As part of our first Pediatric EP blog, it is timely to be able to report on the recent 5th Charleston Symposium on Pediatric Electrophysiology, which took place December 13-15, 2009, in Charleston, South Carolina. Based on feedback from prior years and an interest in keeping the course updated over time, this year’s program was somewhat different by focusing primarily on how to make decisions on care rather than the technical aspects of delivering care. To that end, most of the talks were preceded by a case report that demonstrated the complexity of decision making in the relevant topic and posed a set of questions for the speaker. Apparently, our brochure “sold” this concept well, because of the 100 or so attendees, over half were a mix of non-electrophysiologists, including pediatric cardiologists, adult cardiologists and nurse practitioners. Attendees came from all over the U.S., as well as Japan and five countries in Europe.

The program began on December 13th at 1 PM with a session entitled “Management of Selected Clinical Arrhythmias with Ablation as an Option: Decision Making,” moderated by my co-director, Thomas Paul, MD of The Children´s Heart Center in Germany. After Dr. Edward Walsh of Children´s Hospital in Boston presented a case of an 8-year-old girl who died suddenly after being followed for asymptomatic WPW since infancy, I delivered the first lecture on management of the asymptomatic child with WPW. My review of the topic covered a surprising amount of recent literature supporting the notion that it is in fact the asymptomatic child with WPW who is at the highest risk, indicating that risk assessment is appropriate in children between the ages of 5 and 10 years. After another case report, Dr. Walsh reviewed contemporary management of paroxysmal SVT in the child, focusing on when to ablate, when to use other therapies, and what type of ablation is most appropriate. We were then fortunate to have Dr. Marcus Wharton, an adult cardiologist from MUSC who specializes in atrial fibrillation (AF), with us to provide an overview on who can benefit from AF ablation, which congenital heart patients might be candidates, and what the most current techniques are. By demonstrating that even patients with high left atrial pressures and CHF can improve dramatically after AF ablation, he proposed the intriguing idea that some of our most complex congenital heart patients with AF might not only be candidates for ablation, but that it could improve their systemic ventricular function as well. Dr. Paul finished the session with a talk on idiopathic ventricular tachycardia.

December 14th was devoted to electrical cardiomyopathies and those that can cause sudden death, with the morning sessions focused on diagnosis and the afternoon sessions on risk assessment and therapy. We were indeed fortunate to have for these sessions Dr. Michael Ackerman of the Mayo Clinic, who gave five lectures throughout the course of the day. I began the day providing an overview on syncope and how to differentiate malignant from benign presentations. Then Dr. Ackerman provided an outstanding summary of state-of-the-art genetic testing, how to interpret the tests, and the relevance of polymorphisms. He also introduced the case of a family with hypertrophic cardiomyopathy (HCM) in which two phenotypical and genetically affected children died suddenly and a third child was genetically confirmed with the disease but had a normal phenotype. This case was re-discussed throughout the day in terms of appropriate management of the third child as well as the parents. Next, Dr. John Reed from MUSC covered ECG documentation methodologies and interpretation. The session then turned to management of specific chanelopathies including LQTS and the less common Brugada syndrome, short QT and catecholamine polymorphic ventricular tachycardia (CPVT). Dr. John Triedman of Children’s in Boston introduced the topic with a great case of a child with genetically confirmed Brugada; Dr. Ackerman followed with a 40-minute lecture covering a wide range of clinical issues. The final talks of the morning were on ARVD, delivered by Dr. Mitchell Cohen from Phoenix Children’s, and then back to Dr. Ackerman for a talk on how to risk stratify in patients with HCM. Dr. Cohen provided a terrific overview on ARVD, focusing on how to diagnose it and which patients are best managed with medication, ablation and ICD placement. Then Dr. Ackerman made a compelling argument that, with few exceptions, the specific genetic defect in HCM is not a good risk stratifier, but having more than one risk factor including family history, syncope, VT, massive LVH, BP drop with exercise and LVOTO is a pretty good predictor for who might benefit from an ICD. Mike was also able to provide extensive data from his own large patient population of both HCM and LQTS patients.

