title: Univentricular heart and Fontan staging: analysis of factors impacting on body growth.
abstract: OBJECTIVE
  The optimal timing of the Fontan staging for a univentricular heart and its impact on growth remains debateable. In a Fontan cohort, the influence of staged interventions and patient factors on somatic development was explored.
  
  
  METHODS
  We reviewed 64 total cavopulmonary connection (TCPC) patients treated since 1992. Serial anthropometric parameters recorded from birth to the latest follow-up (mean 12.5 ± 6.1 years) and at each intervention [neonatal surgery, bidirectional cavopulmonary anastomosis (BCPA), TCPC, catheter treatment] were converted to z-scores. The influence of saturation, heart failure treatment and surgery intervals on growth was determined.
  
  
  RESULTS
  The mean z-scores for weight and height changed significantly at each surgery up to the TCPC (-0.3 ± 1.2 and 0 ± 1 at birth, -1.3 ± 1.9 and -0.9 ± 1.7 at neonatal surgery, -2.1 ± 1.2 and -1.6 ± 1.3 at the BCPA, -1.2 ± 1.3 and -0.7 ± 1.4 at the TCPC for weight and height, respectively; P < 0.05 for each interval), with the largest decline before the BCPA, and the most marked improvement before the TCPC. Z-scores did not change significantly after the TCPC. Younger age at the BCPA had a positive influence on the weight z-score at the TCPC (P < 0.05); somatic growth at the latest follow-up (FU) was negatively influenced by heart failure treatment (P < 0.05).
  
  
  CONCLUSIONS
  Body growth is severely impaired in Fontan patients. A close interstage follow-up between the first surgery and the BCPA must be targeted at optimizing nutritional support to counter the important growth retardation occurring before the BCPA. The better catch-up growth at the TCPC when the BCPA is performed earlier in life supports the current trend to perform the BCPA at a younger age. Heart failure treatment after a Fontan completion is independently associated with decreased late somatic development.
authors.affiliation: Department of Cardiac Surgery, Cardiac Centre, University Hospital Ghent, Ghent, Belgium. katrien.francois@ugent.be
authors.forename: Katrien
authors.initials: K
authors.lastname: François
authors.forename: Thierry
authors.initials: T
authors.lastname: Bové
authors.forename: Joseph
authors.initials: J
authors.lastname: Panzer
authors.forename: Katya
authors.initials: K
authors.lastname: De Groote
authors.forename: Kristof
authors.initials: K
authors.lastname: Vandekerckhove
authors.forename: Hans
authors.initials: H
authors.lastname: De Wilde
authors.forename: Daniel
authors.initials: D
authors.lastname: De Wolf
mesh_terms: D000367:Age Factors; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000494:pharmacology; D000886:Anthropometry / Q000379:methods; D001827:Body Height / Q000187:drug effects / Q000502:physiology; D001835:Body Weight / Q000187:drug effects / Q000502:physiology; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006130:Growth Disorders / Q000209:etiology* / Q000503:physiopathology; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors