Midaortic Syndrome And Renovascular Hypertension Program | Frequently Asked Questions

LIke ThisLIke ThisLIke ThisLIke ThisLIke This

Contact the Midaortic Syndrome and Renovascular Hypertension Program

  • 617-355-8544
  • International: +1-617-355-5209

Boston Children's Hospital's Midaortic Syndrome and Renovascular Hypertension (MAS/RVH) Program diagnoses, treats and provides long-term care for pediatric patients with either or both of these rare disorders.

Why is it so important to see a pediatric specialist for MAS and/or RVH? 

Not only are MAS and RVH rare and potentially life-threatening conditions; they can also affect different children in very different ways. It's essential to seek care from a team of pediatric specialists who are highly experienced in diagnosing and treating these conditions and all related complications.

How are MAS and RVH usually diagnosed?

Most children with midaortic syndrome and/or renovascular hypertension are diagnosed after being examined for signs of severe high blood pressure (hypertension).

The exact symptoms of MAS/RVH will depend on the individual child, but can include:

  • headaches
  • seizures
  • vision changes
  • abdominal pain that begins after a meal
  • discomfort or weakness in the legs during exercise
  • poor growth

Some of the tests our doctors may use to confirm that a child has MAS and/or RVH are:

  • angiography—injection of a special dye into blood vessels, then using X-ray imaging to look for narrowing 

  • ultrasonography—a painless, non-invasive procedure that uses special sound waves to create pictures of a child's organs

  • Computed Tomography (CT)

  • Magnetic Resonance Imaging (MRI)

What are the standard treatment options for MAS and RVH?

The treatment approach for each child depends very much on:

  • whether she has MAS, RVH or both
  • which blood vessels are affected (and how extensively)
  • her particular symptoms
  • her age and overall health 

 Treatment options typically include one or more of the following:

  • medication to control blood pressure and improve heart and/or kidney function

  • angioplasty—the use of a narrow tube called a catheter to insert and inflate a balloon inside the narrowed artery, widening it and improving blood flow to the affected organs

  • stenting—the placement of a special tube called a stent in a narrowed artery to hold it open 

In the most serious cases, doctors may recommend:

  • bypass graft surgery—an operation that makes detours, or "bypasses," around narrowed arteries, creating new paths for blood to flow

  • autotransplantation—moving a kidney to a new location where the blood flow can be improved

  • kidney transplantation (in cases where both kidneys are damaged to the point of causing irreversible kidney failure)   

Boston Children's groundbreaking new treatment

In a very exciting development, Boston Children's Hospital has pioneered a novel way of "stretching" the healthy vessels in the aorta to make them long enough to adequately replace the narrowed vessels. This procedure is called Tissue Expander Stimulated Lengthening of Arteries (TESLA)

 

Glossary

  • Anesthesiologist: a trained medical doctor who specializes in managing pain and administering anesthesia during medical and surgical procedures
     
  • Angiography: injecting a special dye into blood vessels, then using X-ray imaging to look for signs of narrowing 
     
  • Angioplasty: the use of a narrow tube called a catheter to inflate a balloon inside a narrowed artery, widening it and improving blood flow to affected organs
     
  • Aorta: the main artery that delivers oxygen-rich blood throughout the body
       
  • Autotransplantation: taking a healthy artery from another part of the body to replace a damaged artery
     
  • Bypass graft surgery: an operation that makes detours, or "bypasses," around narrowed arteries, creating new paths for blood to flow to the heart
     
  • Cardiac catheterization: threading a narrow tube (catheter) up through the blood vessels and into the heart, allowing doctors to check for various problems
     
  • Cardiac surgeon: a trained medical doctor who specializes in surgical procedures to repair heart defects and treat heart disorders
     
  • Cardiologist: a trained medical doctor who specializes in diagnosing and treating heart defects and disorders
     
  • Computed Tomography (CT) scan: an imaging study that moves an X-ray beam around a patient in a circular motion, taking detailed pictures of a particular organ or body part
     
  • Hypertension: high blood pressure
     
  • Interventional radiologist: a trained medical doctor who specializes in minimally invasive imaging procedures to help diagnose and treat different conditions
     
  • Midaortic syndrome (MAS): a rare disorder that causes parts of the aorta to narrow, leading to insufficient blood flow through the chest and abdomen 
     
  • Magnetic Resonance Imaging (MRI): a procedure that uses strong magnets, radiofrequency waves and powerful computers to create 2- and 3-dimensional images of organs and tissues
     
  • Nephrologist: a trained medical doctor who specializes in diagnosing and treating kidney disease and hypertension disorders
     
  • Renovascular hypertension (RVH): high blood pressure caused by narrowing of the arteries that transport blood to the kidneys
     
  • Stenting: placement of a tube called a stent in a narrowed artery in order to hold it open 
     
  • Tissue Expander Stimulated Lengthening of Arteries (TESLA): a new procedure, developed at Boston Children's, that uses a device to "stretch" healthy vessels in the aortaeventually making them long enough to replace the narrowed vessels
     
  • Transplant surgeon: a trained medical doctor who specializes in surgical procedures that transplant healthy organs to replace damaged or missing ones
     
  • Ultrasound:  a non-invasive procedure that uses special sound waves to create pictures of organs
         
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

Close