title: Physiological differences between various types of Eisenmenger syndrome and relation to outcome.
abstract: BACKGROUND
  Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) related to congenital heart disease. Several studies have suggested that the presence and location of the shunt defines the natural history of these patients by influencing right ventricular adaptation to PAH. We aimed to echocardiographically assess differences in cardiac physiology and outcome between various types of ES.
  
  
  RESULTS
  In this longitudinal cohort study, 191 patients with ES and non-complex congenital heart disease were recruited, 36 with pre-tricuspid and 155 with post-tricuspid shunts. Patients with pre-tricuspid shunts were older, had higher BNP concentrations and lower exercise tolerance compared to patients with post-tricuspid shunts. Right ventricular (RV) function was impaired in patients with atrial septal defects, with larger right ventricles, impaired systolic function and adaptation. The left ventricular eccentricity index was significantly higher in pre-tricuspid defects. Within post-tricuspid shunts, patients with atrio-ventricular septal defects had better right ventricular function compared to ventricular septal defects, while in those with a patent ductus arteriosus this was worse. There was a trend towards lower mortality in patients with post versus pre-tricuspid shunts, which was significant for patients above the age of 48 years.
  
  
  CONCLUSIONS
  The presence of a post-tricuspid shunt appears to carry physiological and possibly prognostic benefits in ES compared to patients with pre-tricuspid shunts. This should be borne in mind when management decisions and advanced therapies are considered.
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; Cardiology Department, Pasteur Hospital, University Hospital of Nice, France.
authors.forename: Pamela
authors.initials: P
authors.lastname: Moceri
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Kempny
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Emmanouil
authors.initials: E
authors.lastname: Liodakis
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso Gonzales
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Germanakis
authors.affiliation: Division of Adult Congenital Disease, Department of Cardiovascular Medicine, University Hospital of Münster, Germany.
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Lorna
authors.initials: L
authors.lastname: Swan
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Philip S
authors.initials: PS
authors.lastname: Marino
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Sonya V
authors.initials: SV
authors.lastname: Babu-Narayan
authors.affiliation: Cardiology Department, Pasteur Hospital, University Hospital of Nice, France.
authors.forename: Emile
authors.initials: E
authors.lastname: Ferrari
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK. Electronic address: k.dimopoulos02@gmail.com.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
mesh_terms: D000328:Adult; D004452:Echocardiography; D004541:Eisenmenger Complex / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D016019:Survival Analysis
keywords: Congenital heart disease; Echocardiography; Eisenmenger syndrome; Pulmonary arterial hypertension; Right ventricular function