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Congenital Heart Disease

The association between scoliosis and congenital heart disease is well established.
Owing to advances in heart surgery,
children are now living longer than in the past, and many with severe scoliosis are candidates for operative correction.
The incidence of scoliosis associated with congenital heart disease is approximately 4%.
For those with congenital
heart disease who have undergone cardiac surgery, the incidence of scoliosis is higher (11%).
This observation has
led some authors to conclude that there may be an association between thoracotomies for congenital heart disease and
the development of scoliosis.
Others refute this conclusion.
A recent study reported that 12.5% of 128 mature
patients who underwent median sternotomy for the treatment of congenital heart disease before age 8 years later
developed scoliosis exceeding 20 degrees.
Patients operated on before age 18 months had a significantly increased
risk of developing scoliosis compared with those operated on later. The presence of scoliosis was not related to the type of
congenital heart disease. Although surgical intervention for heart disease in children may be associated with the
development of scoliosis, it appears that these two events have a multifactorial relationship.
Two types of scoliosis are seen in conjunction with congenital heart disease: congenital scoliosis and developmental
scoliosis. For congenital scoliosis, the curve patterns and natural history appear to be unaffected by the coexisting heart
disease. Curve progression requires limited spinal fusion performed according to the standard guidelines described
previously in the section on congenital scoliosis.
Children with developmental curves present at an average age of 11 years.
Left and right convexities occur with
equal frequency. Usually, however, convex left thoracic curves are found in the upper thoracic spine, and convex right
curves are seen in the lower thoracic region.
There is no significant relationship among the age of the child at the time
of cardiac surgery, the age at the onset of scoliosis, and the severity of the scoliosis (Fig. 12-99).

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Those with mild developmental curves (<30 degrees) require only observation. In children with developmental curves
exceeding 30 degrees, the curves may progress as much as 9 degrees per year, tend not to respond to bracing, and are
likely to require posterior spinal fusion. Before spinal surgery is initiated, repair of cardiac anomalies or temporary cardiac
shunting procedures should be completed. Intraoperative management by experienced cardiopulmonary anesthesiologists
and postoperative intensive care are requisite for orthopaedic surgical intervention.

*See references
[48, 55, 210, 329, 344, 438, 532, 566, 594, 622, 656]

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FIGURE 12-99A and B, Clinical appearance of a girl aged 9 years 6 months who was born with double-outlet right ventricle mitral atresia.
She had previously undergone pulmonary artery banding, a Blalock-Taussig shunt, and bilateral Glenn shunts with takedown of the Blalock
shunt. C, The initial radiograph showed a 70-degree thoracolumbar curve accompanied by a small thoracic prominence. D, The patient
underwent anterior instrumentation and fusion between T11 and L3. Postoperatively, the curve measured 40 degrees. E, Four years later, the
scoliosis had changed. The patient now had a 67-degree thoracic curve and a 62-degree lumbar curve, and she was scheduled for further
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