Malrotation is not associated with adverse outcomes after cardiac surgery in patients with heterotaxy syndrome.

BACKGROUND: Patients with heterotaxy syndrome (HS) often have asymptomatic malrotation. There is a lack of consensus regarding the management of these patients, particularly in patients with complex congenital heart disease (CHD). We sought to describe the prevalence of malrotation and incidence of volvulus in a population of patients with complex CHD and to identify the impact of malrotation on morbidity and mortality following cardiac surgery.

METHODS: We performed a retrospective review of all patients with HS and complex CHD who required cardiac surgery in the first year of life at a single center between October 1995 and September 2015. Malrotation was diagnosed by abdominal imaging or by direct inspection during abdominal surgery. Demographic data was collected along with details of hospitalization following cardiac and GI surgeries. Descriptive analysis along with appropriate hypothesis testing was conducted to evaluate the results.

RESULTS: We identified 49 patients with HS, 42 with single ventricle anatomy and 7 with biventricular anatomy. Of the 49 patients, 29 (59%) were diagnosed with malrotation, 6 (12%) had normal intestinal rotation, and 14 (29%) had no evaluation of intestinal rotation. The prevalence of malrotation in the population who underwent abdominal imaging was 29 out of 35 (83%). There was no difference in survival following cardiac surgery between patients with malrotation and those with unknown or normal intestinal anatomy. Comparing patients with malrotation and patients with normal or unknown intestinal rotation, there was also no difference in surrogate markers of morbidity. Of the 29 patients with known malrotation, only 2 patients (7%) underwent therapeutic Ladd procedures and 19 (65%) underwent prophylactic Ladd procedures.

CONCLUSIONS: We conclude that the outcomes following cardiac surgery for patients with HS are not impacted by the presence of malrotation. Furthermore, we also found that the incidence of volvulus in the studied group is low. Given these findings, and the understanding that patients with HS and significant CHD are frequently tenuous and high risk surgical candidates, we do not believe performing prophylactic Ladd procedures is warranted.

LEVEL OF EVIDENCE: III.