BACKGROUND: Imaging evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains challenging. Myocardial strain assessment by echocardiography is an increasingly utilized technique for detecting subclinical left ventricular (LV) and right ventricular (RV) dysfunction. We aimed to evaluate the diagnostic and prognostic utility of LV and RV strain in ARVC. METHODS: Patients with suspected ARVC (n = 109) from a multicenter registry were clinically phenotyped using the 2010 ARVC Revised Task Force Criteria and underwent baseline strain echocardiography. Diagnostic performance of LV and RV strain was evaluated using the area under the receiver operating characteristic curve analysis against the 2010 ARVC Revised Task Force Criteria, and the prognostic value was assessed using the Kaplan-Meier analysis. RESULTS: Mean age was 45.3±14.7 years, and 48% of patients were female. Estimation of RV strain was feasible in 99/109 (91%), and LV strain was feasible in 85/109 (78%) patients. ARVC prevalence by 2010 ARVC Revised Task Force Criteria is 91/109 (83%) and 83/99 (84%) in those with RV strain measurements. RV global longitudinal strain and RV free wall strain had diagnostic area under the receiver operating characteristic curve of 0.76 and 0.77, respectively (both P <0.001; difference NS). Abnormal RV global longitudinal strain phenotype (RV global longitudinal strain > −17.9%) and RV free wall strain phenotype (RV free wall strain > −21.2%) were identified in 41/69 (59%) and 56/69 (81%) of subjects, respectively, who were not identified by conventional echocardiographic criteria but still met the overall 2010 ARVC Revised Task Force Criteria for ARVC. LV global longitudinal strain did not add diagnostic value but was prognostic for composite end points of death, heart transplantation, or ventricular arrhythmia (log-rank P =0.04). CONCLUSIONS: In a prospective, multicenter registry of ARVC, RV strain assessment added diagnostic value to current echocardiographic criteria by identifying patients who are missed by current echocardiographic criteria yet still fulfill the diagnosis of ARVC. LV strain, by contrast, did not add incremental diagnostic value but was prognostic for identification of high-risk patients.

Excellence in recording and interpretation of electrocardiogram (ECG) is a necessity for optimal electrocardiography. This includes data to properly interpret the ECG, including data on age, gender, cardiovascular diagnosis, medications, abnormal laboratory findings (eg, data on electrolytes), and the indications for the electrocardiogram. The ECG needs to be performed by a qualified technician and interpreted by an experienced physician.

This case report describes the electrocardiographic findings of a man in his 40s with palpitations lasting more than 30 hours, no prior symptoms, and a wide complex tachycardia with a rate of 180 beats/min.

Electrocardiographic (ECG) changes during head-up tilt have been previously reported in patients undergoing tilttable testing.For instance, in a large study including 150 patients with dissimilar diagnoses [including but not limited to orthostatic hypotension, vasovagal syncope, and postural tachycardia syndrome (POTS)], 135 had some ECG changes, most of them T-wave changes in inferior leads II, III and aVF and in anterolateral leads V3-V6.There was no evidence of myocardial ischemia in any of these patients.The relevance of these changes was unclear, but it was hypothesized that these alterations may be related to changes in the autonomic tone [1].More recently, we reported that, among a sample of 180 patients, ST-segment changes during head-up tilt were found in 32% [2].Of these, 28 (87.5%) were women averaging 41 years of age.Since none of these women had a clinical history suggestive of coronary disease, it was postulated that these ST-segment changes were not due to ischemic heart disease.Still, these ECG abnormalities during head-up tilt remained largely unexplained.In this issue of Clinical Autonomic Research, Vajapey and colleagues [3] confirm the relative high frequency of ST-segment changes during head-up tilt in patients with POTS.They studied an impressive sample of 255 patients with POTS who underwent both tilt-table testing and cardiac stress testing.Of these 255 patients, 45 had ST-segment changes or depression on head-up tilt.Forty-eight (91%) were women, with an average age of 36 years.However, when evaluated with cardiac stress testing, none of these patients with ST-segment depression during head-up tilt had ischemic changes.This is a major contribution to the literature, thus clarifying in a systematic way that ECG changes

