Thanks go to Dr. Frank Marcus who provided us with information for the following progress reports. Dr. Marcus is an ARVD expert/doctor/researcher and Professor Emeritus at the University of Arizona, Sarver Heart Center, Tucson, AZ. He is also the Principal Investigator of the NIH "Multidisciplinary Study of Right Ventricular Dysplasia." Additionally, Dr. Marcus initiated and has been directing the course toward a long overdue modification of the Task Force Criteria (TFC) for the Diagnosis of ARVD.

Progress Report: Modification of the TFC for the Diagnosis of ARVD
In mid 2007, our site reported on a conference which was held to consider modifications of the TFC for the Diagnosis of ARVD. A subsequent meeting was held on November 3, 2007, in Orlando, Florida, U.S. Within the following progress report, you will read reference to the very notable representatives who attended the meeting (listed in alphabetical order):

Dr. Cristina Basso, Italy Dr. Hugh Calkins, US Dr. Domenico Corrado, Italy Dr. Moniek Cox, Netherlands Dr. James Daubert, US Dr. Guy Fontaine, France Research RN Kathy Gear Dr. Richard Hauer, Netherlands

Dr. Frank Marcus, US Dr. William McKenna, UK Dr. Gaetano Thiene, Italy Dr. Jeffrey Saffitz, US Dr. Jon Steinberg, US Dr. Hari Tandri, US Dr. Jeffrey Towbin, US Dr. Wojciech Zareba, US

Querying Dr. Marcus about the present state of affairs, he wrote to me:

"The meeting in Orlando was held the morning of Nov 3. Attending were Dr. Bill McKenna from UK, from Italy Drs. Corrado, Basso and Thiene, from the Netherlands Drs. Hauer and Cox, and Dr. Fontaine from Paris. The US representatives include Dr. Saffitz from Harvard, Drs. Tandri and Calkins from Hopkins, Dr. Towbin from Houston, Drs. Daubert and Zareba from Rochester NY, Dr. Steinberg from NY, Dr. Picard from Boston and Kathy Gear and myself.

We reviewed our preliminary report from the meeting at the Heart Rhythm Society in May. Various presentations were made that were stimulated by the previous meeting including the best parameters to measure the delay in RV depolarization and best cutoff value for number of PVCs per 24 hours to distinguish normals from ARVD patients. Dr. Picard received data from the Padua group on several hundred normal echos that will be used to distinguish ARVD patients. Bill McKenna gave Dr. Steinberg data on several hundred normal signal Averaged ECGs.

As you can see, we are gathering a database of normal values for several tests used for the diagnosis of ARVD. From this information, the statistician will be able to determine quantitative values to distinguish normals from ARVD patiens. This information was previously unavailable Following the meeting Dr. McKenna wrote a draft of the manuscript that will serve as the basis for publication of the Task Force modification criteria.

I met with Duane Sherrill, statistician last week to discuss the various issues. Next Monday we will have a conference call to include Dave Bluemke (MRI) , Mike Picard (ECHO) Bill MCKenna and I with the statistician to discuss how best to quantify the ECHO and MRI findings to distinguish them from normals.

As you can see it is a detailed and extensive process. We hope to finalize the recommendations by December and submit a manuscript for publication by JAN 1. We couldn't have made this progress without your support and that of your friends."

Progress Report: Manuscript, NIH Study of ARVD
Since the near completion of this study, Dr. Marcus and the many other excellent researchers who have been working alongside him have worked hard to create a manuscript reporting the study results. Prior to receiving the manuscript, we learned of a presentation available at the Heart Rhythm Society's (HRS) website. This presentation was made available at the time of the HRS May 2007 Scientific Session, noted in its list of Late-Breaking Clinical Trials II. You can view that presentation by clicking this link.

The manuscript on this study is imminent. About that, Dr. Marcus wrote:

"With regard to the main manuscript, it is progressing slowly. We decided to write two papers, one focusing on the diagnosis of ARVD; the other on the genetics."

By MICHELINE LONG
Published: October 5, 2007

ANNOUNCEMENT:

JOHNS HOPKINS 9TH ANNUAL ARVD FAMILY SEMINAR PLANNED FOR MAY 3RD, 2008

Set your calendars! Crystal Tichnell, program coordinator of the Johns Hopkins ARVD Program has announced that the next ARVD Family Seminar will be held on Saturday, May 3rd at Johns Hopkins Hospital in Baltimore, Maryland.

Stay tuned! We will post more conference details when we receive them.

