The Pediatric and Adult Congenital Electrophysiology Society (PACES) held a one-day seminar entitled Sudden Cardiac Arrest in the Young on May 8, 2007, the day prior to this year’s Heart Rhythm Society (HRS) meeting in Denver, Colorado. The meeting was deemed an official satellite symposium by HRS, and occurred under the auspices of President John Triedman of PACES. The event was co-chaired by Dr. Stuart Berger, from Children’s Hospital of Wisconsin and the Medical College of Wisconsin, and by Dr. Robert Campbell from Children’s Healthcare of Atlanta and Emory University. Dr. Michael Schaffer and his staff at Denver Children’s Hospital coordinated the event itself.

Figure 1.

The conference organizers (from left to right: Drs. John Triedman, Mike Schaffer, Stu Berger and Robert Campbell).

       The goal of this symposium was to present a state-of-the-art, succinct review of the issues important in understanding and preventing sudden cardiac arrest (SCA) and sudden cardiac death in children and adolescents. The symposium was divided into four sessions. The sessions included a disease-based session, a session to discuss ICDs, a session covering primary and secondary prevention, and a dinner session discussion of the psychology of sudden cardiac arrest and its therapy. After each session, the 170-person audience was polled via an audience response system. Questions asked included audience demographic information as well as questions with regard to attitudes about the diagnosis and therapy of the entities presented during the symposium. The answers to the queries were presented at the annual PACES meeting on the following evening, prior to a keynote address by Barry Maron, MD.

Figure 2.

Charlie Berul during his presentation on ICD indications.

Figure 3.

Maully Shah during her presentation on ICD complications.

       The disease-based session was moderated by Martin Runciman, MD and Philip Saul, MD. Jeff Towbin, MD started this session by providing a summary and update on hypertrophic cardiomyopathy in children and adolescents, and included a classification and review of the molecular genetics. He also discussed the area of restrictive cardiomyopathy. Silvia Priori, MD followed with a discussion of LQTS as well as CPVT. In each case, an updated summary of the genetics was followed by a risk stratification and discussion of rationale therapy based on the evidence that is currently available. Next, Dr. John Triedman discussed the rarer electrical abnormalities of Brugada syndrome and short QT syndrome in children. Peter Frommelt, MD reviewed the congenital coronary artery abnormalities associated with SCA, primarily the coronary artery that arises from the opposite sinus of Valsalva and courses in an interarterial course. A discussion of the echo diagnosis of these abnormalities, classification based on the course of the anomalous coronary artery, the potential role in SCA, and treatment strategies was also presented. Robert Hamilton, MD FRCP(C) ended this session with a presentation of ARVC and dilated cardiomyopathy in children, as well as approaches to diagnosis and therapy.

Figure 4.

Barbara Deal and John Triedman.

       The second session was moderated by Drs. Barbara Deal and Michael Silka. During this session, Charles Berul, MD covered the topic of ICD therapy in children. In his presentation, he pointed out — amongst many things — that we do not have prospective studies on this topic, nor do we have uniform agreement with regard to implant indications. Maully Shah, MBBS also presented information with regard to the potential complications associated with ICD therapy in children.

Figure 5.

The conference co-chairs Stu Berger and Robert Campbell.

       The third session was moderated by Drs. Seshandra Balaji and Chris Anderson. Robert Campbell, MD started this session with a discussion of pre-participation screening. He reviewed the current data on pre-participation screening and stressed the importance of a uniform, comprehensive pre-participation approach, as offered by the American Academy of Pediatrics (AAP), in optimizing the prevention of SCA. Vicki Vetter, MD followed with a review of electrocardiogram (EKG) screening programs, and detailed the successes of the screening programs in Japan and Italy. She also discussed the results of her local pilot EKG and echocardiographic screening programs, suggesting that expanded studies in this area would be very important. Dr. Stuart Berger reviewed secondary prevention with AED programs, including the utility and legislative and policy issues, as well as the barriers and criticisms of such programs, especially those targeted for schools. Lastly, Michael Ackerman, MD presented the molecular genetics of SCA as well as the utility of genetic testing. He discussed the role of genetic testing in cases of sudden cardiac death in which the autopsy results fail to find a specific etiology for the death.

