Open AccessFactors influencing outcomes in patients with Eisenmenger syndrome: a nine‐year follow‐up study
Author(s) -
Clavé Mariana M.,
Maeda Nair Y.,
Castro Claudia R. P.,
Bydlowski Sergio P.,
Lopes Antonio A.
Publication year - 2017
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045893217721928
Subject(s) - medicine , eisenmenger syndrome , hazard ratio , quartile , confidence interval , proportional hazards model , cohort , pulmonary hypertension
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‐naïve 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.