title: Exercise intolerance in patients with congenitally corrected transposition of the great arteries relates to right ventricular filling pressures.
abstract: BACKGROUND
  Patients with congenitally corrected transposition of the great arteries (ccTGA) have significantly reduced exercise tolerance. Progressive right ventricular (RV) dysfunction with tricuspid regurgitation (TR) and other haemodynamic lesions are common among them. We hypothesised that interaction of these factors may result in increased systemic RV filling pressure, which in turn impact on exercise capacity.
  
  
  METHODS
  Patients with ccTGA in functional class I or II, able to perform treadmill exercise and without resting cyanosis were enrolled. All patients underwent cardiopulmonary exercise testing and transthoracic echocardiographic examination. RV filling pressure was estimated using tissue Doppler imaging (TDI) techniques by measuring early annular diastolic velocity (Ea) and the ratio of the transtricuspid inflow to the early annular diastolic velocity (E/Ea).
  
  
  RESULTS
  A total of 27 patients (mean age 41 years, 48% female) were assessed, the majority (63%) asymptomatic. Many patients had coexistent haemodynamic lesions including shunts, pulmonary stenosis, TR and systemic ventricular dysfunction. Average percentage predicted peak oxygen consumption, VE/VCO(2) slope and heart rate reserve were abnormal in this population. Patients with moderately/severely impaired exercise capacity (≤ 60% predicted peak VO(2)) had significantly higher E/Ea ratios compared to those with normal/mildly impaired exercise capacity (septal E/Ea = 17.1 ± 9.7 vs 8.8 ± 1.6 and lateral E/Ea = 11.5 ± 5.8 vs 6.6 ± 1.3, p = 0.007 and 0.01 respectively).
  
  
  CONCLUSIONS
  Reduced exercise capacity is common in adults with ccTGA even among asymptomatic patients and relates to increased RV filling pressures assessed by TDI. This index could potentially be used to optimize therapy or prognosticate adverse events in ccTGA patients.
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
authors.forename: Edgar L W
authors.initials: EL
authors.lastname: Tay
authors.forename: Alexandra
authors.initials: A
authors.lastname: Frogoudaki
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.forename: Georgios
authors.initials: G
authors.lastname: Giannakoulas
authors.forename: Matina
authors.initials: M
authors.lastname: Prapa
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.forename: George
authors.initials: G
authors.lastname: Pantely
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D004452:Echocardiography; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption / Q000502:physiology; D011379:Prognosis; D011652:Pulmonary Circulation / Q000502:physiology; D014188:Transposition of Great Vessels / Q000503:physiopathology / Q000601:surgery*; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000503:physiopathology*; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*; D017725:Ventricular Pressure / Q000502:physiology*