Domenico Corrado *, Peter J. van Tintelen, William J. McKenna, Richard N.W. Hauer, Aris Anastastakis, Angeliki Asimaki, Cristina Basso, Barbara Bauce, Corinna Brunckhorst, Chiara Bucciarelli-Ducci, Firat Duru, Perry Elliott, Robert M. Hamilton, Kristina H. Haugaa, Cynthia A. James, Daniel Judge, Mark S. Link, Francis E. Marchlinski, Andrea Mazzanti, Luisa Mestroni, Antonis Pantazis, Antonio Pelliccia, Martina Perazzolo Marra, Kalliopi Pilichou, Pyotr G.A. Platonov, Alexandros Protonotarios,

Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

Arrhythmogenic cardiomyopathy (ACM) is an inherited arrhythmia syndrome characterized by severe structural and electrical cardiac phenotypes, including myocardial fibrofatty replacement and sudden cardiac death. Clinical management of ACM is largely palliative, owing to an absence of therapies that target its underlying pathophysiology, which stems partially from our limited insight into the condition. Following identification of deceased ACM probands possessing ANK2 rare variants and evidence of ankyrin-B loss of function on cardiac tissue analysis, an ANK2 mouse model was found to develop dramatic structural abnormalities reflective of human ACM, including biventricular dilation, reduced ejection fraction, cardiac fibrosis, and premature death. Desmosomal structure and function appeared preserved in diseased human and murine specimens in the presence of markedly abnormal β-catenin expression and patterning, leading to identification of a previously unknown interaction between ankyrin-B and β-catenin. A pharmacological activator of the WNT/β-catenin pathway, SB-216763, successfully prevented and partially reversed the murine ACM phenotypes. Our findings introduce what we believe to be a new pathway for ACM, a role of ankyrin-B in cardiac structure and signaling, a molecular link between ankyrin-B and β-catenin, and evidence for targeted activation of the WNT/β-catenin pathway as a potential treatment for this disease.

Domenico Corrado *, Peter J. van Tintelen, William J. McKenna, Richard N.W. Hauer, Aris Anastastakis, Angeliki Asimaki, Cristina Basso, Barbara Bauce, Corinna Brunckhorst, Chiara Bucciarelli-Ducci, Firat Duru, Perry Elliott, Robert M. Hamilton, Kristina H. Haugaa, Cynthia A. James, Daniel Judge, Mark S. Link, Francis E. Marchlinski, Andrea Mazzanti, Luisa Mestroni, Antonis Pantazis, Antonio Pelliccia, Martina Perazzolo Marra, Kalliopi Pilichou, Pyotr G.A. Platonov, Alexandros Protonotarios,

I first observed the treatment of arrhythmias by catheter ablation in Paris when I took a sabbatical in 1979–1980 under the supervision of Dr Guy Fontaine. At that time, catheter ablation was performed using high-energy direct current (DC) shock (fulguration) by inserting a catheter localized to the site of ventricular arrhythmias. Fulguration was limited to areas of the thick myocardium to avoid perforation. Therefore, only ablation of the atrioventricular (AV) node, septal accessory pathways and left ventricular tachycardia could be performed. This technique is not free of risks since it is associated with a large amount of electric current within a short period of time as well as barotrauma.

The epsilon wave of the electrocardiogram (ECG) together with fragmented QRS (fQRS), the terminal conduction delay, incomplete right bundle branch block (IRBBB) and complete/advanced RBBB (CRBBB) of peripheral origin are part of a spectrum of ventricular depolarization abnormalities of arrhythmogenic cardiomyopathy (AC). Although the epsilon wave is considered a major diagnostic criterion for AC since 2010 (AC Task Force Criteria), its diagnostic value is limited because it is a sign of the later stage of the disease. It would be more appropriate to say that the epsilon wave is a "hallmark" of AC, but is of low diagnostic sensitivity. Although the epsilon wave has high specificity for AC, it can be present in other pathological conditions. In this update we will cover the nomenclature, association with disease states and electrocardiographic aspects of the epsilon wave.

Arrhythmogenic cardiomyopathy is a genetic disease that is manifested clinically with ventricular arrhythmias between the ages of 20 and 50. There is no single ‘standard’ for the diagnosis that needs to be based on a combination of electrical structural and genetic abnormalities. Two-dimensional echocardiography is the usual imaging modality that is preferred in patients suspected of the disease, but cardiovascular magnetic resonance has the advantage of quantitative assessment of right and left ventricular function, as well as being more precise in evaluating cardiac wall motion abnormalities. Other imaging modalities, such as three-dimensional echocardiography and cardiac computed tomography scanning, are promising diagnostic techniques in identifying those individuals who present with dominant left ventricular involvement.

