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Cardiac scientists at Lankenau Institute for Medical Research release consensus report on J-wave syndromes

July 13, 2016 News Releases

Report aims to educate health care professionals on how to understand, diagnose increasingly researched arrhythmic conditions that lead to sudden cardiac death

Two cardiovascular experts from Lankenau Institute for Medical Research (LIMR) and Lankenau Heart Institute (LHI), part of Main Line Health, led an international task force to develop a first-of-its-kind consensus report and book on J-wave syndromes, an increasingly researched cause of ventricular fibrillation responsible for sudden cardiac death.

The publications follow months of work from a task force of cardiac specialists from 17 biomedical facilities from around the world, led by Charles Antzelevitch, PhD, professor and executive director of cardiovascular research at LIMR and director of research at LHI, along with Gan-Xin Yan, MD, PhD, professor at LIMR and clinical cardiologist at Lankenau Medical Center.

A book, titled J-wave syndromes, will accompany the manuscript J-wave syndromes expert consensus conference report—the latter of which will be published simultaneously in the medical journals Heart Rhythm, Journal of Arrhythmia and EP-Europace. The consensus report and book were written to assist the health care community in understanding the underlying mechanisms that lead to these syndromes, how to diagnose them and how to treat them.

“While the ultimate judgment regarding care of a particular patient must be made by the health care provider based on all of the facts and circumstances presented, our expert consensus report is intended to assist health care professionals in their clinical decision making,” said Dr. Antzelevitch. “It is designed to keep physicians fully informed as to new genetic information, diagnostic criteria, risk stratification strategies and approaches to therapy for these serious heart conditions.”

Manifestations of J-wave syndromes

Research around J-wave syndromes has followed stories of seemingly healthy young people dying suddenly—often during sleep—driving cardiovascular experts to discover the cause and risk factors associated with these deaths.

The two most common manifestations of J-wave syndromes are early repolarization syndrome and Brugada syndrome (BrS), which are both associated with a person’s susceptibility to develop a rapid and irregular heart rhythm called polymorphic ventricular tachycardia and ventricular fibrillation. This can lead to sudden cardiac death in young adults—especially young men—who have no apparent structural heart disease. These cardiac conditions also can lead to sudden infant death syndrome (SIDS).

The authors noted that early repolarization pattern (ERP)—prevalent in up to five percent of the population—often occurs in healthy individuals, particularly in young males, black individuals and athletes. ERP has also been associated with variants in seven genes and has been shown to run in families. With a relatively high prevalence of ERP in the general population, this pattern was previously thought to be benign. Dr. Antzelevitch and his team were the first to provide evidence that not all ERP are benign and that some can lead to life-threatening arrhythmias.

“The challenge ahead is to determine who among those presenting with ERP are truly at risk, and provide early intervention,” Dr. Antzelevitch said.

BrS causes a disruption of the heart's normal rhythm, leading to irregular heartbeats in the heart's lower chambers; if untreated, it can cause fainting, seizures, difficulty breathing or sudden death. It has been associated with variants in 18 genes. The mean age at the time of cardiac arrest in Brugada patients is approximately 40 years, and the majority develops symptoms between 20 and 65 years of age, although it can appear in the first few weeks of life in rare cases, contributing to SIDS. The prevalence of BrS worldwide is .05 percent, but it occurs more frequently in people of Southeast Asian descent. It is also nearly 10 times more common in men than women.

J-wave syndromes task force methodology

Task force members were selected to include professionals involved with the medical care of patients with J-wave syndromes, as well as those involved in research into the mechanisms underlying these conditions. Task force members undertook a comprehensive review of the literature on J-wave syndromes. They evaluated methods of diagnosis, risk stratification, approaches to therapy and mechanistic insights, including assessment of the risk/benefit ratio. The level of evidence found and the strength of their recommendations regarding particular management options were weighed and graded to complete the report.

The expert consensus document has received endorsement from heart care associations from around the world, including the Heart Rhythm Society, the Europe-based Heart Rhythm Association, the Asia Pacific Heart Rhythm Society and Sociedad Latino Americana de Estimulación Cardíaca y Electrofisiologia, which serves Latin American nations.

