title: Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial.
abstract: OBJECTIVE
  Heterotaxy syndrome is associated with intestinal abnormalities. We sought to define the gastrointestinal anatomy and determine both the risk of volvulus and benefit of screening upper gastrointestinal fluoroscopy (UGI) in these patients.
  
  
  METHODS
  Medical records from 2003 until 2011 at Children's Hospital Los Angeles were reviewed in patients with heterotaxy for cardiovascular diagnosis, gastrointestinal symptoms, imaging and surgical arrangement of viscera, perioperative morbidities, and overall mortality.
  
  
  RESULTS
  224 patients were identified. Fifteen had polysplenia, 41 had asplenia, 50 had normal splenic morphology, 13 had inversus, and 104 were uncharacterized. UGI was performed in 4 patients for suspected volvulus and 20 for obstructive symptoms. Sixty-two had "screening" UGIs. Of 138 asymptomatic patients without imaging, none developed volvulus during the study period. In 30 patients with duodenojejunal malposition (DJM) who underwent surgery, none had malrotation or narrow mesentery. Eleven developed complications, with 8 requiring reoperation for obstruction. Of 8 patients with malrotation, 7 received a Ladd's procedure, and 2 had volvulus with viable bowel. One patient required reoperation and resection for obstruction.
  
  
  CONCLUSIONS
  While rotational abnormalities are common in heterotaxy, risk of volvulus is low. Following operation, the risk of bowel obstruction and of need for reoperation is higher. We advocate avoiding operation in the asymptomatic patient.
authors: Department of Pediatric Surgery, Children's Hospital Los Angeles, CA 90027, USA.
authors: Stephanie
authors: S
authors: Papillon
authors: Catherine J
authors: CJ
authors: Goodhue
authors: Osnat
authors: O
authors: Zmora
authors: Shalini S
authors: SS
authors: Sharma
authors: Winfield J
authors: WJ
authors: Wells
authors: Henri R
authors: HR
authors: Ford
authors: Jeffrey S
authors: JS
authors: Upperman
authors: Kasper S
authors: KS
authors: Wang
authors: Gerald A
authors: GA
authors: Bushman
authors: Richard
authors: R
authors: Kim
authors: James R
authors: JR
authors: Pierce
mesh_terms: D058070:Asymptomatic Diseases; D005260:Female; D005471:Fluoroscopy; D005500:Follow-Up Studies; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000209:etiology / Q000601:surgery; D045822:Intestinal Volvulus / Q000000981:diagnostic imaging* / Q000209:etiology / Q000517:prevention & control / Q000601:surgery; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D011183:Postoperative Complications / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D016896:Treatment Outcome