Objective Changes in heart rate variability (HRV) associated with breathing (respiratory sinus arrhythmia) are known to be parasympathetically (vagally) mediated when the breathing rate is within the typical frequency range (9-24 breaths per minute [bpm]; high-frequency HRV). Slow yogic breathing occurs at rates below this range and increases low-frequency HRV power, which may additionally reflect a significant sympathetic component. Yogic breathing techniques are hypothesized to confer health benefits by increasing cardiac vagal control, but increases in low-frequency HRV power cannot unambiguously distinguish sympathetic from parasympathetic contributions. The aim of this study was to investigate the autonomic origins of changes in low-frequency HRV power due to slow-paced breathing. Methods Six healthy young adults completed slow-paced breathing with a cadence derived from yogic breathing patterns. The paced breathing took place under conditions of sympathetic blockade, parasympathetic (vagal) blockade, and placebo. HRV spectral power was compared under 11 breathing rates during each session, in counterbalanced order with frequencies spanning the low-frequency range (4-9 bpm). Results HRV power across the low-frequency range (4-9 bpm) was nearly eliminated (p = .016) by parasympathetic blockade (mean (SD) spectral power at breathing frequency = 4.1 (2.1)) compared with placebo (69.5 (8.1)). In contrast, spectral power during sympathetic blockade 70.2 (9.1) and placebo (69.5 (8.1)) was statistically indistinguishable (p = .671). Conclusions These findings clarify the interpretation of changes in HRV that occur during slow-paced breathing by showing that changes in low-frequency power under these conditions are almost entirely vagally mediated. Slow-paced breathing is an effective tool for cardiac vagal activation.

Objectives: To verify accurate placement of the precordial ECG leads by identifying the 4th and 5th intercostal spaces as a function of the length of the sternum. This should decrease the percentage of lead misplacement leading to misdiagnoses. Methods: The population consisted of patients and healthy volunteers. The proposed method compared palpation of the 4th and 5th intercostal spaces to a percentile of the sternal length. Location of the 4th and 5th intercostal space using a simple device was evaluated to assist in proper placement of the precordial leads to obtain accurate diagnosis. Results: The location of the 4th and 5th intercostal space is related to the length of the sternum. It is 77% of the sternal length that measures 15 cm for the 4th intercostal space. The position of the VI and V2 electrodes decreases to 57% when the sternal length is 26 cm. Similar data was obtained to locate the 5th intercostal space with proper position of V4-V6 electrodes. Tables are provided to facilitate this process. An instrument was designed to measure the 4th and 5th intercostal space as a function of the sternal length. Conclusions: The location of the 4th and 5th intercostal space is identified based on the length of the sternum. (C) 2017 Elsevier Inc. All rights reserved.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by the patchy replacement of myocardium by fatty or fibrofatty tissue. These changes lead to structural abnormalities including right ventricular enlargement and wall motion abnormalities that can be detected by echocardiography, angiography, and cine MRI. ARVC/D is a genetically heterogeneous disorder, since it has been linked to several chromosomal loci. Myocarditis may also be a contributing etiological factor. Patients are typically diagnosed during adolescence or young adulthood. Presenting symptoms are generally related to ventricular arrhythmias. Concern for the risk of sudden cardiac death may lead to the implantation of an intracardiac defibrillator. An ongoing multicenter international registry should further our understanding of this disease.

