title: Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein's anomaly of the tricuspid valve.
abstract: BACKGROUND
  Ebstein's anomaly of the tricuspid valve often results in biventricular dysfunction and functional deterioration. Little is known about the relation between exercise capacity, disease severity and outcome in adults with Ebstein's anomaly.
  
  
  METHODS
  Data on all patients with Ebstein's anomaly of the tricuspid valve who underwent cardiopulmonary exercise testing in our tertiary center were collected. The relation between exercise parameters, anatomic severity (Glasgow outcome score) and the combined end-point of death, non-elective hospitalization and surgical repair was studied using Cox regression analysis.
  
  
  RESULTS
  A total of 51 adult patients fulfilled inclusion criteria (49% male, mean age 37.8±13.6 years). Mean peak oxygen uptake (peak VO2) was 63.2±18.7% of predicted, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) 37.4±11.4, heart rate reserve (HRR) 23.6±22.7 bpm. A significantly lower peak VO2 was found in patients with a higher Glasgow outcome score, higher cardiothoracic ratio and documented atrial shunt. Peak VO2 (HR for value <60% of predicted 3.47, 95% CI: 1.28–9.44, p=0.015) and HRR (HR for value <25 bpm 3.07, 95% CI: 1.24–7.61, p=0.016) were significant predictors of outcome, the former being the strongest on multivariable analysis.
  
  
  CONCLUSIONS
  Reduced exercise capacity in patients with Ebstein's anomaly relates to severity of the underlyingdisease and is a strong and independent predictor of outcome. Cardiopulmonary exercise testing should be incorporated in the follow-up and risk stratification of patients with this relatively uncommon and challenging cardiac defect.
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK. Electronic address: jelenaradojevic0708@gmail.com.
authors.forename: Jelena
authors.initials: J
authors.lastname: Radojevic
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; National Heart and Lung Institute (NHLI), Imperial College, London, UK.
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso-Gonzalez
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Francesco
authors.initials: F
authors.lastname: Borgia
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Georgios
authors.initials: G
authors.lastname: Giannakoulas
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; National Heart and Lung Institute (NHLI), Imperial College, London, UK.
authors.forename: Matina
authors.initials: M
authors.lastname: Prapa
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.
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.
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; National Heart and Lung Institute (NHLI), Imperial College, London, UK.
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; National Heart and Lung Institute (NHLI), Imperial College, London, UK.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; National Heart and Lung Institute (NHLI), Imperial College, London, UK.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000378:metabolism; D010101:Oxygen Consumption / Q000502:physiology*; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000002:abnormalities* / Q000378:metabolism*; D055815:Young Adult
chemical_list: D010100:Oxygen