title: Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study.
abstract: BACKGROUND
  Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients.
  
  
  METHODS
  In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters.
  
  
  RESULTS
  We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59;P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39;P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65;P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88;P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66;P<0.001) remained significant predictors of death.
  
  
  CONCLUSIONS
  There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.). kempny@gmail.com.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Kempny
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Cristel Sørensen
authors.initials: CS
authors.lastname: Hjortshøj
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Hong
authors.initials: H
authors.lastname: Gu
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Alexander R
authors.initials: AR
authors.lastname: Opotowsky
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Landzberg
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Annette Schophuus
authors.initials: AS
authors.lastname: Jensen
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Lars
authors.initials: L
authors.lastname: Søndergaard
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Mette-Elise
authors.initials: ME
authors.lastname: Estensen
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Ulf
authors.initials: U
authors.lastname: Thilén
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Werner
authors.initials: W
authors.lastname: Budts
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Mulder
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Ilja
authors.initials: I
authors.lastname: Blok
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Lidia
authors.initials: L
authors.lastname: Tomkiewicz-Pająk
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Kamil
authors.initials: K
authors.lastname: Szostek
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Scognamiglio
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Katja
authors.initials: K
authors.lastname: Prokšelj
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D004452:Echocardiography; D004541:Eisenmenger Complex / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption; D010641:Phenotype; D011379:Prognosis; D016016:Proportional Hazards Models; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D000070857:Walk Test; D055815:Young Adult
chemical_list: D015415:Biomarkers
keywords: Eisenmenger complex; mortality; pulmonary hypertension