“Heterotaxy is synonymous with `visceral heterotaxy' and `heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as `situs solitus', nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus'.” The purpose of this manuscript is to review the data about heterotaxy in the STS Congenital Heart Surgery Database.
We examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 - December 31, 2009, inclusive. This analysis resulted in a cohort of 77,153 total index operations. Of these 77,153 index operations, 1,505 (1.95%) were performed in patients with heterotaxy. Of the 1,505 index operations performed in patients with heterotaxy, 1,144 were in patients with “asplenia” and 361 were in patients with” polysplenia”.
In every STS–EACTS Congenital Heart Surgery Mortality Category (2009) (STS–EACTS Category), discharge mortality is higher in patients with heterotaxy compared to patients without heterotaxy. Discharge mortality after Systemic to pulmonary artery shunt is 6.6% in a cohort of all Single Ventricle patients except those with heterotaxy, while it is 10.8% in Single Ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all Single Ventricle patients except those with heterotaxy, while it is 4.2% in Single Ventricle patients with heterotaxy.
The STS Database is largest Congenital Heart Surgery Database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.