Somatic Growth after Fontan and Mustard Palliation

OBJECTIVE: Patients with complex congenital heart disease frequently develop early growth failure; however, the long-term outcome for growth after surgery for single ventricle or anatomic right ventricle as systemic ventricle is not clear. This study was designed to determine long-term growth in patients following the Fontan and Mustard operations. METHOD: We retrospectively reviewed the growth parameters of children who had previously undergone the Fontan (n = 80) or Mustard (n = 66) palliation at the Riley Hospital for Children, Indiana. RESULTS: Both the Fontan and Mustard groups had normal height and weight at birth. At the time of their Fontan or Mustard palliation, there was a significant retardation in weight (Z-score: -0.98 and -1.79, respectively) and height (Z-score: -0.96 and -1.03, respectively). Both cohorts postoperatively demonstrated significant catch-up in their weights. Although the Mustard group normalized their heights, the Fontan patients continued to demonstrate short statures in long-term follow-up. CONCLUSION: Children with single ventricles and those with palliated d-loop transposition of the great arteries suffer somatic growth delay prior to definitive surgery, despite being of normal size at birth. Catch-up growth in weight occurs after the Fontan and Mustard operations. In the Mustard population, height also normalizes, whereas in patients with univentricular circulation, height remains abnormally low.