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News Archive 2 - September 18, 2006 through May 27, 2007

By MICHELINE LONG
Published: May 27, 2007

ANNOUNCEMENT:

RESULTS FROM THE NORTH AMERICAN MULTIDISCIPLINARY STUDY OF ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY

Enrollment in this very important study extended from 2002 to 2007. Thanks to a webcast which has arrived since the completion of the Heart Rhythm Society's 2007 Scientific Session, you can view a report on the results of this study. Click here to view this valuable slide and sound presentation.

Our thanks goes to everyone who contributed to the value of this study; dedicated researchers, participating patients and family members, and the NIH.

By MICHELINE LONG
Published: May 18, 2007

BREAKING NEWS:

CHARACTERISTIC ECG PATTERNS ARE PRESENT IN THE MAJORITY OF ARVD PATIENTS AND CAN BE USED TO PREDICT THE DISEASE

In 2006, Dr. Li Zhang presented the abstract, "Are Epsilon Waves in ARVD 'the Right Precordial Leads only' Phenomena?" (Li Zhang, MD; Andrés Ricardo Pérez Riera, MD; Jieling Pu, MD, PhD et al.; CARDIOSTIM Europace 2006;8 Suppl265/4.) In 2007, she is presenting yet another abstract at the Heart Rhythm Society's Scientific Session in Colorado, U.S.

Presentation title: A Spectrum of ECG Abnormalities in Patients with Arrhythmogenic Right Ventricular Dysplasia: Can ECG Predict the Disease?

Author block:

Li Zhang, MD, Guy H. Fontaine, MD, PhD, Wojciech Zareba, MD, PhD, Bortolo Martini, MD, Micheline Long, No Degree, Catherine Stephan, BS, Jielin Pu, MD, PhD, Dayi Hu, MD, Wenling Liu, MD, PhD, Jeffrey A. Towbin, MD, Steven J. Compton, MD, Jeffrey L. Anderson, MD and G. Michael Vincent, MD. LDS Hospital, Intermountain Healthcare and Univ. of Utah School of Medicine, Salt Lake City, UT, Hospital La Pitie-Salpetriere, Paris, France, Univ. of Rochester Medical Center, Rochester, NY, Boldrini Hospital, Thiene, Italy, International ARVD Family Support Network, Palmdale, CA, Fu Wai Cardiovascular Hospital, Beijing, China, People's Hospital of Peking Univ., Beijing, China, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, Alaska Heart Clinic, Anchorage, AK

"1) The prevalence of common ECG abnormalities in this cohort of ARVD patients are notched, peaked, wide or flat P waves (50%), 1st degree AVB (19%), CRBBB (27%), right end conduction delay (21%), reduced QRS amplitude 6 leads (66%), QRS squiggles 3 leads (62%), QRS in Rr' or Ss' (50%), S-terminal prolongation in V1-2 (50%), QRS duration V1-2>V5-6 (54%), epsilon waves (30%), ST elevation (25%) or depression (17%), inverted T waves in V1-2 or beyond (74%), left QRS axis (22%), and RV premature beats/tachycardia (26%) with QRS notches (86%) and the reduced amplitude(89%).

2) The accuracy of ARVD prediction by ECG patterns was measured as sensitivity (85%), specificity (97%), positive and negative predictive values of 98% and 82%, respectively."

"Characteristic ECG patterns are present in the majority of ARVD patients and can be used to predict the disease. This approach is simple, cost effective and highly reproducible for ARVD screening purposes. Recognizing ARVD-related ECG abnormalities will improve clinical diagnosis and help identify presymptomatic patients and affected family members for the purpose of SD prevention especially in young athletes."

By MICHELINE LONG
Published: May 18, 2007

FOR THE RECORD:

NON-INDUCIBLE IN THE EP LAB YET STILL AT HIGH RISK OF SD WITHOUT AN ICD

How valuable are the results of Electrophysiology lab (EP lab) "induciblility" tests? Should they be used to determine which ARVD patient is "not" at the risk of developing a lethal arrhythmia outside of the lab? Should they be used to decide who does or does not receive the lifesaving benefit of an ICD? Research reports appear to indicate, "No!" on both counts. The results of several studies have provided information which calls into question the prognostic significance of EP inducibility testing in ARVD diagnosed patients.

