An interrupted aortic arch (IAA) is a rare congenital heart defect that interferes with blood flow in the aorta, the body’s largest blood vessel. It’s typically diagnosed in infancy but, in rare cases, is diagnosed in adults. No matter the age of diagnosis, the experts at the Adult Congenital Heart Program at UK Gill Heart & Vascular Institute provide high-quality, lifelong care for adults with IAA.
The aorta carries blood from the heart to other body parts. It starts at the aortic valve in the hearts’s left lower ventricle, or chamber. Then, it goes up toward the head before curving down toward the pelvis. The section of the aorta going up from the lower left ventricle is called the ascending aorta. The descending aorta goes down. The curved part that connects them is the aortic arch. Several smaller arteries branch off from the aortic arch, including the:
- Brachiocephalic artery, also known as the innominate artery
- Left carotid artery
- Left subclavian artery
Each smaller artery routes blood to a different area. In patients with an IAA, the aortic arch doesn’t develop fully in the womb. This causes a gap that blocks blood flow to one or more smaller arteries.
In cases where IAA isn’t diagnosed until adulthood, the gap may only be partial at birth, developing into a complete block of blood flow over time.
Patients with an IAA often have a large ventricular septal defect (VSD), a hole between the lower heart chambers. They may also have additional congenital heart defects, including:
- Atrial septal defect, a hole between the upper heart chambers
- Truncus arteriosus, having one main artery instead of two
In infants, symptoms of IAA are often severe. However, in adults, symptoms can range from absent to significant and may include:
- Absent or weak pulse
- Congestive heart failure
- Headache
- High blood pressure, also known as hypertension
- Malaise, or a general sense of discomfort or not feeling well
- Pain in the legs with exercise that goes away with rest, also known as claudication
- Significantly different blood pressure readings in the upper and lower body
Without surgery, nine in 10 infants with an interrupted aortic arch die within the first year of life. With surgery, about eight in 10 children with an IAA are alive 10 years after surgery.
In the rare cases when IAA is diagnosed in adulthood, about 13 out of 100 adults have a life-threatening complication at the time of diagnosis. With surgical treatment, adults with IAA generally have a good prognosis.
An interrupted aortic arch is often caused by a genetic condition. However, sometimes the cause is never known.
About half of all people with IAA also have a genetic condition called DiGeorge syndrome, also known as 22q11.2 deletion syndrome. This condition may cause a range of issues, including other congenital diseases, developmental disabilities, hearing or vision issues or seizures.
CHARGE syndrome, another genetic condition, has also been linked to IAA.
Children with congenital heart disease become adults with congenital heart disease (CHD). Ninety percent of children born with CHD have a normal life expectancy due to improved surgical interventions and better medical management. At all ages, close monitoring and expert care for CHD patients are essential. Despite this, studies indicate that 2 out of 3 young adults with CHD don’t transition from pediatric to adult CHD care. Not getting continued cardiac care can lead to a variety of health problems and poor outcomes.
To guide teens and their families through the transition, UK Gill Heart & Vascular Institute team members talk about the transition early and often. Starting at age 15, teens with CHD begin transition education. One Wednesday every month, a special transition clinic staffed by adult congenital heart disease providers, is held to meet with teens and their families to discuss important topics, including:
- Abilities and limitations experienced as an adult with CHD
- Lifelong need for proper CHD monitoring and treatment
- What adult CHD care may involve over the years
Additionally, once you transition to adult care with the Adult Congenital Heart Program at UK Gill Heart & Vascular Institute, we work hard to enable you to keep your appointments and testing on track. We also work hard to empower you with knowledge and assist you in being your best advocate to live your best life. If you miss an appointment, we will call to reschedule.
During your first visit to the UK Gill Heart & Vascular Institute’s Adult Congenital Heart Program, you’ll meet our clinic nurse, social worker and other members of your care team. You’ll also undergo imaging exams, which may include an echocardiogram or a cardiac MRI.
Once imaging is complete, you will meet with an adult congenital cardiologist and other team members for up to one hour. You’ll discuss the results of your echocardiogram and your health history and symptoms. You will also get in-depth education about your diagnosis, how it may affect you moving forward and possible treatment options. The meeting is also a great opportunity to ask any questions about living with CHD.
When coming to your appointment:
- Bring a list of any medications or supplements you take and surgeries you’ve undergone.
- Dress comfortably.
- Have your insurance information on hand. UK HealthCare accepts most insurers, and financial assistance is available.
- You may want a family member or friend with you to take notes or help ask questions.
Where we are located
The UK Gill Heart & Vascular Institute is located at 800 Rose St. in Pavilion G, on the first floor of UK Albert B. Chandler Hospital. We will mail you a map and directions when your appointment is scheduled.
Patient drop-off
Patients can be dropped off in front of the main entrance to Albert B. Chandler Hospital at 1000 S. Limestone. If you are dropped off at the main entrance, you’ll enter the revolving doors on the ground floor of the hospital. Take the main stairs just to the left (or elevator across the atrium) to the first floor of Pavilion A.
After climbing the stairs or taking the elevator, turn left. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
Parking
It is easiest to park in the UK HealthCare Parking Garage, across from the hospital at 110 Transcript Ave.
There are two paths from this garage to the Gill clinic:
- You may take the free shuttle from Level A of the parking garage. You’ll exit the shuttle at the Pavilion A/ Pavilion G stop in front of the main entrance to the hospital. After climbing the stairs or taking the elevator, turn left. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
- From Level C of the parking garage you may walk or take a golf cart across the pedway to the first floor of Pavilion A. Turn left past the help desk and walk away from the dining hall. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
If you need help finding your way, information desks are located inside the main entrances to Pavilion A, both on the ground floor and at the end of the pedway on the first floor. You may also call the information desk at 859-323-5816.
Clinical trials provide access to novel treatments for those in the adult CHD program at UK Gill Heart & Vascular Institute. Through our Cardiology Clinical Research Center, patients benefit from advances in valve replacement and other therapies before they become standard treatment. Your care team will notify you if you qualify for an ongoing clinical trial.
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