Rare Conditions Treated with Care by Experts in Aorta and Vascular Program

  • March 13, 2019

Sometimes blood vessels can be too large, too small or twisted. When this happens in children’s hearts, they may be evaluated for one of the rare conditions treated by the Aorta and Vascular Program at Sibley Heart Center Cardiology.

The Aorta and Vascular Program treats a variety of rare, usually heritable blood vessel diseases, and it is one of very few in the Southeastern U.S. totally dedicated to the diagnosis and treatment of these diseases in children. Dr. Denver Sallee, MD, Director of the Aorta and Vascular Program, and his team are top experts in the field, which can provide great comfort to families facing one of these diagnoses.

The conditions treated in the Aorta and Vascular Program include:

  • Marfan Syndrome
  • Loeys-Dietz Syndrome
  • Aortic Aneurysm
  • Bicuspid Aortic Valve (BAV)
  • Ehlers-Danlos Syndrome (EDS)
  • Noonan Syndrome
  • Turner Syndrome
  • Alagille SyndromeWilliams Syndrome
  • Tuberous Sclerosis
  • Aortic Dissection
  • Aortic Ectasia
  • Enlarged or dilated vessels
  • Stickler Syndrome
  • Dilated aortic root or aorta

Patients with these conditions often need follow-up care and management. The program clinic offers close monitoring, medical management, educational support, and surgical intervention, if necessary. They also facilitate multidisciplinary coordination of care with ophthalmology, orthopedics and pulmonary physicians.

Because of the exceedingly rare nature of these conditions, families may have already interacted with providers who were unfamiliar with the disorders, driving them to do their own investigations on the internet. When they first come to the Aorta and Vascular Program clinic, they’re often relieved to have someone who can finally answer their questions and speak to their concerns.

“Many of these conditions are complex, so the appointment times are longer in our clinic than in our general cardiology clinics to permit the families to get their questions answered and determine if there is a need to involve other subspecialists to treat the other aspects of these disorders,” Dr. Sallee said. “Our expertise helps to ensure that appropriate testing and treatments are provided.”

Many of the conditions treated in the Aorta and Vascular Program are “heritable,” meaning that they can be passed on from parent to child.  The program includes two genetic counselors who can determine if screening of parents or siblings is warranted. In addition, if the patient has a heritable aortic or vascular condition, they may be at risk to pass on the condition to their offspring. Speaking with a genetic counselor helps families understand the implications of a heritable aortic condition.

For those patients who are found to have an aortic or vascular disorder, we hope that families will be comforted to know that they will have a specialized team dedicated to minimizing the impact of the condition on the long-term health of their child,” Dr. Sallee said. “With proper care and surveillance, many will not need to have surgery or other vascular interventions during childhood. We are also actually able to give good news many times, when we can rule out that they have one of these conditions. It’s rewarding to tell them they don’t require any special treatment or follow-up visits.”

Relationships can be long-lasting at the clinic since some aortic and vascular disorders can be detected at birth, and the team follows a child until he turns 21. At that time, they help transition the patient to adult providers who have familiarity with their particular issues. Because of the rarity of these disorders, Sibley doctors network with other physicians in the world who care for similar patients to pool their collective experiences, allowing them to inform families of the most current treatment recommendations.

“The physicians, nurse practitioner, and genetics counselors involved in the program are dedicated to helping families receive the best care for their children with these aortic conditions,” Dr. Sallee said.

To learn more about the Aorta and Vascular Program, visit these resources: 

Marfan Foundation

Marfan awareness walk/fundraiser

GENTAC Alliance website

CHOA Aorta and Vascular Program website

For more information about Sibley Heart Center Cardiology and our pediatric cardiology specialists, click here.