ARVD patients have been sharing their "non-inducibility" experiences for over 8 years. One man, non-inducible in the lab, asked his doctor for the permission to prove his ventricular tachycardia (VT.) In his hospital room and on telemetry, the man performed a few Taekwondo moves and immediately broke into VT. A young woman hopped on an exercise bike, a quick and inexpensive solution to stimulating a life threatening arrhythmia outside the lab.

"Well," you say, "There you go, those people were performing a strenuous exercise." Correct, but that was not the case for a yet undiagnosed patient. A medical professional, this young woman was merely performing non-strenuous job duties when she felt herself go into a fast heart rhythm. Retreating to a room with an EKG machine, she and a doctor witnessed and recorded her ventricular tachycardia. At the time, this woman was on a medication to prevent arrhythmias. Later, prior to going into the EP lab, she had ceased all medications for several days. Even so, VT could not be triggered by programmed ventricular stimulation (PVS.) Fortunately for the latter woman, her risk had already been documented on paper.

One of the long known hallmarks of ARVD is that, despite the best medication treatments, diagnosed patients can break through their "meds" and go into VT and/or ventricular fibrillation (VFib.) In 1982, the article, "Ventricular Dysplasia: A Report of 24 Adult Cases" (Circulation, Vol 65, 384-398; FI Marcus, GH Fontaine, G Guiraudon et al.) stated:

"Of the 22 patients referred for recurrent ventricular tachycardia, 10 could be treated medically; antiarrhythmic drug therapy, alone or in combination, was not effective in 12 patients and they had surgical treatment."

"Twenty-nine of these patients experienced a cardiac arrest. Eighteen patients (62%) were involved in routine activity, 9 (31%) were involved in active exercise, 1 (3.5%) was pregnant, and 1 (3.5%) was in bed at the time of death."

The "myth of non-inducibility in ARVD patients indicating a lack of risk for sudden death" has been debunked. The truth in this crucial matter is now known.

In 2003, the authors of, "Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia;" (Circulation. 2003;108:3084-3091; Domenico Corrado MD, PhD, Loira Leoni MD, Mark S. Link MD et al.) wrote:

"Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device."

Also:

"Of 98 patients who were inducible at programmed ventricular stimulation, 50 (51%) did not experience ICD therapy during the follow-up, whereas 7 (54%) of 13 noninducible patients had ICD interventions. Overall, the positive predictive value of programmed ventricular stimulation was 49%, the negative predictive value was 54%, and the test accuracy was 49%. The incidence of appropriate ICD discharge did not differ between patients who were or were not inducible at programmed ventricular stimulation, regardless of clinical presentation (Table 4). The type of ventricular tachyarrhythmia inducible at the time of electrophysiological study did not predict the occurrence of ventricular fibrillation/flutter during follow-up."

A recent presentation at the Heart Rhythm Society's 2007 Scientific Session also spoke to debunk the myth.

Presentation Title: Non-inducibility of ventricular tachycardia does not predict low likelihood of appropriate therapy in patients with ARVD.

Authors: Adam S. Budzikowski, MD, PhD, James P. Daubert, MD, Henry W. Sesselberg, MD et al.

Researchers had hypothesized that "VT inducibility at PVS can be used to stratify patients for future arrhythmic events and provide a guide to ICD therapy." A study of 67 ICD implanted ARVD diagnosed patients seems to have disproven the hypothesis:

"Non-inducible ARVD patients had a high: (26% at 1 year and 39% at 2 years) risk of arrhythmic events."

and

"Conclusions: Non-inducible ARVD patients with ICD have a high risk of arrhythmic events, thus questioning the prognostic significance of EP inducibility testing in these patients."

