Heterotaxy Syndrome

What is heterotaxy syndrome?

Heterotaxy syndrome is a rare condition where the heart or other organs are not formed correctly or are in the wrong position within the body.

There are several types of heterotaxy syndrome. All involve some type of heart defect and may also involve defects in the intestines, spleen, lungs, liver and other organs. These organs may not be in the correct place inside the body.

Specific problems can include:

  • holes in the walls that divide the right and left sides of the heart (septal defects)
  • problems involving the valve and blood vessels in the heart
  • structural problems in the lungs
  • having no spleen (asplenia) or many small spleens (polysplenia) that may not work properly
  • intestinal malrotation, an abnormal twisting of the intestines
  • a liver that is in the wrong location

Care for heterotaxy syndrome

Our team at the Boston Children’s Hospital Heart Center treats some of the most complex and challenging pediatric heart conditions in the world, including heterotaxy syndrome. At Boston Children’s, we frequently can achieve “two ventricle” repairs for many of these patients that were previously treated as though they only had one ventricle.

Each patient requires specialized treatment because of the many types of heart defects found in children with heterotaxy syndrome.

Our areas of innovation for heterotaxy syndrome

Boston Children’s has a long-standing commitment to — and experience with — heterotaxy syndrome. With so many team members focused specifically on children with hetereotaxy we have developed real solutions for children suffering from these challenging heart defects.