Figure 6.

A panel discussion/question/answer period with Vickie Ziegler, Sam Sears, Michael Vincent and Alice Lara.

       The last session, which was held after dinner, was moderated by Drs. Berger and Campbell. Dr. Sam Sears, the first speaker, gave an overview of the psychology of SCA and issues related to therapy, including ICD therapy in the young. Vicki Ziegler reviewed the results of her dissertation detailing the attitudes of the young as it relates to SCA and its therapy; for her dissertation, she had interviewed a population of young patients who were diagnosed, treated and were at risk for SCA. Alice Lara, Executive Director of the Sudden Arrhythmia Death Syndromes (SADS) Foundation, represented the patient community and spoke on the role of patient advocacy for both health care professionals and families. Finally, Dr. Michael Vincent finished the program with reflections on family care, once again emphasizing the need to provide the entire family with consistent and accurate information.

Figure 7.

Denver Children’s Hospital, the venue for “Sudden Cardiac Arrest in the Young”.

Conclusion
       Overall, the symposium was well attended and well received. The organizers feel that this symposium will serve as a stepping stone to additional meetings and discussions about topics related to sudden cardiac arrest in the young.
       It is also quite timely that HRS launched and announced The Sudden Cardiac Arrest Coalition at the scientific sessions this year (see sidebar). PACES is hoping to be a key member of that coalition, as well as further the cause with future endeavors, which include future meetings with focus on more detailed discussion of topics such as the disease-based causes of sudden cardiac arrest. In addition, there continues to be tremendous interest in screening, including the optimization of the pre-participation evaluation of athletes and non-athletes, the feasibility of EKG screening of athletes as well as all school children, the feasibility of echocardiographic screening, and also the feasibility and cost-effectiveness of secondary prevention programs such as school-based AED programs. The organizers are hoping for a future consensus conference and for future work in the area of sudden cardiac arrest in children and adolescents via the Sudden Cardiac Arrest Task Force of PACES.

About PACES
       PACES, formerly known as the Pediatric Electrophysiology Society (PEPS), began in the early 1980s as a small group of individuals interested in diagnostic electrophysiology in children. It has evolved into an international organization focused on the entire range of electrophysiology, including diagnostic, ablation/intervention, implants of pacemakers and ICDs, genetics, and sudden cardiac death in infants, children and adults with congenital heart disease.

For more information on PACES, please visit: www.pediatricepsociety.org

In the News: Heart Advocacy Groups Announce First-Ever Coalition Urging Federal Government Action to Stop Sudden Cardiac Arrest

- More than 25 leading heart advocacy groups announced the formation of the Sudden Cardiac Arrest (SCA) Coalition, the very first coalition dedicated to advancing increased research, awareness and educational efforts to address the nation’s number one cardiovascular killer. The Coalition, announced as part of Heart Rhythm 2007, the Society’s 28th Annual Scientific Sessions, will urge Congress and the federal agencies involved in national health policies and programs, to devote more resources to greater public awareness of SCA, research and access to life-saving therapies.  “Sudden Cardiac Arrest is a leading cause of death in this country, taking the lives of more than 250,000 people each year — more than breast cancer, lung cancer, stroke or AIDS,” said Dr. Dwight Reynolds, president of the Heart Rhythm Society, one of the founding members of the SCA Coalition. To address this public health crisis, the SCA Coalition is made up of advocates from across the spectrum — from physicians and allied health professionals, to patients and caregivers, to first responders and athletic trainers. These groups come together under the Coalition to use their collective strength as a national voice to stop sudden cardiac arrest. Over the next year, the SCA Coalition will encourage the introduction and passage of an Omnibus bill that, if passed, will provide the U.S. Department of Health and Human Services with the resources and the imperative to develop and implement a comprehensive education and research program for SCA. This will include appropriations for medical screening and tracking studies, as well as public awareness and education campaigns. The bill will also include a resolution to create a National Sudden Cardiac Arrest Week, to set aside time each year to focus national public attention on this deadly killer.