The strong association between congenital heart disease and spinal deformit
y is well established, but data on the risks and outcome of spinal fusion s
urgery in patients with congenital heart disease are scarce. The purpose of
this study was to identify predictors of perioperative risk and outcome in
a large series of children and adolescents with congenital heart disease w
ho underwent spinal fusion for scoliosis or kyphosis. In the authors' retro
spective analysis of 74 consecutive patients with congenital heart disease
undergoing spinal fusion, there were two deaths (2.7%) and 18 significant c
omplications (24.3%) in the perioperative period, Preoperative cyanosis (ar
terial oxygen saturation <90% at rest) with uncorrected or incompletely cor
rected congenital heart disease was associated with both deaths. Complicati
ons occurred in nine of 18 (50%) patients with cyanosis and in 11 of 56 (20
%) patients without cyanosis, As judged by multivariate analysis the best p
redictors of perioperative outcome were the overall physical status of the
patient as represented by the American Society of Anesthesiologists' preope
rative score and a higher rate of intraoperative blood loss. Seventeen of 4
3 patients (40%) with an American Society of Anesthesiologists score of 3 o
r higher experienced complications including two perioperative deaths, Succ
essful spinal fusion and correction were achieved in 97% of patients. Child
ren and adolescents with congenital heart disease can undergo elective spin
al fusion with risks that relate to overall cardiac status. Careful assessm
ent of preoperative status by pediatric cardiologists and cardiac anesthesi
ologists familiar with surgical treatment of patients with congenital heart
disease will assist the orthopaedic surgeon in providing the most realisti
c estimate of risk.