Skip to main content
  • American Heart Association
  • Science Volunteer
  • Warning Signs
  • Advanced Search
  • Donate

  • Home
  • About this Journal
    • Editorial Board
    • General Statistics
    • Circulation Doodle
      • Doodle Gallery
      • Circulation Cover Doodle
        • → Blip the Doodle
    • Information for Advertisers
    • Author Reprints
    • Commercial Reprints
    • Customer Service and Ordering Information
    • Subscribe to AHA Journals
  • All Issues
  • Subjects
    • All Subjects
    • Arrhythmia and Electrophysiology
    • Basic, Translational, and Clinical Research
    • Critical Care and Resuscitation
    • Epidemiology, Lifestyle, and Prevention
    • Genetics
    • Heart Failure and Cardiac Disease
    • Hypertension
    • Imaging and Diagnostic Testing
    • Intervention, Surgery, Transplantation
    • Quality and Outcomes
    • Stroke
    • Vascular Disease
  • Browse Features
    • AHA Guidelines and Statements
      • Recently Published Guidelines
    • Bridging Disciplines
    • → Articles Bridging Discplines
    • Go Red for Women Issue
    • Cardiovascular Case Series
    • Circulation at ESC
    • Circulation at AHA Scientific Sessions
    • Global Impact of the 2017 ACC/AHA Hypertension Guidelines
    • Circulation at ACC 2018
    • Circulation Supplements
    • ECG Challenge
    • Hospitals of History
      • Brigham and Women's Hospital
      • Hartford Hospital
      • Hospital Santa Maria del Popolo, Naples, Italy
      • Instituto do Coração-INCOR (São Paulo, Brasil)
      • Minneapolis City Hospital
      • Parkland Hospital: Dallas, Texas
      • Pennsylvania Hospital, Philadelphia
      • Pitié-Salpêtrière Hospital
      • Royal Infirmary of Edinburgh, Scotland
      • Tufts Medical Center
      • University of Michigan
      • Uppsala University Hospital
      • Vassar Brothers Medical Center (Poughkeepsie, NY)
      • Wroclaw Medical University
      • Women's College Hospital, Toronto, Canada
      • Henry Ford Hospital, Detroit, Michigan
      • Instituto Nacional de Cardiología Ignacio Chávez – INCICh México City, México
      • Kuang-Tien General Hospital (Taichug, Taiwan)
    • On My Mind
    • Podcast Archive
    • → Subscribe to Circulation on the Run
    • →Circulation FIT Podcast 2018
    • → #FITFAVs
  • Resources
    • Instructions for Authors
      • Accepted Manuscripts
      • Revised Manuscripts
    • → Article Types
    • → General Preparation Instructions
    • → Research Guidelines
    • → How to Submit a Manuscript
    • Journal Policies
    • Permissions and Rights Q&A
    • Submission Sites
    • Circulation CME
    • AHA Journals RSS Feeds
    • International Users
    • AHA Newsroom
  • AHA Journals
    • AHA Journals Home
    • Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB)
    • Circulation
    • → Circ: Arrhythmia and Electrophysiology
    • → Circ: Genomic and Precision Medicine
    • → Circ: Cardiovascular Imaging
    • → Circ: Cardiovascular Interventions
    • → Circ: Cardiovascular Quality & Outcomes
    • → Circ: Heart Failure
    • Circulation Research
    • Hypertension
    • Stroke
    • Journal of the American Heart Association
  • Facebook
  • Twitter

  • My alerts
  • Sign In
  • Join

  • Advanced search

Header Publisher Menu

  • American Heart Association
  • Science Volunteer
  • Warning Signs
  • Advanced Search
  • Donate

