title: Impaired Right, Left, or Biventricular Function and Resting Oxygen Saturation Are Associated With Mortality in Eisenmenger Syndrome: A Clinical and Cardiovascular Magnetic Resonance Study.
abstract: BACKGROUND
  Patients with Eisenmenger syndrome (ES) have better survival, despite similar pulmonary vascular pathology, compared with other patients with pulmonary arterial hypertension. Cardiovascular magnetic resonance (CMR) is useful for risk stratification in idiopathic pulmonary arterial hypertension, whereas it has not been evaluated in ES. We studied CMR together with other noninvasive measurements in ES to evaluate its potential role as a noninvasive risk stratification test.
  
  
  RESULTS
  Between 2003 and 2005, 48 patients with ES, all with a post-tricuspid shunt, were enrolled in a prospective, longitudinal, single-center study. All patients underwent a standardized baseline assessment with CMR, blood test, echocardiography, and 6-minute walk test and were followed up for mortality until the end of December 2013. Twelve patients (25%) died during follow-up, mostly from heart failure (50%). Impaired ventricular function (right or left ventricular ejection fraction) was associated with increased risk of mortality (lowest quartile: right ventricular ejection fraction, <40%; hazard ratio, 4.4 [95% confidence interval, 1.4-13.5]; P=0.01 and left ventricular ejection fraction, <50%; hazard ratio, 6.6 [95% confidence interval, 2.1-20.8]; P=0.001). Biventricular impairment (lowest quartile left ventricular ejection fraction, <50% and right ventricular ejection fraction, <40%) conveyed an even higher risk of mortality (hazard ratio, 8.0 [95% confidence interval, 2.5-25.1]; P=0.0004). No other CMR or noninvasive measurement besides resting oxygen saturation (hazard ratio, 0.90 [0.83-0.97]/%; P=0.007) was associated with mortality.
  
  
  CONCLUSIONS
  Impaired right, left, or biventricular systolic function derived from baseline CMR and resting oxygen saturation are associated with mortality in adult patients with ES. CMR is a useful noninvasive tool, which may be incorporated in the risk stratification assessment of ES during lifelong follow-up.
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Annette S
authors.initials: AS
authors.lastname: Jensen
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Craig S
authors.initials: CS
authors.lastname: Broberg
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Riikka
authors.initials: R
authors.lastname: Rydman
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.). m.gatzoulis@rbht.nhs.uk.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: From the National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, United Kingdom (A.S.J., C.S.B., R.R., G.-P.D., W.L., K.D., J.S.W., D.J.P., M.A.G., S.V.B.-N.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (A.S.J.); Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health and Science University Portland (C.S.B.); Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany (G.-P.D.).
authors.forename: Sonya V
authors.initials: SV
authors.lastname: Babu-Narayan
mesh_terms: D000328:Adult; D002423:Cause of Death; D003661:Decision Support Techniques; D018450:Disease Progression; D004452:Echocardiography; D004541:Eisenmenger Complex / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D005080:Exercise Test; D005260:Female; D006333:Heart Failure / Q000401:mortality / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000097:blood*; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013318:Stroke Volume; D013997:Time Factors; D006113:United Kingdom; D018487:Ventricular Dysfunction, Left / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
chemical_list: D010100:Oxygen
keywords: Eisenmenger syndrome; follow-up studies; magnetic resonance imaging; mortality