Operative treatment of scoliosis in patients with a Fontan circulation

Study Design. A retrospective chart and radiographic review of patients with a Fontan physiology who underwent surgical correction of their scoliosis. Objective. To evaluate the operative and perioperative treatment of patients with a Fontan physiology and scoliosis. Summary of Background Data. The survival rates in patients with complex congenital heart disease are improving, thus creating a subset of patients with complex physiology who will require surgical treatment of their scoliosis. The Fontan physiology is created surgically in patients born with a single functioning ventricle. These patients represent a significant anesthetic risk during spinal surgery, given their physiologic need for a sustained systemic preload. Currently, to our knowledge, there are no published series in the literature regarding the operative treatment of scoliosis in these patients. Methods. We reviewed our experience with the operative treatment of scoliosis in 7 patients with a Fontan circulation. There were 7 patients identified who were treated with correction and arthrodesis using modern segmental instrumentation. A radiographic and chart review was performed to assess the operative and perioperative treatment. Results. Mean age at surgery was 14 years 9 months, and mean Cobb angle was 75 degrees. There were no intraoperative complications; however, postoperative complications were frequent and significant: 1 delayed paralysis, 2 superior mesenteric artery syndromes, 1 renal tubular necrosis, 2 pulmonary complications, 1 Horner syndrome, and 1 urinary tract infection. Conclusions. Operative treatment of scoliosis in these patients may be successful; however, complications are frequent and significant.