Circulation

  • My alerts
  • Sign In
  • Join

  • Facebook
  • Twitter
  • Home
  • About this Journal
    • Editorial Board
    • General Statistics
    • Circulation Doodle
    • Information for Advertisers
    • Author Reprints
    • Commercial Reprints
    • Customer Service and Ordering Information
    • Subscribe to AHA Journals
  • All Issues
  • Subjects
    • All Subjects
    • Arrhythmia and Electrophysiology
    • Basic, Translational, and Clinical Research
    • Critical Care and Resuscitation
    • Epidemiology, Lifestyle, and Prevention
    • Genetics
    • Heart Failure and Cardiac Disease
    • Hypertension
    • Imaging and Diagnostic Testing
    • Intervention, Surgery, Transplantation
    • Quality and Outcomes
    • Stroke
    • Vascular Disease
  • Browse Features
    • AHA Guidelines and Statements
    • Bridging Disciplines
    • → Articles Bridging Discplines
    • Go Red for Women Issue
    • Cardiovascular Case Series
    • Circulation at ESC
    • Circulation at AHA Scientific Sessions
    • Global Impact of the 2017 ACC/AHA Hypertension Guidelines
    • Circulation at ACC 2018
    • Circulation Supplements
    • ECG Challenge
    • Hospitals of History
    • On My Mind
    • Podcast Archive
    • → Subscribe to Circulation on the Run
    • →Circulation FIT Podcast 2018
    • → #FITFAVs
  • Resources
    • Instructions for Authors
    • → Article Types
    • → General Preparation Instructions
    • → Research Guidelines
    • → How to Submit a Manuscript
    • Journal Policies
    • Permissions and Rights Q&A
    • Submission Sites
    • Circulation CME
    • AHA Journals RSS Feeds
    • International Users
    • AHA Newsroom
  • AHA Journals
    • AHA Journals Home
    • Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB)
    • Circulation
    • → Circ: Arrhythmia and Electrophysiology
    • → Circ: Genomic and Precision Medicine
    • → Circ: Cardiovascular Imaging
    • → Circ: Cardiovascular Interventions
    • → Circ: Cardiovascular Quality & Outcomes
    • → Circ: Heart Failure
    • Circulation Research
    • Hypertension
    • Stroke
    • Journal of the American Heart Association
Correspondence

Response to Letters Regarding Article, “Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia”

Rahul Jain, Darshan Dalal, Amy Daly, Crystal Tichnell, Cynthia James, Ariana Evenson, Rohit Jain, Theodore Abraham, Boon Yew Tan, Hari Tandri, Stuart D. Russell, Daniel Judge, Hugh Calkins
Download PDF
https://doi.org/10.1161/CIRCULATIONAHA.109.929158
Circulation. 2010;121:e406
Originally published May 17, 2010
Rahul Jain
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Darshan Dalal
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Amy Daly
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Crystal Tichnell
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Cynthia James
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ariana Evenson
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rohit Jain
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Theodore Abraham
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Boon Yew Tan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hari Tandri
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Stuart D. Russell
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Daniel Judge
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hugh Calkins
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
  • eLetters

Jump to

  • Article
    • Acknowledgments
    • References
  • Info & Metrics
  • eLetters
Loading

We are grateful for the 2 thoughtful letters concerning our recent article1 on the ECG features of arrhythmogenic right ventricular dysfunction (ARVD). Because ECGs are widely available and inexpensive, it important for those interested in this field to fully explore the diagnostic and prognostic potential of 12-lead ECGs. There were 3 main issues raised in these letters.

The first concerned whether our control population was appropriate. As noted in our article, our study included 2 control populations. The first was a series of 27 patients who were evaluated in the ARVD clinic because of a first- or second-degree relative with ARVD. Each of these patients underwent a comprehensive evaluation to screen for ARVD, including an ECG, signal-averaged ECG, Holter, magnetic resonance imaging, and stress testing. The second control population was a series of 30 patients with a right bundle-branch block ECG pattern who did not have relatives with ARVD and were not being evaluated in our ARVD clinic. Although the point that ARVD is an age-dependent disease is correct, it does not diminish the fact that at the time of our ARVD evaluation, our control patients had no clinical evidence of ARVD. The results of the study by Kies et al,2 although referenced in the letter, are not applicable to our control patient population. In our control population, each patient had a normal baseline 12-lead ECG and no other evidence of ARVD. Of the 12 patients described by Kies et al in whom a diagnosis of ARVD appeared over time, 11 had baseline ECG abnormalities, and each had ventricular arrhythmias with a left bundle-branch block pattern. Current guidelines advise that screening for ARVD in first-degree relatives be undertaken every 2 to 3 years beginning at puberty. This recommendation addresses the very concern raised about the important relationship between age and the onset of clinical manifestations of ARVD. A negative initial screening evaluation of a first-degree relative of a patient with ARVD cannot exclude the development of the clinical features and associated risks of ARVD 5 to 10 years later.

