Surviving CHD: Creating a seamless transition from pediatric to adult care

Photo courtesy of Joseph Lindsey

As a cardiologist and medical director of Texas Children’s Cardiology Transition Medicine Program, I provide care to pediatric and adolescent patients living with congenital heart disease (CHD). Thanks to the groundbreaking medical and surgical advances in CHD care, more and more children with CHD are thriving into adulthood.

When I first met my patient, Joseph Lindsey, he was around 11 or 12-years-old at the time. He was born with a bicuspid aortic valve, a congenital heart defect in which the aortic valve – located between the left ventricle (main pumping chamber of the body) and the aorta (main artery of the body) – contains two leaflets instead of three. Because his aortic valve only had two leaflets, his valve wasn’t able to open fully. Over time, Joseph developed aortic valve stenosis, or narrowing of the valve, which makes it harder for the flow of blood to get from the ventricle into the aorta.

“I was actually asymptomatic most of my life,” Joseph said. “When I turned 15, I became more physically active in high school. I got into cheerleading, so I think that, coupled with puberty, exacerbated my heart condition. I noticed I would get tired faster than everyone else. I never had shortness of breath, but experienced a lot of lethargy. Over time, my stenosis got worse, and when I was 17, Dr. Dean McKenzie, my congenital heart surgeon at Texas Children’s, performed my open heart surgery to replace my aortic valve. A few days after surgery, I remember telling my mom, ‘Wow, I feel like a completely different person.’ I was more awake and alert, and I wasn’t tired all the time. I felt renewed and more energized. I am grateful for my pediatric cardiologist, Dr. Lopez, and surgeon, Dr. McKenzie. Honestly, I wouldn’t be here without them.”

Today, more than 1 million U.S. adults – like Joseph – are living with CHD. CHD is a lifelong condition and as patients survive longer, it is playing a prominent role in adult medicine, too. With CHD, several years can elapse before a patient develops the need for an intervention or has complications from their heart lesion. Patients may begin to experience abnormal heart rhythms or they may need a valve replacement several years down the road. Despite children undergoing heart surgery to repair a CHD, the reality is their condition is fixed, but is not necessarily cured. For this reason, CHD requires lifelong care and as they age, they require disease management by physicians who specialize in taking care of adults with congenital heart disease.

“Since my heart surgery nearly seven years ago, I haven’t had any major complications and I continue to receive follow-up care,” Joseph said. “I still keep in touch with Dr. Lopez, who has been a great mentor. When I first met her, she took the time to get to know me as a patient. She made sure I understood my heart condition, what my prognosis was and what interventions would look like for me in the future. Every time I’ve relocated, I’ve always asked her for recommendations on adult congenital heart providers in my area who I should consider for my care. Dr. Lopez really got to know me on a personal level and built that trust and rapport with me from the start. I think our patient-mentor relationship was important in my overall adherence to care and inspired me to take a more active role as an advocate in my own health care.”

As a pediatric cardiologist, I consider myself both as a health care provider and educator. When I became a cardiology attending in 2012, I was seeing a lot of pre-teen and teenage patients who, universally, didn’t know anything about what was going on with their heart. On multiple occasions, whenever I asked them questions, their parents would answer the questions for them. I realized there was no way these children were going to successfully transition to adulthood if they didn’t know about their condition and weren’t able to answer basic questions about their health or what to do in the event of a medical emergency.

Because of this concern and the serious disparities in the care provided to this growing adolescent CHD survivor population – including poor transfer from pediatric to adult-centered care – my colleagues and I worked together to develop the Cardiology Transition Medicine Program to address the medical, educational and psychosocial needs and skills of young adults with CHD in order to prepare them for a seamless transition and transfer to adult CHD care. When I began developing my cardiology transition team, it was Joseph who shared his ideas on what topics would be most helpful for pre-teens and adolescents.

“As a member of the CHD transition patient advisory panel, I embraced the opportunity to work with Dr. Lopez and share my input during the early stages of this program,” Joseph said. “Since I was in college, I was already in that transition phase, so I was able to share my perspective on what information would benefit patients my age as they transition and transfer to adult care. The Cardiology Transition Medicine Program empowered me to take charge of my own health care. It held me accountable in many ways to ensure I was efficient in scheduling my own appointments and staying on top of my cardiac care needs.”

The Cardiology Transition Medicine Program trains adolescents starting at the age of 14 to be responsible adults with CHD. A trained cardiology nurse and social worker conduct a needs assessment and create an individualized learning plan specific to the adolescent’s needs, skills, and knowledge gaps. Serial educational and skill building sessions are conducted to ensure appropriate knowledge and skill base prior to transferring to adult care. There are also opportunities for patients to meet other teens with CHD and serve as each other’s mentors. When patients turn 18, they transition to our Adult Congenital Heart Program, specifically designed to treat adults with CHD to ensure they live longer, healthier and productive lives despite their heart condition.

Currently, there are 550 board-certified adult congenital heart providers in the U.S. It’s estimated for every one adult congenital heart provider, there are 4,500 CHD patients. Clearly, there remains a critical need for more providers to be trained to care for this growing and still underserved patient population. As more adults are surviving CHD, transition programs like ours are helping adolescents and parents prepare for this transition and transfer to adult care, which is an important step in preventing unnecessary lapses or gaps in their care.

Click here to learn more about Texas Children’s Cardiology Transition Medicine Program. Click here for more information about our Adult Congenital Heart Program.