Building a Joint Pediatric/Congenital Electrophysiology Program

Background

Congenital heart disease (CHD) is the most common birth defect in the modern world, including 1% of all births in the United States.¹ As surgical techniques, post-operative cardiac intensive care, and catheter-based interventions improved over the last century, so did the survival rates of patients with congenital heart disease. Care of these patients in a single medical setting often requires significant resources and personnel, including but not limited to cardiothoracic surgeons, interventional/structural cardiologists, echocardiographers, advanced imaging experts, cardiologists specialized in heart failure/transplantation, and electrophysiologists. All of these subspecialists require training and expertise in the management of congenital heart disease from neonates through adulthood. 

A pediatric electrophysiologist is typically a pediatric cardiologist who spends an additional 1-2 years beyond their formal pediatric cardiology fellowship to gain expertise in the management of heart rhythm disorders of infants, children, adolescents, and young adults. The early pediatric electrophysiologists were self-taught, beginning with diagnostic catheter-based EP studies and eventually with catheter ablation of tachyarrhythmias, as invasive electrophysiology started to develop in adult patients in the 1970s and 1980s.² All pediatric electrophysiologists share extensive expertise in CHD and a lifelong passion for the management of rhythm abnormalities. The patient managed by a pediatric electrophysiologist can range from a fetus with a prenatally diagnosed atrial flutter or paroxysmal SVT, to an adult patient with surgically palliated CHD who presents with acute decompensated heart failure. 

The Pediatric and Congenital EP Society (PACES) was formed in 1980, and has grown considerably to foster collaboration and advance the field for our patients through multicenter research and development of standards for the management of young patients with heart rhythm disorders.^2,3 Adults with CHD represent a unique subset of the population who often need the resources of pediatric and adult cardiology programs. They may need an implanted pacemaker or ICD, a catheter ablation, or a referral for heart transplantation. 

Creation of the Joint Pediatric Heart Care Program 

Most pediatric electrophysiologists practice at large academic hospitals where there is a high volume of cardiology and cardiothoracic surgery fellows training in CHD. Due to the resources necessary in providing longitudinal congenital heart disease care throughout a patient’s lifetime and the relatively low incidence of CHD compared to adult acquired heart disease, it is uncommon for pediatric electrophysiologists to work in a solo practice or community hospital. The U.S. is a very diverse population consisting of different socioeconomic and cultural backgrounds. Most high-volume congenital heart disease centers are based in large metropolitan cities (eg, Boston, New York, Philadelphia, Houston, Los Angeles, etc.). In geographic regions that are not within driving distance to a quaternary level medical center, patients may not have the financial means to travel. This puts a significant burden on them and their families.

In 2015, the leadership of the pediatric cardiology program at the University of Kentucky (UK) reached out to the Heart Institute leadership at Cincinnati Children’s Hospital Medical Center to create a new pediatric heart surgery program for the institution. Cincinnati Children’s has a longstanding record of excellence in pediatric cardiology and cardiothoracic surgery, and is one of the top-rated children’s hospitals in the U.S. In 2016, a formal collaboration was established between the two programs to regionalize the care of patients with congenital heart disease in the geographic region, and the Joint Pediatric Heart Care Program (JPHCP) was created.

With the creation of the JPHCP, a commitment was made to provide high-quality standard of care to pediatric and adult patients with congenital heart disease as close to home as possible for patients. In 2016, I joined the University of Kentucky as the medical director of pediatric/congenital electrophysiology after finishing my clinical electrophysiology fellowship at Texas Children’s Hospital. In the first 18 months here, I worked closely with my senior pediatric electrophysiology mentors and colleagues at Cincinnati Children’s to assemble the necessary resources for pediatric and adult CHD patients to have successful invasive EP procedures on the UK campus. In the spring of 2017, after recruitment of a congenital heart surgeon and pediatric cardiac anesthesiologist, pediatric heart surgery restarted on the University of Kentucky campus. That fall, the first EP study and catheter ablation of SVT in a pediatric patient was performed at the University of Kentucky. Early in the program, higher risk procedures/surgeries and those with more severe forms of congenital heart disease had their cases performed on the Cincinnati Children’s campus, with a simultaneous, steady escalation of case complexity on the UK campus.

The same steady escalation continues to take place for the UK pediatric electrophysiology program. When I started doing invasive EP cases on the UK campus, the cases were mostly adolescents with documented paroxysmal SVT or pediatric patients with ventricular preexcitation in need of risk stratification. The device procedures I performed were usually on teenagers or young adults with post-operative or congenital heart block in need of a generator replacement or a lead revision. While growing the invasive EP volume, I was also seeing a significant number of adult patients in their 30s and 40s with palliated CHD in clinic. Many of these patients had implanted pacing systems or defibrillators, but still relied on seeing their pediatric cardiologist for medical management of their CHD. Some of these adult patients were also lost to follow-up when they “aged out,” and then reestablished care with the Kentucky Adult Congenital Heart Program after they presented with symptoms typical of an adult in acute heart failure or with a tachyarrhythmia. All congenital heart surgeries and invasive cardiac procedures are discussed in a weekly joint conference, and a collective recommendation is made on where the procedure is best performed (on the UK or Cincinnati Children’s campus), independent of the age of the patient. 

Building the pediatric EP program continues to be an exciting and rewarding experience. Our pediatric EP cases take place in a previous catheterization lab that had focused mostly on adult patients. Our first cases were adolescent patients with paroxysmal SVT with normal cardiac anatomy (typical AVNRT or accessory pathway-mediated SVT), and we are steadily increasing our volume with younger patients and those with more complex CHD. In order to safely perform pediatric/congenital electrophysiology cases with the same quality of care at most CHD centers, a 3D electroanatomic mapping system (CARTO, Biosense Webster, Inc., a Johnson & Johnson company) for fluoroscopic reduction was installed in the biplane catheterization lab in addition to having appropriately sized catheters and other supplies for such patients. Having a good working relationship with adult electrophysiologists and interventional cardiologists has allowed us to gain exposure to laser lead extractions, subcutaneous ICD implants, leadless pacemaker implants, CRT, and His bundle pacing. This unique collaboration has also allowed some of our adult CHD and pediatric patients to have access to procedures and interventions that may not be readily available in other hospitals. 

Final Thoughts

Over the past 20 years, there has been data supporting the notion that CHD care should take place in centers with high volume, and that regionalization of CHD care will improve patient outcomes for pediatric and adult patients.^4-7 Interventional fields such as electrophysiology are not immune to this phenomenon. Building a program takes time, collaboration with experienced centers/mentors, and support from one’s own institution to build these bridges. Pediatric electrophysiology is a unique field in that some of the patients we manage or consult with span the entire age spectrum, and their care goes beyond the EP lab (particularly those with complex CHD). In the past decade alone, we have seen advances in technology such as fluoroless ablations, leadless pacing, and His bundle pacing in adult electrophysiology programs. As we continue to provide appropriate care for our patients during a global pandemic, many programs and practices have had to think “outside the box” to continue care of their patients while practicing social distancing. While we have all felt isolated at various times during this pandemic, being part of a joint heart program is a reminder of the value of collaborating and building bridges to provide safe care to our young patients and those with congenital heart disease from birth through adulthood. We look forward to the next stage of growth for our program and for our region. 

Disclosures: Dr. Mohan has no conflicts of interest to report regarding the content herein.

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