[Surgical management of visceral heterotaxy syndrome]

Surgical management of the heterotaxy syndrome including asplenia or polysplenia is still challenging, because they have not only congenital heart defects but also gastrointestinal abnormalities. In most cases, they are Fontan candidates, however, indications and procedures of surgical strategy toward Fontan operation are quite difficult because of cardiac complications or abdominal manifestations. We mentioned indications, surgical technique, peri- and post-operative managements of asplenia syndrome with our experiences and results.