uso crônico da tadalafila, independentemente e comparativamente à Sildenafila, em portadores de hipertensão arterial pulmonar associada a cardiopatias congênitas em sua forma mais avançada (Síndrome de Eisenmenger)
• Beneficiário: Antonio Augusto Barbosa Lopes
• Modalidade de apoio: Auxílio à Pesquisa - Regular