By MICHELINE LONG
Published: May 11, 2007

IN BRIEF:

IPODS INTERFERING WITH THE TELEMETRY OF IMPLANTABLE PACEMAKERS

A presentation at this year's Heart Rhythm Society's Scientific Session reminded those with implantable heart devices to take caution. Electromagnetic interference with implanted pacemakers and defibrillators can cause these devices to malfunction.

Presentation Title: Pacemaker Interference with iPod MP3 Players
Authors: Jay P. Thaker, Mehul B. Patel, MD, Haiyan Li, MD et al.

The presentation explained a study which evaluated "interactions between single and dual-chamber pacemakers (P) and 4 models of iPods (Third generation (3G), Photo, Video and Nano; all devices manufactured prior to 2006)." The conclusions of the study were that researchers found "interference in 50% of patients."

According to a Reuters news article written by Debra Sherman, "IPods Said to Cause Pacemaker Interference:"

"Electrical interference was detected half of the time when the iPod was held just 2 inches from the patient's chest for 5 to 10 seconds."

and

"In some cases, the iPods caused interference when held 18 inches from the chest. Interfering with the telemetry equipment caused the device to misread the heart's pacing and in one case caused the pacemaker to stop functioning altogether."

By MICHELINE LONG
Published: January 8, 2007

ANNOUNCEMENT:

"HEALING HEARTS" ANNOUNCES 2ND ANNUAL BULL & OYSTER ROAST

I mentioned to my husband that Healing Hearts would be holding a fund raising event in Maryland soon, I wondered how we could work together with them. As I told him the story of how Healing Hearts was formed, and that which they had done and were doing (good news travels fast), I couldn't keep the tears from welling in my eyes. Softy that he is, my husband said, "Let's go!" One problem ... I don't fly anymore and we live in California!

Struggling to keep my composure so that I could continue speaking, I further mentioned that HH was selling raffle tickets for the event. The Grand Prize would be $1000.00 and ticket owners would not need to be present to win. "Good enough, let's get raffle tickets, and if we win that Grand Prize, it will go straight to support ARVD," he responded. If we win the iPod, we have 3 grandkids who will be thrilled, and we will have contributed to a good cause!

YES! There is a solution to joining those of Healing Hearts who are doing such a good work. Even though they are not in our local neighborhood, they are near enough to join.

Join those working to raise funds for ARVD research, and enjoy the company of others who are doing the same! "Healing Hearts" of Maryland has announced their 2nd annual Bull & Oyster Roast. All proceeds from the event will be gifted to support the great work being done by the ARVD Program at the Johns Hopkins Heart Institute.

This evening event will be held on February 16th, 2007, at Michaels Eighth Avenue in Glen Burnie, Maryland. The night will include dinner, dancing, and a silent auction (auction items to include O.C. condo weekend, Longaberger baskets, and sports memorabilia.) Raffle tickets are being sold (prizes include a $1,000 Grand Prize, iPod, and dining certificates.)

If you are not local to the event, do you have friends or family who are? Can you treat them to event dinner tickets, a shot at the raffle prizes?

If you would like to purchase event or raffle tickets to support this very worthy cause, you can contact Shannon Murr at 410-916-5322. Additionally, you can read more about Healing Hearts at their website.

ARVD-ARVC-Info.com honors the memory of Bonnie Milner who died from ARVD at the very young age of 26 years old. We hope that you will too, by supporting this event.

By MICHELINE LONG
Published: October 18, 2006

ANNOUNCEMENT:

JOHNS HOPKINS ARVD FAMILY SEMINAR DATE SET AND SPEAKER ANNOUNCED

Crystal Tichnell has announced that the yearly Johns Hopkins ARVD Family Seminar will be held on May 5th, 2007. Dr. William (Bill) McKenna of The Heart Hospital in London will be the guest speaker. Active in ARVD research for many years, Dr. McKenna, his group and Dr. Nikos Protonotarios were responsible for identifying the first ARVD gene mutation (Naxos ARVD.) This gene identification sprung the doors of ARVD genetic research wide open. Stay tuned to this page to learn more as we do. Follow the "Upcoming Events" page.

