Univentricular heart and Fontan staging: analysis of factors impacting on body growth

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. 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. 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). 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.