Monday afternoon led off with a dramatic case presentation by our MUSC EP fellow, Dr. Marty LaPage, of a 3 year old with LQTS who has had over 100 ICD shocks after a left stellate ganglionectomy at Mayo Clinic and a wide variety of maximal drug therapies. Dr. Ackerman then discussed the latest treatment strategies for LQTS, including stellectomy. He presented convincing evidence that most LQTS patients can be managed with medication. Of the 15% of his cohort who have received an ICD, only 21% have received an appropriate shock, and none of those had LQT3. Stellectomy seems to be very effective for ICD patients who continue to be appropriately shocked despite adequate medical therapy or for patients who cannot or don’t want to take beta-blockers. After another case presented by Dr. Thomas Kriebel, Dr. Triedman reviewed the indications and technical choices for the pediatric patient who needs an ICD. A variety of non-transvenous approaches were discussed. There was such interest in Dr. Ackerman’s presentations that he agreed to stay through the afternoon break to answer questions. After the break, he did his final presentation on the 2005 published Bethesda Guidelines for sports participation with the conditions discussed during the day, the role an ICD plays in those guidelines and the relative lack of evidence to support the guidelines. His conclusion was that the Bethesda Guidelines are very conservative and his recommendations were that these decisions could be more individualized, potentially allowing select patients with well-informed families to participate in more sports. The day finished up with a panel discussion on sports participation in channelopathy and genetic cardiomyopathy patients, which attracted a lot of questions and attention from the audience. Unfortunately, we had to bid Dr. Ackerman goodbye to catch an evening plane just before the panel.

Tuesday, December 15th was a full day, with a morning session on cardiac resynchronization (CRT) in the pediatric patient with and without congenital heart disease (CHD), and an afternoon session on atrial and ventricular tachycardias in the postoperative patient with CHD. The CRT session began with another case from Dr. LaPage, who presented on a patient with an atrial switch for d-TGA, surgical heart block, dyssynchrony and late ventricular dysfunction who was upgraded to a CRT device, but had minimal to no improvement. He posed the questions: who should have such devices, how do we put them in patients with abnormal anatomy, and how do we decide if they are working. These were the critical questions for management, and all were addressed by the speakers that followed. First, Dr. Hamilton Baker from MUSC reviewed the echocardiographic assessment of synchrony, followed by Dr. Cohen reviewing the role of CRT in the patient without congenital heart disease who has either a primary cardiomyopathy or one secondary to pacing for complete heart block (CHB). Dr. Triedman then presented cases of a young child with d-TGA and surgical CHB as well as a 60+ year old with corrected TGA, both who improved dramatically after CRT. Following these cases, Dr. Jan Janousek from the University of Leipzig in Germany provided an excellent review and meta-analysis of the available published data series on patients with CHD who have undergone CRT. He ended that presentation with a pair of slides proposing the concept that patients with pacing-induced dyssynchrony are very likely to have a virtual cure by CRT, while patients with primary pump failure and dyssynchrony may have some improvement with CRT, but it is likely to be transient due to the progressive nature of the underlying myopathy. After the break, Dr. Cohen presented another illustrative case on a single ventricle patient who underwent successful CRT pacing, followed by Dr. Barbara Deal from Children’s Memorial in Chicago presenting an excellent overview of how to evaluate the need for ICD backup in some pediatric patients with CRT. Finally, after Dr. Walsh presented a case on the complications that can be encountered in pacing a child with CCHB from infancy through adulthood, Dr. Janousek provided an excellent review of the transvenous versus epicardial pacing in the child and made the convincing case that almost all children < 30 kg should have their first device placed epicardially and the lead placed as close to the LV apex as possible to optimize synchrony. The audience seemed to be mostly in agreement when surveyed informally.

The final afternoon session had a nice mix of a few “experts” providing a brief 10-15 minute how-to on their management techniques of a variety of arrhythmia categories: Dr. Walsh on atrial tachycardia in the patient with two ventricles, Dr. Janousek on atrial tachycardia in the atrial switch patient, myself on atrial tachycardia in the Fontan patient, and Dr. Cohen on ventricular tachycardia in the patient with congenital heart disease. The session ended with updates on the techniques and outcomes for catheter ablation of atrial tachycardias by Dr. Triedman, substrate mapping of ventricular tachycardias by Dr. Paul, and surgical management of arrhythmias by Dr. Deal.

Review of the evaluations revealed that our focus on management hit the “sweet spot” for a wide variety of practitioner types, including pediatric and adult subspecialties. From the feedback, it is clear the attendees felt the information was very valuable for their practices. Our day on channelopathies and cardiomyopathies appeared to be a big hit. I know I learned a lot as well. We look forward to using the feedback to design an even better course for the future!

J. Philip Saul, MD is a Professor and the Chief of Pediatric Cardiology at the Medical University of South Carolina (MUSC) in Charleston.