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Managing Congenital Heart Disease in the Teen Years and Beyond

Transitioning from childhood to young adulthood is often difficult for teenagers. They face questions and pressure on a number of fronts—from physical and emotional changes to career decisions. For those living with congenital heart disease, the added stress of managing their own care and medication for the first time can make this transition even more challenging. 

A new transition education program within the Guerin Family Congenital Heart Programa collaboration between the Smidt Heart Institute and Guerin Children’s—is designed to help teenage patients with congenital heart disease transition from pediatric providers to specialized adult providers, ensuring a smooth continuum of care throughout each patient’s life.

A Lifelong Medical Home

In the Los Angeles region alone, it is estimated that less than 20% of patients living with congenital heart conditions receive proper specialized care once they reach adulthood. Many of these patients are likely treated by a pediatric cardiologist from the time they are born but do not make a successful transition to adult providers as they emerge into adulthood.

“In addition to pediatric cardiologists and congenital heart surgeons, our Guerin Family Congenital Heart Program is unique in that it includes adult congenital cardiologists and adult cardiac surgeons who are trained in dealing with complex adult manifestations of congenital heart disease,” said Richard Kim, MD, director of Pediatric and Congenital Cardiac Surgery in the Smidt Heart Institute.

“The variety of training and expertise of the specialists within our program gives us the unique ability to provide seamless care from the newborn period through adolescence and well into adulthood,” said Kim. 

The adult congenital cardiologists within Cedars-Sinai’s transition education program have an in-depth understanding of both congenital heart disease and adult cardiology. By combining the expertise of adult congenital cardiologists and pediatric cardiologists within a single highly collaborative environment, the program ensures an optimal experience and improved patient outcomes. 

“It is really important to recognize that a transition of care is a lot more than just a transfer of care,” said Rose Tompkins, MD, associate director of Cedars-Sinai’s Adult Congenital Heart Program in the Smidt Heart Institute.

“It’s a focus on education for the teens and parents, not just an introduction of a new provider. For parents, it can be hard to take a step back and allow their child more autonomy in the management of their care. For teens and young adults, they need to build the confidence and motivation to take the baton from their care providers and start to feel ownership of their health. This includes learning more about their diagnosis and medications, understanding insurance coverage, feeling comfortable making their own medical appointments, considering how their future vocation might impact their situation, and generally adopting healthy lifestyle behaviors. Most important is the understanding that congenital heart disease requires lifelong specialty care,” Rose explained.

The unique program includes a series of readiness tools that help patients and families navigate tough topics, such as physical maturity, the impact of drugs and sex on heart health, and more. A safe space is created for these conversations by including the pediatric specialist with whom the patient has a history as well as adult congenital heart specialists.

While the transition education program is generally designed for patients who are 16-22 years old, participation is encouraged whenever a patient is ready based on their individual maturity. The type of education offered has also been useful for any young adult patients seeking to re-establish care, some of whom may have experienced a lapse in care after adolescence and may be finding their own health insurance for the first time. 

“We find that we see a loss of patients in that 16- to 26-year-old range,” said Tompkins. “The transition from pediatric to adult care is a particularly vulnerable point that has been well studied, and if missed, when patients re-enter care later in life, they may have additional issues or be more ill than if they would have continued care seamlessly.” 

The Cedars-Sinai Difference

Cedars-Sinai is uniquely positioned to be that lifelong medical home for congenital heart patients. The Guerin Family Congenital Heart Program is not only a vital part of Guerin Children’s but is housed in the Smidt Heart Institute, which was recognized by U.S. News & World Report as #1 in California and #3 in the nation for Cardiology & Heart Surgery in 2022. 

“We have both broad and specialized expertise at Cedars-Sinai,” said Kim. “Although patients with congenital heart disease could be treated or operated on as a newborn, they might need multiple procedures or interventions throughout their life. Some patients come to us at birth, but some we don’t see until they are 60 or 70 years old.” 

Evan Zahn, MD, director of the Guerin Family Congenital Heart Program and the division of Pediatric Cardiology in the Smidt Heart Institute, considers patient experience the highlight of being treated at Cedars-Sinai. 

“Every patient we treat, from fetal diagnosis well into their senior years, is discussed weekly in great detail with our entire multidisciplinary team,” said Zahn. “These discussions are greatly enhanced by the attendance of pediatric and adult cardiologists, surgeons, anesthesiologists, intensivists, and any other specialists who might have an important perspective that could improve our patients’ experience and outcome.” 

Though it is a relatively young program, Kim estimates they have already had about 110 surgical cases to date with zero mortality, even considering the high complexity of the patient population. 

A Commitment To Collaboration

Collaboration has been a staple of the transition education program from its inception, and the program continues to grow in expertise and capability, including a surgical component. As the program grows, so does the team’s commitment to working closely with referring pediatricians, pediatric and adult cardiologists, and primary care providers. 

“Our entire team believes strongly in the importance of developing collaborative relationships with physicians who trust us to care for their patients,” said Kim. “We do everything we can to remove barriers to communication. When referring physicians want to speak with us, we want them to know we’ll answer and be ready to have a thoughtful, respectful conversation.” 

The team’s goal is to be a collaborative part of the patient’s care team and a useful resource for referring physicians. 

“The importance of our referring physician community to our patients’ care cannot be overstated. We recognize and respect the deep bond that exists between patients and their primary providers and believe it is essential to maintain and enhance that relationship,” said Zahn. “When you come to our program, no one loses a patient or a doctor. Your team just gets bigger.” 

Tompkins, Kim and Zahn hope to continue to expand the program’s expert staff and patients’ access to subspecialty care, especially for those teenagers and young adults dealing with congenital heart disease. 

“Teenagers and young adults tend to feel invincible—even those with congenital heart disease,” said Tompkins. “We can provide the care, expertise and tools they need to navigate this tricky time. We don’t want to take that feeling of invincibility away; we want to provide a seamless continuum of care that helps them hold on to it throughout a long, fulfilling life.”