Management of Patients with Heterotaxy Syndrome

Heterotaxy syndrome (HS) represents abnormal symmetry and therefore distribution of usually lateralized organs. It is considered as one of the most serious syndromic conditions associated with congenital heart disease (CHD), often of complex nature. It is found in approximately 1 in 10,000 of live births and accounts for 2–4% of all forms of CHD. HS is characterized by a wide variety of cardiac and extracardiac congenital malformations that are primarily induced by disorders of the left-right axis determination during early embryonic development.