title: [Factors limiting adaptation to exercise after the Fontan operation: role of altered pulmonary function].
abstract: BACKGROUND
  The abnormal adaptation to exercise after Fontan operation, is primarily due to a suboptimal increase in cardiac output. We postulated that abnormal pulmonary function could cooperate in reducing effort tolerance of these patients (pts).
  
  
  METHODS
  We have evaluated 10 pts, mean age 15 +/- 8 yrs, with a mean postoperative follow-up of 6 +/- 2 yrs. Seven pts were asymptomatic while 3 had mildly decreased effort tolerance. These pts were evaluated with echocardiogram, cycle ergometer stress test, basal and effort spirometry and perfusion lungs scanning.
  
  
  RESULTS
  Basal ejection fraction was normal in 9 pts and mildly depressed in 1 pt. Basal cardiac index was 1.8 + 2.2 l/min/m2 and increased to 4.8 + 5 l/min/m2 during exercise. Effort tolerance was 73% +/- 21% of predicted values, with maximal O2 consumption of 60% +/- 13% of predicted. Basal spirometry showed a mild restrictive pattern in 7 pts with a decrease in total pulmonary capacity and in forced vital capacity (mean values respectively 79% +/- 14% and 75% +/- 18% of predicted), otherwise respiratory reserve resulted normal in all pts. Carbon monoxide diffusion was impaired in all pts with a mean value of 49% +/- 10% of predicted. O2 saturation near normal in basal condition decrease during exercise (mean value 96% +/- 2% and 91% +/- 7%). The VE/VO2 and Vd/Vt ratio were both abnormally increased (respectively 111% +/- 28% and 186% +/- 39% of predicted). Lung scanning was abnormal in 6 pts with defects of perfusion localized prevalently in left lung (5/6) especially in upper lobe (4/6).
  
  
  CONCLUSIONS
  After Fontan operation exercise tolerance is reduced as consequence of 1) a suboptimal increase in cardiac output and 2) abnormal gas diffusion probably due to a damage of alveolo-capillary membrane and/or to a ventilation/perfusion mismatch as showed by abnormalities in lungs perfusion and by increase Vd/Vt ratio.
authors.affiliation: Departimento Cardiologico de Gasperis, Ospedale Niguarda CA Granda, Milano.
authors.forename: G
authors.initials: G
authors.lastname: Vignati
authors.forename: A
authors.initials: A
authors.lastname: Corato
authors.forename: C
authors.initials: C
authors.lastname: Sacerdoti
authors.forename: F
authors.initials: F
authors.lastname: Banfi
authors.forename: P
authors.initials: P
authors.lastname: Austoni
authors.forename: L
authors.initials: L
authors.lastname: Mauri
authors.forename: A
authors.initials: A
authors.lastname: Figini
mesh_terms: D000222:Adaptation, Physiological; D000293:Adolescent; D000328:Adult; D002302:Cardiac Output; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005069:Evaluation Studies as Topic; D005080:Exercise Test; D018729:Fontan Procedure / Q000009:adverse effects*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D008168:Lung / Q000502:physiology*; D013147:Spirometry
vernacular_title: Fattori limitanti l'adattamento allo sforzo dopo intervento di Fontan: ruolo di eventuali anomalie polmonari.