Surgical Correction of Scoliosis in Children with Severe Congenital Heart Disease and Palliated Single Ventricle Physiology

Study Design. Case series. Objective. In this study we focus on this group, and describe the largest cohort to date of single ventricle patients undergoing surgical correction of scoliosis at a single institution. Summary of Background Data. Outcomes of spinal surgery for patients with congenital heart disease (CHD) have evolved and most affected patients have uncomplicated procedures. The risk of perioperative complications remains highest in patients with single ventricle (“Fontan") physiology. Methods. We reviewed patients with single ventricle CHD and operative scoliosis repair over a 25-year span. Patients who had undergone a Fontan, bidirectional Glenn, and/or Kawashima procedure before operative scoliosis repair were included. Patients were excluded if they lacked preoperative cardiac care and/or sufficient medical history at our institution. Results. Twenty-three patients were included. The average age at surgery was 13.6 years (range, 5–23). Seventy percent of the cohort was female (16/23). The majority of patients underwent a Fontan before scoliosis surgery (20/23, 87%). Patients without intraoperative tranexamic acid (TXA) had a 67% complication rate (8/12) versus 36% (4/11) in those with intraoperative TXA ( P = 0.29). There was a significant difference in estimated blood loss by weight (cc/kg) between non-TXA and TXA patients ( P = 0.016). Twelve patients experienced complications (52%), all of which occurred postoperatively. There were no deaths, cerebrovascular events, adverse perioperative cardiac or hemodynamic complications, or wound infections. Using the Clavien-Dindo-Sink classification for postoperative complications, four patients had serious adverse events, including one permanent neurologic deficit. Conclusion. Spinal surgery for scoliosis has been performed in selected patients with single ventricle physiology at a single institution without mortality for 25 years. Operative blood loss may be reduced by routine use of TXA. Complications occur most commonly in the postoperative period, and can include pleural effusion. Level of Evidence: 4