Heterotaxy Program

The Heterotaxy Program at Boston Children’s Hospital provides comprehensive care to infants and children with heterotaxy. Our team of expert clinicians provides a full range of care for our patients, from diagnosis and treatment, through a lifetime of follow up.

Why choose the Heterotaxy Program?

Heterotaxy is a complex syndrome that affects not only the heart, but also many organ systems throughout the body, including the lungs, immune system, and intestines. We understand that children with heterotaxy need a whole-body approach to their condition. That’s why our team includes not only cardiologists and cardiac surgeons, but also clinicians from across multiple specialties.

We are the first and only program in the country who work as a team to care for patients with heterotaxy syndrome. Our goal is to provide a proactive approach to each patient’s care, providing regular screenings and check-ups, rather than waiting for potential problems to arise.

Your child’s heterotaxy care team may include:

Cardiologists: The cardiologists on our team identify and treat abnormalities of the heart associated with heterotaxy syndrome — from mild problems to the most complex. They use advanced imaging techniques, including echocardiography, cardiac magnetic resonance imaging (MRI), cardiac computed tomography (CT), three-dimensional modeling, and cardiac catheterization to gain a complete understanding of your child’s heart. Our cardiologists collaborate closely with our cardiac surgeons to ensure the best possible heart treatment for your child.

Cardiac surgeons: Our cardiac surgeons have extensive experience performing heart surgery for infants, children, and adults who have congenital heart disease associated with heterotaxy. Our surgeons use a wide range of techniques, including biventricular repair, for many forms of complex heterotaxy. Our surgeons also have expertise in single ventricle surgeries, including those for patients who have unusual or complicated forms of heterotaxy.

Electrophysiologists: Many children with heterotaxy have abnormal heart rhythms, such as bradycardia or complete heart block, where the heart beats too slowly, or tachycardia, where the heart beats too quickly. Our electrophysiologists are experts in diagnosing and treating heart rhythm problems and will provide a comprehensive assessment for your child.

Pulmonologists: Some patients with heterotaxy have a lung condition called primary ciliary dyskinesia (PCD) that can increase the risk for infection. While not all patients with heterotaxy have PCD or pulmonary disease, our pediatric pulmonologists can help determine when testing is necessary. These screenings may include genetic testing, nasal nitric oxide measurements, and looking at cilia structure with electron microscopy.

Otolaryngologists (ear, nose, and throat specialists): Our otolaryngologist works closely with our pulmonologists to help identify PCD and to treat other ear, nose, and throat problems that may occur in heterotaxy.

Gastroenterologists, hepatologists, and general surgeons: Some children with heterotaxy also have problems with their internal organs. These can include malrotation, a problem in the way the stomach and intestines form, and biliary atresia, a problem with the way the gallbladder is formed. Our gastroenterologists and hepatologists will screen and treat children for these issues as needed. If your child needs surgery, they will work with our general surgeon to coordinate care.

Immunologists: Children with heterotaxy are at higher risk for having an absent or non-functioning spleen. This can put them at risk for severe infections. Our immunologist will assess the function of your child’s spleen and may recommend antibiotics or additional vaccinations to provide extra immune protection.

Geneticists: A geneticist can help identify if there is a genetic cause of heterotaxy in your child. This information may help you learn more about how heterotaxy will affect your child. It can also be useful for future family planning.

Radiologists: Our radiologists use various imaging techniques, such as x-rays, computed tomography, ultrasound, and nuclear medicine, to find problems in the body related to heterotaxy. They work with other team members to develop a treatment plan for your child.

Anesthesiologists: Our cardiac anesthesiology team provides care to children undergoing cardiac imaging, as well as cardiac catheterization and cardiac surgery. Our team is very familiar with heterotaxy and the multiple challenges it can present in terms of pulmonary function, variable vascular anatomy, and cardiac disease. Our anesthesiologists work closely with your child’s cardiologist and surgeon to make sure your child receives safe and excellent care.

Our approach to heterotaxy

The team at Boston Children’s has extensive experience in the medical and surgical care of the most complex and challenging conditions associated with heterotaxy.

Because heterotaxy affects each child in an individual way, our program reflects that need for individualized care. We understand that each child with heterotaxy has a unique set of health issues, and each of the specialists on our team has an in-depth knowledge of the condition and how it affects a child’s overall health.

Your child will be examined by each of the relevant specialists on our team, depending on your child’s individual condition. Once all of the assessments are complete, our team members will meet to create a treatment plan to address all the health needs of your child.

Our areas of research and innovation for heterotaxy

With so many team members focused specifically on children with heterotaxy, we have developed real solutions for children born with these challenging heart defects.

Some areas of innovation in heterotaxy include:

  • biventricular repairs for some children with heterotaxy, many of whom may not be candidates for this option at other heart centers
  • advanced cardiac imaging, such as 3-D echocardiography, cardiac magnetic resonance imaging (MRI), cardiac computed tomography (CT), and cardiac catheterization
  • 4-D flow modeling, a technique that allows our cardiologists and surgeons to create computer models of different surgical approaches to decide on the best procedure for your child
  • standardized screenings for all children with heterotaxy
  • a heterotaxy registry to track patients and outcomes and identify and spread best practices and research within Boston Children’s, as well as outside our institution