PRESS RELEASE
Published: September 18, 2006

Johns Hopkins Medicine
Media Relations and Public Affairs
Media contact: David March
410 955 1534; dmarch1@jhmi.edu

YES, DOCTOR, IT CAN BE DONE: MRIs MADE SAFE FOR PEOPLE WITH MODERN DEFIBRILLATORS AND PACEMAKERS

Low-energy fix for machine, other steps vastly reduce risk

Click here to view a brief video of "A satisfactory image from MRI of a patient's heart with an implanted defibrillator (the dark void at top)."

Researchers at Johns Hopkins have painstakingly figured out how to safely perform magnetic resonance imaging (MRI) scans on men and women who have any one of 24 modern types of implanted defibrillators and pacemakers.

Henry Halperin, M.D., and his team have developed a combination of methods that reduce the risk of life-threatening meltdowns and other complications posed by MRI's ability to charge and manipulate the electrical properties of cells to produce real-time images from inside the body.

"We have turned a once exceptional procedure into one that is now a routine at Hopkins," Halperin says.

Among other things, the Hopkins group reprograms the devices, fixing them to a specific sequence. This makes the implanted devices "blind" to their external environment, reducing the potential for their electronics to confuse the radiofrequency generated by the MRI with an irregular heartbeat and preventing misfires. They also turn off the defibrillators' shocking function for the brief duration of the MRI scan, about 30 to 60 minutes.

Also changed is the amount of electrical energy used at peak scanning in MRI. The Hopkins team reduced the strength of the electromagnetic field by half, from as much as 4 watts per kilogram to 2 watts per kilogram per patient.

"This lower-energy scan still provided images of sufficient quality to make an accurate diagnosis in more than 90 percent of cases tested," Halperin said in an article published in the Sept. 18 issue of the journal Circulation, reporting on 55 of more than 100 patients scanned at Hopkins so far.

Their report comes just two years after the same journal published the team's initial, positive findings in animals, stirring fierce debate at several international conferences as to whether or not MRI could truly be made safe.

Since 2004, the Hopkins team says its expanded use of MRI has made more than a dozen potentially life-saving diagnoses, despite the fact that the tiny, battery-driven heart devices, which help the body's main pump maintain a beat, have long been considered unsafe and off limits for testing.

"The risk to patients of burning heart tissue or misfiring is still there," Halperin cautions. "But our results show that with appropriate precautions, MRI is a safe and effective diagnostic tool to use for those with modern implanted heart devices." An electro-physiologist and professor of medicine, radiology and biomedical engineering at The Johns Hopkins University School of Medicine and its Heart Institute, Halperin has long led efforts to expand access to MRI.

Except for research purposes, the U.S. Food and Drug Administration has not authorized any implanted cardiac device for MRI testing. But Halperin says opening up this diagnostic option is important for the estimated 2 million Americans, many of them elderly, who have these implanted devices but who are also denied the benefits of the quick and accurate images that MRI provides.

"Once these precautions are better understood and further refined, we hope policy makers will see fit to review current restrictions on scanning anyone with a device," says lead author Saman Nazarian, M.D., a cardiac electrophysiology, clinical and research fellow at Hopkins.

"These images are critical to early diagnosis of certain cancers of the brain, head and neck, and to guide invasive procedures," he notes.

Of those scanned in the study, 31 had a pacemaker and 24 had an implantable defibrillator. Only modern devices - pacemakers made after 1996 and defrillators manufactured after 2000 - were tested, Nazarian says, because the latest models were deemed to be safer than older versions. Newer models are made of titanium, a non-magnetic metal, he points out, and they are smaller and more lightweight and have better protection from the radiofrequency energy of the MRI scanner.

Using a single scanner, a 1.5 Tesla by General Electric, the Hopkins group was able to help plan artery-opening procedures for more than a half-dozen in the test group; improve measurements of tumor growth in nine others; and detect two strokes, a benign brain mass and a blood clot in the spine that had been missed by alternative imaging from CT scanning.

