Congenital heart disease and heterotaxy: Upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial

Purpose: 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. Conclusion: 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.