Background The association of heterotaxia with intestinal rotation anomalies is well documented. Controversy still persists though in regards to the optimal diagnostic strategy, as well as to the benefit of surgical correction of identified rotational abnormalities in patients with no gastrointestinal symptoms, especially given the frequent associated cardiac anomalies. The aim of this study was to test the validity of the watchful wait approach in a series of patients with heterotaxia.

Method A retrospective analysis of the case notes and electronic records of patients diagnosed with heterotaxia syndromes was performed at a single tertiary paediatric referral centre that offers paediatric cardiac surgery services. All patients with situs anomalies, born within the 1st January 1998 to 31st December 2013, were included.

Results 59 patients were identified, of which 13 underwent contrast studies to identify rotational anomalies. Only 2 studies were performed in symptomatic patients (vomiting, failure to thrive). 10 of the studies identified abnormal anatomy, but only 4 patients proceeded to surgical correction. No complications were reported following the Ladd’s procedures. There was a single death within 30 days postoperatively, attributed to cardiac failure. Regarding the patients that had no surgery, during the follow-up period (median 9.2 years, range 11- 187 months), no patients presented clinically with malrotation or volvulus.

Conclusions This study supports the ‘watch and wait’ approach for children with heterotaxia, provided that close surveillance can be achieved. Diagnosis and treatment should be individualised and tailored to each patient’s clinical characteristics in order to avoid unnecessary morbidity and mortality.