Methods and results: We included 210 adult patients with ES (age 37.1±12.9 ... predictive of outcome in this contemporary ES cohort and, thus, cannot be solely.
Congenital heart disease: predictors of outcome / Congenital heart disease: aortic disease
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P2107 | BEDSIDE Six minute walk test but not functional class predicts outcome in adult patients with Eisenmenger syndrome
P2110 | BEDSIDE Bicuspid aortic valve morphology may have prognostic value in fetal Turner syndrome
A. Kempny 1 , K. Dimopoulos 1 , R. Alonso-Gonzalez 1 , M. Alvarez-Barredo 1 , A. Uebing 1 , D. Marchewka 2 , A. Tutaj 2 , L. Swan 1 , G.P. Diller 1 , M.A. Gatzoulis 1 . 1 Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom; 2 AGH University of Science & Technology, Cracow, Poland
K. Van Engelen 1 , M.M. Bartelings 2 , A.C. Gittenberger-De Groot 2 , M.J.H. Baars 1 , A.V. Postma 3 , B.J.M. Mulder 4 , M.R.M. Jongbloed 5 . 1 Academic Medical Center, Department of Clinical Genetics, Amsterdam, Netherlands; 2 Leiden University Medical Center, Department of Anatomy and Embryology, Leiden, Netherlands; 3 Academic Medical Center, Heart Failure Research Center, Dept. of Anatomy, Embryology and Physiology, Amsterdam, Netherlands; 4 Academic Medical Center, University of Amsterdam, Department of Cardiology, Amsterdam, Netherlands; 5 Leiden University Medical Center, Department of Cardiology, Leiden, Netherlands
Objectives: We assessed the prognostic value of 6-minute-walk-test (6MWT) in adult patients with Eisenmenger syndrome (ES). Background: ES represents the extreme manifestation of pulmonary arterial hypertension in patients with congenital heart disease, associated with significant exercise intolerance and mortality. Even though 6MWT is routinely used in these patients, little is known about its prognostic value in comparison to functional class. Methods and results: We included 210 adult patients with ES (age 37.1±12.9 years, 141 female, 37% in WHO functional class >II) who underwent a total of 822 6MWTs. Median walking distance (6MWD) was 330m [IQR 260-395], oxygen saturation (SO2) at baseline 86% [IQR 82-91%] and SO2 at peak-exercise 69% [IQR 60-80%]. During a follow up of 3.3 years, 29 patients died. On timedependent Cox analysis, 6MWD (HR 0.94 per 10m, 95% CI: 0.91-0.97, p