Nazarian cautions that only physicians specially trained in MRI safety, or with access to specialists familiar with the specific precautions taken in his study, should undertake this approach. "It is also important at this time to restrict MRI use to those with implanted devices specifically tested, and scanners of the same type and magnetic strength as that used in our study," he adds.

All study participants were closely monitored during the scans with electrocardiography and pulse oximetry, and staff were on hand to resuscitate patients in the event of an emergency.

All subjects were over age 19 and were followed from three months to six months to look for any post-test heart damage or changes in the devices' programming.

Patients were disqualified from testing if they had any leads placed on the surface of the heart or leads that were capped with metal, and therefore not connected to the battery and at greater risk of overheating. Leads are the electrical components connecting the device to the heart muscle, and when the leads are attached to the battery or embedded in the blood vessels, they are less likely to be overheated by the MRI field.

An analysis of records showed that scans provided definitive answers to physicians' diagnostic questions 100 percent of the time for conditions affecting areas outside the chest, and 93 percent of the time for conditions that affected the heart and the upper body. In the latter category, the remaining 7 percent of the scans were too distorted by imaging artifacts from the implanted devices to make a clear diagnosis.

Device monitoring showed that lead sensing did not fluctuate or change during the scan. Battery measurements showed that scans did not deplete or strengthen the battery's charge. Indeed, pacemakers and defibrillators performed successfully after the scans without any premature firing or false alarms.

Pacemakers and defibrillators are implantable devices used to treat people with an abnormal heartbeat, a condition known as arrhythmia. More than 2.2 million Americans are living with arrhythmia. It can occur in a healthy heart and be of minimal consequence, or it can lead to more serious heart disease, stroke or sudden cardiac death.

Funding for this study was provided by the National Institutes of Health, and the Donald W. Reynolds Foundation. Halperin is a paid consultant to defibrillator manufacturer Medtronic, and co-investigators Ronald Berger, M.D., Ph.D., and Albert Lardo, Ph.D., are paid consultants to Guidant Corp., another device manufacturer. Co-author David Bluemke, M.D., Ph.D., has also received honoraria from General Electric Health Care for presentations. None of these companies provided funding for the study, and the terms of the physicians' arrangements are managed by The Johns Hopkins University in accordance with its conflict of interest policies.

Other investigators in this research, conducted solely at Johns Hopkins, were Ariel Roguin, M.D., Ph.D.; Menekhem Zviman, Ph.D.; Timm Dickfeld, M.D., Ph.D.; Robert G. Weiss, M.D.; and Hugh Calkins, M.D.

A complete listing of heart devices made safe for MRI scanning is provided below.

- JHM -

LIST OF TESTED IMPLANTED CARDIAC DEVICES

Pacemakers with Satisfactory MRI Testing

Manufacturer: St Jude
1. Pacesetter AFP (262)
2. Trilogy (2360)
3. Entity (5326)
4. Affinity (5130, 5330)
5. Integrity (5142, 5342, 5346)
6. Identity (5172, 5370, 5376, 5380, 5386)

Manufacturer: Guidant
7. Vigor (1232)
8. Discovery (1272)
9. Insignia (1194, 1290)

Manufacturer: Medtronic
10. EnPulse (AT-500, E2SRO1, E2DRO1)
11. Kappa (701, 901)
12. Prodigy (7860)
13. In Sync BiV (8040, 8042)

Defibrillators with Satisfactory MRI Testing

Manufacturer: St Jude
14. Photon (V-194, V-230, V-232)
15. Atlas (V-240)
16. Epic (V-197, V-235, V-239)

Manufacturer: Guidant
17. Prizm (1850, 1851, 1852, 1860, 1861)
18. Contak (1823, H119, H170, H175)
19. Vitality (T125, T135)

Manufacturer: Medtronic
20. Maximo (7232)
21. Gem-II (7273)
22. Gem-III (7275)
23. Marquis (7274)
24. InSync (7272)