Purpose: Intestinal rotation abnormalities and complex congenital heart disease associated with heterotaxia coexist. Despite the risk for midgut volvulus, performing a Ladd procedure for asymptomatic malrotation with heterotaxia remains to be controversial because the presumed risk for postoperative complications is thought to exceed the benefits of the operation. The purpose of this study was to review the incidence of complications after a Ladd procedure in asymptomatic patients with heterotaxia to guide recommendations for this patient population.
Methods: The medical records of all patients with heterotaxia who underwent a Ladd procedure for asymptomatic malrotation between 1984 and 2004 were reviewed. Type of cardiac disease, postoperative complications, and survival were recorded.
Results: Twenty-two patients (9 boys and 13 girls) with heterotaxia underwent an elective Ladd procedure after their medical stabilization or surgical correction or palliation of their cardiac anomaly. Of these patients, 19 were younger than 1 month at the time of the operation. The remaining 3 patients underwent the operation when they were between 2 and 5 months old. Three of the 22 patients (14%) developed postoperative intestinal obstruction: lysis of adhesions was performed in 1 patient; another patient required a staged bowel resection for a closed loop obstruction; and yet another patient had recurrent midgut volvulus 4 years after an incomplete initial Ladd procedure. All patients survived the initial and secondary procedures. Four deaths, all more than 1 month after the surgery, occurred as sequelae of the underlying cardiac anomaly. Length of follow-up ranged from 1 to 17 years.
Conclusions: We report on a 14% risk of postoperative bowel obstruction after an elective Ladd procedure, as compared with a small but significant incidence of midgut volvulus in patients with malrotation in the setting of complex congenital heart disease. Our results support the conclusion that an elective Ladd procedure at a time of relative cardiac stability for selected patients with heterotaxia has an acceptably low morbidity and should be considered to prevent midgut volvulus.