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Scoliosis Surgery in Children With Congenital Heart Disease

Kadhim, Muayad MD*; Spurrier, Ellen MD; Thacker, Deepika MD; Pizarro, Christian MD§; Mackenzie, William G. MD*

doi: 10.1097/BRS.0000000000000083

Study Design. Retrospective cohort study.

Objective. To describe preoperative evaluation, anesthetic and perioperative management, and complications in patients with congenital heart disease (CHD) who underwent surgery to correct a spine deformity.

Summary of Background Data. Patients with surgically palliated or repaired CHD may have nearly normal circulation or may have important residual abnormalities that affect the planning and conduct of surgery to correct a spine deformity.

Methods. We examined the records of 21 patients with spine deformity who had previous surgical intervention for CHD. Three types of spine surgery and instrumentation were examined, posterior spinal fusion with instrumentation (PSFI), growing rod (GR) instrumentation, and vertical expandable prosthetic titanium rib instrumentation (VEPTR). To objectify the degree of preoperative cardiac physiological derangement, patients were classified into 3 groups: single ventricle physiology and Fontan circulation (S), two ventricles with no residual abnormal cardiac physiology condition (2N), and two ventricles with residual cardiac physiology problem (2R).

Results. Subjects were 8 boys and 13 girls with mean age of 11.1 ± 5.2 years. Sixteen patients underwent surgery to correct scoliosis, 1 to correct kyphosis, and 4 did not undergo surgery. Total number of surgical procedures was 23 (16 PSFI, 5 GR, and 2 VEPTR). On the basis of cardiac physiology, 2 patients belonged 2N, 11 were 2R, and 8 were group S. Mean estimated blood loss was 1685 mL during PSFI, 515 mL during GR, and 150 mL during VEPTR. Mean volume of blood transfusion was 44 mL/kg for PSFI, 19 mL/kg for GR, whereas no transfusion was administered during VEPTR. Median intensive care unit stay was 2 days ranging from hours to 78 days. Median hospital length of stay was 7 days ranging from 3 to 93 days. There were no deaths.

Conclusion. Given meticulous multidisciplinary planning and execution, major spine surgery can be safely and successfully performed in patients with significant residua of CHD.

Level of Evidence: 4

Each patient with congenital heart disease needs to be thoroughly evaluated as a candidate for spine surgery when needed, and generalizations about risk and perioperative management cannot be made. With precise planning, major spine surgery can be safely and successfully performed in patients with significant residua of congenital cardiac disease.

Departments of *Orthopaedic Surgery


Cardiology, and

§Cardiac Surgery, Alfred I. duPont Hospital for Children, Wilmington, DE.

Address correspondence and reprint requests to William G. Mackenzie, MD, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE; E-mail:

Acknowledgment date: February 8, 2013. Revision date: September 11, 2013. Acceptance date: October 14, 2013.

Pedicle screws are FDA approved for adolescent idiopathic scoliosis in adults and adolescents. Growing rods are commonly used for the indication of correcting spine deformity in early onset scoliosis specifically for thoracic curves to improve the respiratory function. The patients evaluated in this manuscript had syndromic spinal deformity. Pedicle screws and growing rods are not FDA approved for this particular indication.

No funds were received in support of this work.

Relevant financial activities outside the submitted work: board membership, travel/accommodations/meeting expenses.

© 2014 by Lippincott Williams & Wilkins