title: Cardiopulmonary function in adult patients late after Fontan repair.
abstract: OBJECTIVE
  The clinical status and exercise assessment of adult patients late after the Fontan operation were reviewed to determine cardiovascular function.
  
  
  BACKGROUND
  The Fontan operation is the final operation for many patients with tricuspid atresia or a single ventricle. Follow-up reports describe most patients to be in Canadian Cardiovascular Society functional class I or II. Objective measures of cardiac performance in the pediatric age group have shown significant dysfunction.
  
  
  METHODS
  Forty-seven adult patients were seen late after the Fontan operation at the Toronto Congenital Cardiac Centre for Adults. Thirty of these underwent cycle ergometry to determine maximal exercise capacity. Maximal ventilation, maximal oxygen uptake and anaerobic threshold were determined from a ramp exercise protocol. Ejection fraction at rest and during exercise was measured with gated radionuclide angiography. Results were compared with those of eight normal volunteers. Results are given as mean +/- SD.
  
  
  RESULTS
  Thirty patients underwent cardiopulmonary exercise testing 6.7 +/- 3.9 years after a first Fontan operation. Clinically 93% were in functional class I or II. The Fontan group patients had a significantly lower maximal work load (548 +/- 171 vs. 1,094 +/- 190 kilopond-meters, p < 0.00001), anaerobic threshold (11.2 +/- 2.9 vs. 23.6 +/- 4.6 ml/kg per min) and maximal oxygen consumption (14.8 +/- 4.5 vs. 42.1 +/- 10.0 ml/kg per min). Systemic ventricular ejection fraction was lower at rest (38 +/- 12% vs. 58 +/- 7%) and during exercise (40 +/- 15% vs. 70 +/- 8%).
  
  
  CONCLUSIONS
  Despite a clinical impression of good function, by objective measures adult patients continue to have significant cardiovascular limitation late after the Fontan operation.
authors.affiliation: Toronto Congenital Cardiac Centre for Adults, Toronto Hospital, Ontario, Canada.
authors.forename: D A
authors.initials: DA
authors.lastname: Harrison
authors.forename: P
authors.initials: P
authors.lastname: Liu
authors.forename: J E
authors.initials: JE
authors.lastname: Walters
authors.forename: J M
authors.initials: JM
authors.lastname: Goodman
authors.forename: S C
authors.initials: SC
authors.lastname: Siu
authors.forename: G D
authors.initials: GD
authors.lastname: Webb
authors.forename: W G
authors.initials: WG
authors.lastname: Williams
authors.forename: P R
authors.initials: PR
authors.lastname: McLaughlin
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D015637:Gated Blood-Pool Imaging; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000503:physiopathology* / Q000601:surgery; D006801:Humans; D016014:Linear Models; D008297:Male; D011446:Prospective Studies; D013318:Stroke Volume / Q000502:physiology; D013997:Time Factors; D016896:Treatment Outcome; D018785:Tricuspid Atresia / Q000453:epidemiology / Q000503:physiopathology* / Q000601:surgery