• C Pérez-Caballero Macarrón, E Sobrino Ruiz, J Burgos Flores, Jl Vázquez Martínez, A Coca Pérez, E Alvarez Rojas, and Jj Sánchez Ruas.
• 1 Paediatric Intensive Care Unit, Hospital Ramón y Cajal, Madrid, Spain.
• Cardiol Young. 2014 Feb 1; 24 (1): 73-8.

IntroductionThe management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis.Materials And MethodsThis was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed.ResultsWe identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12-21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 μg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8-44.6). Surgical drainages were maintained for a mean time of 3 days (range 1-7). The mean hospital stay was 9.2 days (range 6-19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound.ConclusionsSpinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.

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