Objectives: 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.