title: Evaluating a management strategy for malrotation in heterotaxy patients.
abstract: BACKGROUND
  There is disagreement over the management of malrotation in children with heterotaxy and congenital heart disease (CHD). We sought to evaluate the outcomes of management with a Ladd procedure compared to observation in this cohort of patients.
  
  
  METHODS
  We performed a retrospective review of CHD children with heterotaxy and malrotation identified on radiographs treated from 8/2002 until 4/2014. Primary outcomes evaluated were readmission for small bowel obstruction (SBO) or volvulus.
  
  
  RESULTS
  We identified 88 patients with cardiac heterotaxy and malrotation. Sixty-eight (77%) had a Ladd procedure. Eighteen (26%) of the 68 had abdominal symptoms, but only one had an underlying volvulus without ischemia. Twenty (23%) patients died of cardiopulmonary complications, 8 before and 12 after the Ladd procedure. Sixty-eight patients survived to the review date (median: 5years): 56 in the Ladd cohort and 12 observed. Six of the 56 (11%) surviving Ladd patients were readmitted to hospital with an SBO, and 2 required surgical intervention. None of the 12 surviving nonoperative patients developed a volvulus.
  
  
  CONCLUSIONS
  Eleven percent of patients developed SBO after their Ladd procedure. Conversely, no observed patients developed a volvulus. This suggests that complications from a Ladd procedure occur with higher frequency than complications from observing heterotaxy patients with malrotation.
authors.affiliation: Division of Pediatric Surgery, Houston, TX, United States; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, United States.
authors.forename: Paulette I
authors.initials: PI
authors.lastname: Abbas
authors.affiliation: Division of Cardiology, Texas Children's Hospital, Houston, TX, United States.
authors.forename: Heather A
authors.initials: HA
authors.lastname: Dickerson
authors.affiliation: Division of Pediatric Surgery, Houston, TX, United States; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, United States. Electronic address: dewesson@texaschildrens.org.
authors.forename: David E
authors.initials: DE
authors.lastname: Wesson
mesh_terms: D013505:Digestive System Surgical Procedures; D005260:Female; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000209:etiology; D045822:Intestinal Volvulus / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D007421:Intestine, Small; D008297:Male; D011859:Radiography; D012189:Retrospective Studies
keywords: Cardiac heterotaxy; Ladd procedure; Malrotation; Small bowel obstruction; Volvulus