Clinical implication of hiatal hernia in patients with right isomerism.

OBJECTIVE

Despite a reported association between hiatal hernia in patients with heterotaxy syndrome, the clinical significance has remained unclear. Based on large patient cohorts, this study specifically aimed at defining the implication of hiatal hernia in patients with right isomerism.

METHODS

From 1994 to 2002, 143 patients were identified as having right isomerism. Among them, 119 received ultrafast computed tomography (CT) to determine the presence of hiatal hernia as well as any cardiovascular anomalies.

RESULTS

Hiatal hernia was found in 17 patients (seven females and 10 males, 14.3%). The upper gastrointestinal (GI) series in six patients confirmed the diagnosis of hiatal hernia in all and revealed severe gastro-oesophageal reflux in four. The most common symptom of hiatal hernia was vomiting (47%), followed by recurrent bronchiolitis or pneumonia (41.2%) and upper gastrointestinal bleeding (11.8%). Three patients with hiatal hernia underwent fundoplication for medically refractory vomiting. Seven patients were found to have midgut malrotation (5.9%), and four of these had both hiatal hernia and malrotation. Pulmonary atresia was closely associated with the presence of hiatal hernia (p=0.02). One patient with hiatal hernia died suddenly at 6 mo. However, overall mortality was similar between those patients with hiatal hernia and those without.

CONCLUSIONS

The incidence of hiatal hernia was considerably high in patients with right isomerism, especially in those with pulmonary atresia, and it is this that may have led to vomiting and recurrent airway infections. While an association between sudden death and hiatal hernia may well exist, this does require further clarification.