title: Late cardiopulmonary and musculoskeletal exercise performance after repair for total anomalous pulmonary venous connection during infancy.
abstract: OBJECTIVE
  We evaluated cardiopulmonary function at rest and during exercise in children after surgical repair for total anomalous pulmonary venous connection.
  
  
  BACKGROUND
  Long-term assessment of cardiopulmonary function during exercise in children after repair for total anomalous pulmonary venous connection during infancy is limited.
  
  
  METHODS
  Resting lung function and cardiopulmonary function during maximal ramp cycle ergometry were evaluated in 27 patients (age = 11 +/- 4 years, 20 were male). Peak oxygen consumption, ventilatory anaerobic threshold, and physical working capacity were compared with normal reference values. Neurologic assessment included neuromuscular function, inattentiveness, and hyperactivity. Patient- and procedure-related variables were assessed for association with peak oxygen consumption, ventilatory anaerobic threshold, and physical working capacity.
  
  
  RESULTS
  Compared with healthy children, peak oxygen consumption (88% +/- 16% of predicted) and ventilatory anaerobic threshold (91% +/- 21% of predicted) were mildly reduced. Chronotropic impairment was observed in 7 patients (32%). Patients with impaired resting lung mechanics were more likely to have impairment in peak oxygen consumption (P < .05). Breathing reserve was normal. Specific anatomy and all operative factors did not have a significant impact on overall exercise performance. Composite score for fine and gross motor function was associated with lower ventilatory anaerobic threshold (P < .05).
  
  
  CONCLUSIONS
  Exercise performance is mildly impaired at long-term follow-up after total anomalous pulmonary venous connection repair during infancy. Residual pulmonary abnormalities are common and associated with lower exercise performance. Neurologic abnormalities are evident in a subgroup, but the impact on late exercise performance is inconclusive.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa 19104, USA. mcbride@email.chop.edu
authors.forename: Michael G
authors.initials: MG
authors.lastname: McBride
authors.forename: Paul M
authors.initials: PM
authors.lastname: Kirshbom
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
authors.forename: Richard F
authors.initials: RF
authors.lastname: Ittenbach
authors.forename: Gil
authors.initials: G
authors.lastname: Wernovsky
authors.forename: Robert R
authors.initials: RR
authors.lastname: Clancy
authors.forename: Thomas B
authors.initials: TB
authors.lastname: Flynn
authors.forename: Diane M
authors.initials: DM
authors.lastname: Hartman
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Spray
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.forename: Mayra C
authors.initials: MC
authors.lastname: Santiago
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Paridon
mesh_terms: D015308:Anaerobic Threshold; D002648:Child; D004452:Echocardiography; D005080:Exercise Test; D017079:Exercise Tolerance; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D006334:Heart Function Tests; D006801:Humans; D016014:Linear Models; D008297:Male; D009460:Neurologic Examination; D010101:Oxygen Consumption / Q000502:physiology; D011667:Pulmonary Veins / Q000002:abnormalities*; D012129:Respiratory Function Tests; D015656:Respiratory Mechanics