By MICHELINE LONG
Published: July 24, 2007

ANNOUNCEMENT:

SUPPORT ARVD RESEARCH AND RECEIVE THE NEW ARVD BOOK

Greetings friends... Recently I was thumbing through my copy of the new book Arrhythmogenic RV Cardiomyopathy/Dysplasia - Recent Advances (editors Marcus, Frank I.; Nava, Andrea; Thiene, Gaetano). I was also taking great pleasure in reading the autograph of Dr. Marcus just within the front cover. This doctor/scientist has worked so hard for so many years, and continues to do so much for the ARVD community. What a pleasure it was to read this leading edge scientist's handwritten words to my husband and I. It was also humbling to read a "thanks" to us for supporting Dr. Marcus' ARVD research. "My goodness," I thought, "It is the least we can do for all that you have done. You have helped us, you have helped so many." I was reminded of how Dr. Marcus and Kathy Gear have been there with their knowledge and for our family for over 8 years and through some incredibly trying times. I was reminded of how many others have received the same care.

Thinking a while longer... (I'm always thinking, always looking for ways to stimulate donations for ARVD research)... I wrote to Dr. Marcus. I suggested that others might also like to receive an autographed copy of the new ARVD book. I proposed that if people would send a check of $300.00 or more to support his ARVD research, might they also be able to receive an autographed copy of the new book? Well, before the end of the day, my suggested proposal was arranged.

I have subsequently learned that the actual "cost" of this scholarly book is the same to everyone who buys it (i.e. even to the editors of the book.) So, if you send a check of $300.00, only that portion of the check which exceeds the cost of the book ($169.00, plus any applicable taxes, shipping/handling) will be tax deductible (in the U.S.) and go to ARVD research. My estimated figures show that that a $300.00 check will yield an approximate donation of around $100.00 to ARVD research. I love that! But can I tell you that I would love it even more if checks arrived as high over $300.00 as financially possible for those who send them? I realize that this isn't possible for everyone, but for those whom it is, I can only hope that they write their checks as high as possible.

My husband no longer has a diagnosis of ARVD. Even so, we cannot help but support ARVD research -- so many of our dear friends are affected by this diagnosis. Our own family was for years. We want to continue our support of ARVD research and ask that everyone who can partner with us will do so.

If you would like to donate to ARVD research and receive this new book autographed by its leading editor (Frank I. Marcus, MD, Professor, University of Arizona), here is how you can:

Write your $300.00 or more check payable to: UA Foundation/Sarver Heart Center
In the memo of the check write: ARVD Book & Gift to ARVD

Send your check to:
Frank I. Marcus, MD
C/Of UA Foundation/Sarver Heart Center
1501 N. Campbell Ave, Room 5153
Tucson, AZ 85724
USA

At this point, I would like to thank everyone who will join our family in supporting ARVD research with your financial donations.

By MICHELINE LONG
Published: July 19, 2007

BREAKING NEWS:

NEW BOOK
"ARRHYTHMOGENIC RV CARDIOMYOPATHY/DYSPLASIA - RECENT ADVANCES"

Newly published by Springer in 2007, I have just received and thumbed through this new book. The book is a "must have" for physicians, whether they are general internists, cardiologists, or electrophysiologists. In fact, although there may be some difficult bits for diagnosed patients to see or read within this book, those who like to stay informed about their disease may want to order and have it on hand.

The following is a note from Kathy Gear, research nurse working with Dr. Frank Marcus of the NIH Multidisciplinary Study on ARVD:

"A book that resulted from the Venice Arrhythmia Meeting of a top panel of scientists, both European and American, in Italy, October, 2005, and covering all aspects of ARVD/C, has just been published. This is the second monograph in 10 years on ARVD/C. The tremendous strides that have been made in the recognition and understanding of the disease are summarized in Arrhythmogenic RV Cardiomyopathy/Dyplasia: Recent Advances. (Marcus, Nava, Thiene [Eds] 2007 Springer.) It should be a useful reference for cardiologists and electrophysiologists. The scope of the book is broad and encompasses diagnosis, risk stratification and genetics."

If you happen to go to Amazon to purchase this book, you are likely to see a little confusing information in their "Customer Reviews" section. Amazon's "Product Details" sections for this new book shows "Publisher: Springer; 1 edition (June 27, 2007)." This is likely to be correct information. Unfortunately, Amazon seems to have attributed some old "Customer Reviews" (from an excellent, but previously published ARVD book i.e. in 1997) to the new book. If you go to Amazon looking to read customer reviews of this new book, make sure to check the date upon which the review was posted. Hopefully, Amazon will get this squared away soon.

