Impact of age at fontan completion on functional status at mid-term follow up.

Introduction: Although the Fontan palliation is now being applied to younger patients, the influence of patients' age at the time of surgery on mid- and long-term results remains unclear. It has been our policy to perform Fontan operations when patients are around 5 years old in order to fit a larger conduit and minimize the risk of later obstruction. We sought to investigate whether age at Fontan completion affects subsequent clinical status in patients followed up for more than 3 years.

Methods: We carried out a cross-sectional study of Fontan patients who underwent total cavopulmonary connection between 1997 and 2009 and were followed up for 3 years with detailed chart review, ECG, echocardiogram, and functional status questionnaire.

Results: Of the total of 58 patients there were 56 long-term survivors. Of these, 41 had undergone surgery 3 years prior to the time of the study. We were able to contact 37 patients, who comprised our study cohort (mean follow up 6.3, range 3.0-11.0 years). Mean age at operation was 7.4 years (median 5.5, range 3.0-29.5 years). At latest follow up, 97% were in NYHA class I or II. In terms of somatic development, 21% (8/37) for weight and 19% (7/37) for height were below the 25th percentile for age-matched controls. Excluding one patient with developmental delay due to an underlying syndrome, there were 5 patients (13.5%) with mild learning disabilities and one with moderate delays. No additional surgeries had been performed. Tissue Doppler imaging measurements of systolic and diastolic velocities of the lateral annulus of the dominant ventricle revealed abnormalities of both systolic function and diastolic compliance, although qualitatively the systolic function of the single ventricle was considered adequate in all patients.

Conclusions: Although earlier Fontan palliation is considered beneficial in the long term from the viewpoint of exercise capacity and hemodynamics, even late Fontan completion provides acceptable mid-term results in terms of the patients' functional status.