The second concern focused on the ARVD patient population enrolled in the Johns Hopkins ARVD Registry. It is correct that the Johns Hopkins ARVD Registry follows both newly diagnosed patients with ARVD and patients with established ARVD who contact the Johns Hopkins ARVD Program and agree to participate in our registry. As pointed out, this differs from the North American ARVD Registry, which enrolled only patients with newly diagnosed ARVD. This difference likely explains some of the differences in the ECG findings in these 2 studies. However, it is important to note that among the 108 probands whose ECG characteristics were reported by Marcus et al,3 only 74 were phenotyped as affected with ARVD. The ECG features of this subset of the patient population who in facto were diagnosed with ARVD are not presented separately in the article. Therefore, the distribution of ECG findings reported in the letter is of only limited relevance to this discussion.

The third issue raised in these 2 letters concerned the role of low QRS voltage as a diagnostic criterion for ARVD. Although as the authors point out the QRS voltage of the ARVD ECGs included as the Figure in our article is lower than in our control populations, in our experience, low QRS voltage is a fairly nonspecific finding that can result from a large variety of cardiovascular diseases. We did not specifically focus on QRS voltage in our study because of this fact and because it has never been previously identified as an ECG marker for ARVD. On the basis of the study by Steriotis et al,4 further research is needed on the sensitivity and specificity of QRS voltage as an ECG marker for ARVD. The final issue raised in these letters draws attention to the fact that right precordial T-wave inversion is age dependent and can be seen in 5% of males and females 12 to 18 years of age.5 This important point needs to be considered when children are screened for ARVD. Our analysis was limited to adults >18 years of age.

Although we agree that our study has limitations and that the results need to be confirmed in future prospective clinical trials, we feel strongly that the results of our study remain valid and should be of clinical value to those screening patients with ARVD. We also want to highlight the fact that our study is the first to address the important, and previously ignored, issue of how to analyze ECGs for ARVD in patients with a complete or incomplete right bundle-branch block pattern. We look forward to continuing to work collaboratively with all of the researchers around the world who are interested in clinical investigation of this important disease.

Acknowledgments

Disclosures

None.

References

  1. ↵
    Jain R, Dalal D, Daly A, Tichnell C, James C, Evenson A, Jain R, Abraham T, Yew Tan B, Tandri H, Russell SD, Judge D, Calkins H. Electrocardiographic features of arrhythmogenic right ventricular dysplasia. Circulation. 2009; 120: 477–487.
    OpenUrlAbstract/FREE Full Text
  2. ↵
    Kies P, Bootsma M, Bax JJ, Zeppenfeld K, Van Erven L, Wijffels MC, Van Der Wall EE, Schalij MJ. Serial reevaluation for ARD/C is indicated in patients presenting with left bundle branch block ventricular tachycardia and minor ECG abnormalities. J Cardiovasc Electrophysiol. 2006; 17: 586–593.
    OpenUrlCrossRefPubMed
  3. ↵
    Marcus FI, Zareba W, Calkins H, Towbin JA, Basso C, Bluemke DA, Estes NA III, Picard MH, Sanborn D, Thiene G, Wichter T, Cannom D, Wilber DJ, Scheinman M, Duff H, Daubert J, Talajic M, Krahn A, Sweeney M, Garan H, Sakaguchi S, Lerman BB, Kerr C, Kron J, Steinberg JS, Sherrill D, Gear K, Brown M, Severski P, Polonsky S, McNitt S. Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: results from the North American Multidisciplinary Study. Heart Rhythm. 2009; 6: 984–992.
    OpenUrlCrossRefPubMed
  4. ↵
    Steriotis AK, Bauce B, Daliento L, Rigato I, Mazzotti E, Folino A, Marra MP, Brugnara L, Nava A. Electrocardiographic pattern in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol. 2009; 103: 1302–1308.
    OpenUrlCrossRefPubMed
  5. ↵
    Marcus FI. Prevalence of T wave inversion beyond V1 in young normal individuals and usefulness for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Am J Cardiol. 2005; 95: 1070–1071.
    OpenUrlCrossRefPubMed
View Abstract
Back to top
Previous ArticleNext Article

This Issue

Circulation
May 18, 2010, Volume 121, Issue 19
  • Table of Contents
Previous ArticleNext Article