Patients and family members... This book is not inexpensive, as books go. On the other hand, this is a scholarly reference book with some 200 pages of focussed and up to date information. Is it possible that your doctor would "prescribe" the purchase of this book to help you understand your diagnosis?

Considering the cost of the book, it doesn't appear to be much more than that of about 4 family dinners at a local restaurant. How might the information in this book benefit you and your family in your desire to be proactive in your care? Might showing a few family members some of the information and graphics within this book get them seriously considering the evaluation for ARVD that they have avoided?

Might the information in this book help to save a life?
YOU BET!

By MICHELINE LONG
Published: June 15, 2007

BREAKING NEWS:

MODIFICATION OF TASK FORCE CRITERIA FOR DIAGNOSIS OF ARVD/C EXPECTED BEFORE 2008

Have you been diagnosed with ARVD/C? If so, were the Task Force Criteria for the diagnosis of ARVD/C (TFC) used to determine your diagnosis? Well, that is very important! You see, before a disease can be appropriately treated, it must first be accurately diagnosed. An accurate diagnosis of ARVD/C is made by use of the TFC, but hold on ... there is more. The aging TFC is about ready to receive a much needed face lift.

Since 1994 physicians have been using a specific set of diagnostic criteria to assist them in diagnosing ARVD/C. The article, "Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia" (Indian Pacing Electrophysiol. J. 2003;3(3):148-156; Julia H. Indik, MD PhD and Frank I. Marcus, MD) explains why a diagnostic criteria was established for this disease and a little about it:

"Since there is no single 'gold standard' diagnostic test to reliably verify this condition, a combination of diagnostic criteria have been established by the Task Force of the Working Group on Myocardial and Pericardial Disease. Major criteria include demonstration of severe wall motion abnormalities, fibrofatty replacement by biopsy, epsilon waves and family history of histologically confirmed disease. Minor criteria include milder alterations of ventricular function, inverted T waves in the right precordial leads, late potentials, ventricular tachycardia with left bundle branch morphology or frequent premature ventricular complexes (>1000 PVCs in a 24 hour period), or family history of suspected or clinically diagnosed ARVC/D. A diagnosis can then be made if at least two major, 1 major and 2 minor, or 4 minor criteria from different categories are fulfilled."

Fortunately, some 13 years ago, a task force put their knowledge together to assist physicians in diagnosing people with ARVD/C. Also fortunately, knowledge has been gained since 1994.

In 2006, Dr. Frank Marcus (a leading ARVD/C researcher) envisioned the need for another task force meeting to consider the application of new knowledge to the aging TFC. The need was great. While the former TFC was very valuable, it deserved an update. Dr. Marcus laid out the reason and plans for a new task force meeting and submitted them to the U.S. NIH (National Institute of Health). The NIH received and supported the conference with a grant, but more funding would be necessary to accomplish this work. Efforts were made to gather the funding. The patient community, as well as the professional community joined the vision and gifted to support to a TFC modification conference. In October of 2006, Dr. Marcus announced that private contributions, as well as a generous grant from the International Society for Holter and Noninvasive Electrocardiology ((ISHNE) had arrived -- the budget necessary for planning the modification conference had been promised, planning could proceed.

In May of 2007, Dr. Marcus chaired the "Modification of the Task Force Criteria for Diagnosis of ARVD/C" conference. He wrote:

"The meeting was held in Denver, CO from May 12 - 14th with Dr. William McKenna as my cochairman. All the attendees said it was a great success. Assembled were the world experts on all aspects relating to the diagnosis of the disease including imaging experts in MRI, Echo and angiography, pathologists, geneticists and electrocardiographers. They came from Italy, France, the Netherlands, Japan, Argentina, England and of course the US. At the conclusion of the meeting we agreed that it was necessary and desirable to make many changes in the Task Force Criteria, published in 1994, on which to base the diagnosis. It will take some time to complete the changes since we need to examine several databases to be certain of normal values for the dimensions of the right heart, including differences between men and women. My tentative plan is to accumulate the necessary information, write a tentative draft and have several of us meet at the American Heart Association meetings in November to draft final recommendations. I plan to publish the document before the end of the year."

It appears that everything is in sync for a final draft of modification recommendations to be distributed to task force conferees in November 2007. If all continues to go well, the publication of the new, modified Task Force Criteria for the diagnosis of ARVD/C (TFC) will be published before the end of December 2007, before 2008.

Thank you Dr. Marcus, research nurse Kathy Gear RN, and all ARVD expert doctor/researcher who are working so hard to help those who deserve a diagnosis and treatment for ARVD/C.