Ross procedure a durable option for children with aortic valve disease

Sept. 27, 2022

In aortic valve disease, the valve between the left ventricle and the body's main artery, the aorta, doesn't work the way it should. The valve may be thickened and stiff or it may not open or close properly, causing blood to flow backward. A damaged or diseased aortic valve can affect blood flow to the rest of the heart and body.

Risk factors

Many factors can raise the risk of aortic valve disease. These include calcium deposits that can accumulate on the aortic valve as people age, as well as infection, rheumatic fever, endocarditis and other health conditions. Treatment for aortic valve disease depends on the type and severity of disease, and some patients need surgery to repair or replace the aortic valve.

Aside from these risks that can present during the lifespan, some babies are born with a missing, extra or fused valve flap. This condition increases the risk of aortic valve regurgitation. Proper diagnosis and treatment can help reduce the risk of complications in these younger heart patients.

Children and aortic valve disease

Aortic valve disease may occur later in life or it may be present at birth in the form of congenital heart disease. When this disease presents at birth or in early childhood, treatment is especially complicated because a child's body is still growing.

"Surgical options available to adults aren't suitable for children because they will outgrow them," explains Elizabeth H. Stephens, M.D., Ph.D., a cardiovascular surgeon at Mayo Clinic Children's Center in Rochester, Minnesota. "Anything that is surgically put inside to correct aortic valve disease won't grow with the child and will have to be replaced."

Joseph A. Dearani, M.D., is a cardiovascular surgeon and the director of Pediatric and Adult Congenital Heart Surgery at Mayo Clinic in Rochester, Minnesota. Dr. Dearani explains this treatment dilemma in the following way: "The challenge with treating aortic valve disease in children centers around the low probability of being about to repair — save the child's own valve — versus the need for replacement."

A durable, long-term option

The aortic valve is an important valve in terms of heart function — and when it doesn't work as it should, the Ross procedure is the first step in a series of interventions, operations and follow-up visits with a cardiologist and a surgeon. Named after European surgeon Donald N. Ross, M.B., B.Sc., the Ross procedure, also known as the switch procedure or pulmonary autograft, is a cardiac surgery in which a diseased aortic valve is replaced with the patient's own pulmonary valve.

Dr. Stephens explains how the Ross procedure works: "If a patient has a good pulmonary valve, we can use it to replace the aortic valve. Once we have the pulmonary valve in place, we use a homograft where the pulmonary valve was."

The pulmonary valve that replaces the diseased aortic valve grows with the child. "It's durable and doesn't require blood thinners," Dr. Stephens says.

Although the durability of the Ross procedure varies depending on factors that include the age of the patient and the surgeon's level of experience, the outlook is good and risks are low.

Positive outcome post-procedure

If the Ross procedure is not reinforced, there's about an 85% 10-year durability for the new aortic valve, with some experienced centers showing over 90% durability over 20 years.

"The ability to participate in sports, go to school and do all the things other children are doing, is overwhelmingly on the patient's side," says Dr. Dearani.

At Mayo Clinic, the Ross procedure is a multidisciplinary effort that requires the expertise of ICU, cardiology, imaging and OR teams to give patients the greatest chance of aortic valve durability and increased quality of life.

"We can work pretty seamlessly with the patient's home cardiologists or home doctors to provide either counsel or opportunities for them to come to Mayo Clinic for surgery," Dr. Stephens explains.

In the end, the outcome of the procedure is positive, with most patients experiencing great longevity and nearly normal — if not normal — life spans.

"There's hope. That's the positive message patients and families need to hear," Dr. Dearani says.

For more information

Refer a patient to Mayo Clinic.