Midaortic Syndrome And Renovascular Hypertension Center

Midaortic syndrome is a very rare condition in which the middle portion of the aorta (the heart’s largest blood vessel) that runs through the chest and abdomen is narrowed. This can lead to insufficient blood flow in the chest, abdomen and lower limbs. Midaortic syndrome is also sometimes called narrowing or coarctation of the abdominal aorta. A related condition called renovascular hypertension is high blood pressure that results from decreased blood flow to the kidneys. Severe hypertension is often the first sign of midaortic syndrome.

These very serious conditions require urgent and thoughtful attention and care. You may find yourself faced with the need to make critical decisions about your child’s health quickly. Yet because midaortic syndrome and renovascular hypertension are rare, few clinicians have the experience and skill to treat them optimally.

Our approach to midaortic syndrome

The Midaortic Syndrome and Renovascular Hypertension Program at Boston Children’s Hospital is the largest and most experienced program dedicated to diagnosing and treating children with these conditions.

Because these conditions involve the whole child, not just the heart and kidneys, we take a team approach to care for all of our patients. When you visit our center, you and your child will meet with a group of skilled clinicians from a range of specialties, including nephrology, vascular surgery, cardiology, and interventional radiology. The Midaortic Syndrome and Renovascular Hypertension Center is one of just a few centers to provide this type of multidisciplinary care to children with midaortic syndrome. Our team of specialists will meet to discuss your child’s case and then formulate a treatment plan together that takes into account all aspects of his or her care. Research suggests that patients with complex conditions who receive their care from a multidisciplinary team tend to have better outcomes than those treated by a single clinician.

About half of all children with midaortic syndrome may also have other associated conditions, including:

  • Alagille syndrome, a genetic disorder leading to reduced bile flow because of too few bile ducts inside the liver
  • Moyamoya disease, a rare condition in which the walls of the internal carotid arteries become thickened and narrowed
  • Neurofibromatosis, a genetic disorder that can cause a variety of medical issues, including vascular anomalies of the abdomen and brain
  • Williams syndrome, a rare, inherited disorder that can cause both neurodevelopmental and cardiovascular complications

If your child also has one or more of these associated conditions, they will have access to clinicians at Boston Children’s who specialize in them as well.

At the forefront of innovation for midaortic syndrome

The clinicians in the Midaortic Syndrome and Renovascular Hypertension Center are leaders in the development of new treatments for these conditions. For instance, Boston Children’s is the birthplace of two groundbreaking treatment approaches. The first, called Mesenteric Artery Growth to Improve Circulation (MAGIC), is a type of surgery that bypasses the aorta using the mesenteric arteries (the arteries that supply blood to the intestines) rather than prosthetic graft material.

Another innovation, known as Tissue Expander Stimulated Lengthening of the Arteries, or TESLA, is a pioneering procedure that involves the placement of a tissue-expanding device behind the child’s aorta to stretch it gradually over time. Although TESLA is still a very new approach, it can help some children with midaortic syndrome, renovascular hypertension or both avoid multiple surgeries as they grow up.

Our physicians have also refined the use of certain endovascular techniques for the treatment of midaortic syndrome. These minimally invasive procedures, such as angioplasty and stenting, can help open narrowed arteries and offer faster recovery times. Along with skilled medical management, such approaches may be used instead of surgery in some patients, or prior to surgery in order to help the child be in a better state of health.