Jump to

  • Article
    • Acknowledgments
    • References
  • Info & Metrics
  • eLetters

Article Tools

  • Print
  • Citation Tools
    Response to Letters Regarding Article, “Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia”
    Rahul Jain, Darshan Dalal, Amy Daly, Crystal Tichnell, Cynthia James, Ariana Evenson, Rohit Jain, Theodore Abraham, Boon Yew Tan, Hari Tandri, Stuart D. Russell, Daniel Judge and Hugh Calkins
    Circulation. 2010;121:e406, originally published May 17, 2010
    https://doi.org/10.1161/CIRCULATIONAHA.109.929158

    Citation Manager Formats

    • BibTeX
    • Bookends
    • EasyBib
    • EndNote (tagged)
    • EndNote 8 (xml)
    • Medlars
    • Mendeley
    • Papers
    • RefWorks Tagged
    • Ref Manager
    • RIS
    • Zotero
  • Article Alerts
    Log in to Email Alerts with your email address.
  • Save to my folders

Share this Article

  • Email

    Thank you for your interest in spreading the word on Circulation.

    NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

    Enter multiple addresses on separate lines or separate them with commas.
    Response to Letters Regarding Article, “Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia”
    (Your Name) has sent you a message from Circulation
    (Your Name) thought you would like to see the Circulation web site.
  • Share on Social Media
    Response to Letters Regarding Article, “Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia”
    Rahul Jain, Darshan Dalal, Amy Daly, Crystal Tichnell, Cynthia James, Ariana Evenson, Rohit Jain, Theodore Abraham, Boon Yew Tan, Hari Tandri, Stuart D. Russell, Daniel Judge and Hugh Calkins
    Circulation. 2010;121:e406, originally published May 17, 2010
    https://doi.org/10.1161/CIRCULATIONAHA.109.929158
    del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo

Related Articles

Cited By...

Subjects

  • Heart Failure and Cardiac Disease
    • Heart Failure
  • Imaging and Diagnostic Testing
    • Electrocardiology (ECG)

Circulation

  • About Circulation
  • Instructions for Authors
  • Circulation CME
  • Statements and Guidelines
  • Meeting Abstracts
  • Permissions
  • Journal Policies
  • Email Alerts
  • Open Access Information
  • AHA Journals RSS
  • AHA Newsroom

Editorial Office Address:
200 Fifth Avenue, Suite 1020
Waltham, MA 02451
email: circ@circulationjournal.org
 

Information for:
  • Advertisers
  • Subscribers
  • Subscriber Help
  • Institutions / Librarians
  • Institutional Subscriptions FAQ
  • International Users
American Heart Association Learn and Live
National Center
7272 Greenville Ave.
Dallas, TX 75231

Customer Service

  • 1-800-AHA-USA-1
  • 1-800-242-8721
  • Local Info
  • Contact Us

About Us

Our mission is to build healthier lives, free of cardiovascular diseases and stroke. That single purpose drives all we do. The need for our work is beyond question. Find Out More about the American Heart Association

  • Careers
  • SHOP
  • Latest Heart and Stroke News
  • AHA/ASA Media Newsroom

Our Sites

  • American Heart Association
  • American Stroke Association
  • For Professionals
  • More Sites

Take Action

  • Advocate
  • Donate
  • Planned Giving
  • Volunteer

Online Communities

  • AFib Support
  • Garden Community
  • Patient Support Network
  • Professional Online Network

Follow Us:

  • Follow Circulation on Twitter
  • Visit Circulation on Facebook
  • Follow Circulation on Google Plus
  • Follow Circulation on Instagram
  • Follow Circulation on Pinterest
  • Follow Circulation on YouTube
  • Rss Feeds
  • Privacy Policy
  • Copyright
  • Ethics Policy
  • Conflict of Interest Policy
  • Linking Policy
  • Diversity
  • Careers

©2018 American Heart Association, Inc. All rights reserved. Unauthorized use prohibited. The American Heart Association is a qualified 501(c)(3) tax-exempt organization.
*Red Dress™ DHHS, Go Red™ AHA; National Wear Red Day ® is a registered trademark.

  • PUTTING PATIENTS FIRST National Health Council Standards of Excellence Certification Program
  • BBB Accredited Charity
  • Comodo Secured