About Lankenau Institute for Medical Research

Lankenau Institute for Medical Research (LIMR) is a nonprofit biomedical research institute located on the campus of Lankenau Medical Center and is part of Main Line Health. Founded in 1927, LIMR’s mission is to improve human health and well-being. Faculty and staff are devoted to advancing innovative new approaches to formidable medical challenges, including cancer, cardiovascular disease, gastrointestinal disorders and autoimmune diseases, such as diabetes and arthritis. LIMR’s principal investigators conduct basic, preclinical and translational research, using their findings to explore ways to improve disease detection, diagnosis, treatment and prevention. They are committed to extending the boundaries of human health through technology transfer and training of the next generation of scientists and physicians.

About Lankenau Heart Institute

The Lankenau Heart Institute is Main Line Health’s premier, comprehensive cardiovascular medicine and surgery program. The Lankenau Heart Institute brings together the clinical expertise of all four Main Line Health acute care hospitals and community cardiology practices to ensure that patients receive a level of quality, service, and experience that is unprecedented in the region. Through the system-wide coordination of services, the Lankenau Heart Institute delivers preventive, diagnostic, therapeutic, and rehabilitative cardiovascular services at each of our locations including Lankenau Medical Center, Bryn Mawr Hospital, Paoli Hospital and Riddle Hospital.

Lankenau Heart Institute continues to be a pioneer in the use of beating-heart techniques and robotic-assisted procedures for coronary artery revascularization, minimally invasive and transcatheter approaches for valve repair and replacement, and complex aortic surgeries. With our growing experience and focus on minimally invasive techniques, Lankenau Heart Institute has expanded participation in clinical trials year over year. Our physicians are frequently invited to participate in clinical/medical device trials, many of these designed to facilitate minimally invasive procedures/approaches.

With a collaborative team of expert consultative cardiologists, interventional cardiologists, electrophysiologists, cardiovascular surgeons and specially trained nurses and technologists, the Lankenau Heart Institute is dedicated to managing and treating patients with heart failure, aortic disease, coronary and peripheral vascular disease, heart rhythm disorders and valve disease. Our team of cardiologists and cardiac specialists provide patients and their families with expert cardiac care, close to home.

About Main Line Health

Founded in 1985,Main Line Health is a not-for-profit health system serving portions of Philadelphia and its western suburbs. Main Line Health’s commitment—to deliver advanced medicine to treat and cure disease while also playing an important role in prevention and disease management as well as training physicians and other health care providers—reflects our intent to keep our community and ourselves well ahead. A team of more than 10,000 employees and 2,000 physicians care for patients throughout the Main Line Health system.

At Main Line Health’s core are four of the region’s most respected acute care hospitals—Lankenau Medical Center, Bryn Mawr Hospital, Paoli Hospital and Riddle Hospital—as well as one of the nation’s recognized facilities for rehabilitative medicine, Bryn Mawr Rehabilitation Hospital.

Main Line Health also includes Mirmont Treatment Center for drug and alcohol recovery; Main Line Health HomeCare & Hospice, which includes skilled home health care, hospice and home infusion services; Main Line Health Centers, primary and specialty care, lab and radiology, and other outpatient services located in Broomall, Collegeville, Concordville, Exton, King of Prussia and Newtown Square; Lankenau Institute for Medical Research, a biomedical research organization; and Main Line HealthCare,one of the region’s largest multispecialty physician networks.

Main Line Health is the recipient of numerous awards for quality care and service, including System Magnet® designation, the nation’s highest distinction for nursing excellence and the Mid-Atlantic Alliance for Performance Excellence (MAAPE) Excellence Award. Main Line Health is committed to creating an environment of diversity, respect and inclusion and has proudly embraced the American Hospital Association’s #123forEquity Pledge to Act to eliminate disparities in care. We are dedicated to advancing patient-centered care, education and research to help our community stay healthy.