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Somatic Growth after Fontan and Mustard Palliation


Babar Sultan Hasan, MD, Department of Pediatric Cardiology, Children's Hospital, Boston, MA, USA. Tel: (+1) 617-560-3164; Fax: (+1) 617-739-6282; E-mail:


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.