A Place of Hope: Juli’s Story

A Place of Hope: Juli’s Story

A Place of Hope: Juli’s Story

My heart hero, Brandon, was born in 2001 before fetal echocardiography was common. Born at 34 weeks in a local hospital with no NICU, Brandon was initially diagnosed with hypoplastic left heart syndrome (HLHS). Two weeks later, Brandon was examined by Dr. Meryl Cohen at Children’s Hospital of Philadelphia’s (CHOP’s) Specialty Care Center in Chalfont. After a few hours of testing, we quickly learned Brandon did not have HLHS, but rather a very stenotic bicuspid aortic valve. Once we received this diagnosis, our care at CHOP Philadelphia hospital began. Dr. Cohen has since become a trusted partner in Brandon’s care, and a once-in-a-lifetime gift to our family.

Juli with Brandon

Brandon’s early care at CHOP included two balloon valvuloplasty procedures. In 2015, he underwent the Ross procedure as well as an aortic reconstruction. Throughout this time, Dr. Cohen’s close monitoring has built an incredible foundation for our son’s health and has also been a tremendous support for our family. Her kind, positive and easy-to-understand explanations, as well as her focused, compassionate care of her patients has made her an incredible steward for countless heart families like ours. We have also valued the family-centered care CHOP provides. From the food service staff to the surgeons, the CHOP team consistently considers every detail of the patient and family experience. Our gratitude for the care we’ve received at CHOP has lessened the concern we once had regarding his CHD.

  • Ross Procedure for Damaged Aortic Valve in Children and Teens

    Jonathan Chen, MD: The Ross procedure is a surgical technique that can be used to replace a damaged or diseased aortic valve. I'm Jonathan Chen, a surgeon in the Pediatric Heart Valve Center at Children's Hospital of Philadelphia.

    My team is one of the most experienced in the country in valve repair and replacement, and we regularly perform the Ross procedure. To better understand the procedure, let's first take a look at the anatomy of your heart. This is your heart. It has four chambers and four valves. Blood passes through a valve before leaving each chamber of the heart. The valves prevent the backward flow of blood.

    To understand the Ross procedure, you'll need to know about the aortic valve and the pulmonary valve. The heart's lower chambers are called ventricles. The left ventricle pumps oxygen-rich blood through the aortic valve and into the aorta, the largest artery in the body. Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients. Blood that has traveled through your body and is now low in oxygen goes into the right side of the heart.

    The right ventricle pumps the blood through the pulmonary valve, into the pulmonary artery and to the lungs for more oxygen.

    If the aortic valve is diseased or damaged, it can be surgically removed and replaced with the body's own healthy pulmonary valve. This is known as the Ross procedure. Here's how it works. First, the surgeon must detach the coronary arteries from the aorta. The coronary arteries are the blood vessels that feed oxygenated blood to the heart.

    Then the surgeon will remove the diseased or damaged aortic valve, as well as the aorta surrounding the valve. The healthy pulmonary valve, surrounded by a cylinder of pulmonary artery, is then removed from its usual location and sewn into the aortic position. This is called an autograft. The surgeon then reattaches the coronary arteries to the autograft.

    A donor cadaver pulmonary valve surrounded by a cylinder of pulmonary artery is then attached replacing the original pulmonary valve. This is called a homograft.

    The Ross procedure is a highly technical surgery. At the Valve Center at Children's Hospital of Philadelphia, our team uses cutting edge imaging to view each patient's heart before surgery. This allows us to develop a highly personalized surgical plan based on your child's unique anatomy. As with any heart surgery, the Ross procedure has advantages and disadvantages.

    Because the Ross procedure uses the patient's own tissue, there's little risk of clotting. This means your child won't need to take long-term blood thinners. The pulmonary valve in the aortic position, remember this is called the autograft, continues to grow with your child, so it won't need to be upsized as your child grows. Sometimes, however, the autograft has to be replaced because of wear. The donor pulmonary valve, remember this is called the homograft, does not grow.

    In kids, it will eventually need to be upsized, which will require a future operation. In teenagers and adults, the homograft won't need to be upsized, but the valve inside the homograft might become leaky. If that happens, the valve can probably be replaced through a catheterization instead of surgery. Our team is a world leader in the Ross procedure.

    We work together and with you, to make the best decisions for your child before, during, and after surgery.
Transcript Transcript

Being a mom of a child with CHD has been difficult; early on I struggled with feelings of guilt, as though I could’ve done something to prevent Brandon’s heart condition. I actively sought therapy to process the impact of CHD on our family. Therapy helped me move quickly from a place of despair to a place of hope. Since then, I find solace in sharing our family’s story with other families and supporting them through the process. I have met countless heart families on our CHD journey, and each one has their own unique story. Opening a space for conversation allows families to be heard, seek community and further advocate for their children.

Juli, Brandon, and family

Over the last two decades, we’ve had the benefit of significant advancements in technology and techniques related to CHD care. Now 21, Brandon will soon require another double prosthetic valve procedure. In 2023, he’ll transition to the Philadelphia Adult Congenital Heart Center, an innovative collaboration between CHOP and the Penn Medicine Health System. We are so fortunate to have navigated his first 21 years at CHOP and feel well-supported in our transition to adult care.

Juli’s Advice for Heart Families

  • Ask for help when you need it
  • Seek support and education through a productive community
  • Find the right medical team that meets your family’s needs
  • Rely on hospital services, such as clinical research opportunities and Child Life