In the late 1950s and early 1960s, the co-authors of this manuscript were in the early stages of their medical residency and cardiology fellowship training and there were only 4 US-based cardiovascular journals published in that era (Circulation, Circulation Research, American Heart Journal, and American Journal of Cardiology), and 6 high-quality US-based general medical/scientific journals that frequently published cardiovascular-related articles (New England Journal of Medicine, JAMA, Science, American Journal of Physiology, Journal of Clinical Investigation, and American Journal of Medicine) (Table). At that time it was feasible to read and keep up with the important cardiovascular literature. During the subsequent 60 years there has been a rapid advance in diagnostic and therapeutic cardiovascular medicine, including antihypertensive therapy, thrombolytic therapy, echocardiography, nuclear cardiology, antilipid medications, percutaneous dilatation and stenting of acute and chronic obstructive coronary lesions, coronary bypass graft surgery, implantable cardiac defibrillators, cardiac resynchronization therapy, ablation of arrhythmogenic substrate, and human genetics–to highlight some of the important contributions to patient care. During this same period of time there was augmented National Institutes of Health and corporate funding for basic and clinical research. Currently, there are 25 cardiovascular-related journals, mostly published on a monthly basis (Table), plus many hundreds of online, Web-based, open-access journals and articles. It is now impossible to keep abreast of the published literature even in one's area of special interest and expertise.TableUnited States-Based Journals That Publish Cardiovascular ArticlesJournal (Society Sponsor)Journal PublisherFirst PublishedPublication FrequencyImpact Factor in 2014A. Established, high-quality general medical/scientific journals that frequently publish cardiovascular articles New England Journal of Medicine (Massachusetts Medical Society)NEJM Group1812Weekly55.873 Journal of the American Medical Association (American Medical Association [AMA])AMA1883Weekly35.289 JAMA-CardiologyAMA2016Monthlyna Science (American Association for the Advancement of Science [AAAS])AAAS1880Weekly33.611 American Journal of Physiology (American Physiological Society [APS]APS1898Monthly4.857 AJP- Heart and Circulatory PhysiologyAPS19772×/mo3.881 Journal of Clinical Investigation (American Society for Clinical Investigation [ASCI])ASCI1924Monthly13.261 American Journal of Medicine (Alliance of Academic Internal Medicine)Elsevier1946Monthly5.610B. Cardiovascular journals sponsored by a society Journal of the American College of Cardiology [JACC] (America College of Cardiology [ACC])ACC1983Monthly16.503 JACC: Basic to Translational Science (open access)ACC2016Monthlyna JACC: Clinical ElectrophysiologyACC20156×/yna JACC: Cardiovascular ImagingACC2008Monthly7.188 JACC: Cardiovascular InterventionsACC20082×/month7.345 JACC: Heart FailureACC2013Monthlyna Circulation (American Heart Association [AHA])Lippincott Williams & Wilkins (LW&W)1950Monthly15.013 Circulation: Heart FailureLW&W2008Monthly6.567 Circulation: Cardiovascular InterventionsLW&W2008Monthly6.324 Circulation: Cardiovascular Quality and OutcomeLW&W2008Monthly5.906 Circulation: Arrhythmia and ElectrophysiologyLW&W2008Monthly4.678 Circulation: Cardiovascular GeneticsLW&W2008Monthly4.631 Circulation: Cardiovascular ImagingLW&W2008Monthly5.555 Circulation Research (AHA and Council on Basic Cardiovascular Sciences)LW&W19532x/month11.091 Heart Rhythm (Heart Rhythm Society and the Cardiac Electrophysiology Society)Elsevier2004Monthly5.076 Journal of Cardiopulmonary Rehabilitation and Prevention (American Association of Cardiovascular and Pulmonary Rehabilitation)Wolters Kluwer19806×/y1.583 Journal of Electrocardiology (International Society for Computerized Electrocardiology and the Society of Electrocardiology)Elsevier19686×/y1.363 Annals of Noninvasive Electrocardiology (International Society of Holter and Noninvasive Electrocardiology)Wiley19966×/y1.131C. Cardiovascular journals not sponsored by a society American Heart JournalElsevier1926Monthly4.464 American Journal of CardiologyElsevier195824×/y3.154 Journal of Cardiovascular ElectrophysiologyWiley1983Monthly3.475 Current Opinion in CardiologyWolters Kluwer19856×/y2.696 Journal of Cardiovascular PharmacologyWolters Kluwer1979Monthly2.111 Blood Pressure MonitoringLW&W19986×/y1.531 Clinical Pathways In CardiologyWolters Kluwer2002Every third monthNoneD. Online, open-access cardiovascular journals∗See text for commentary, because there are innumerable online open-access cardiovascular journals.AHA = American Heart Association; AMA = American Medical Association; NEJM = New England Journal of Medicine; na = not available.∗ See text for commentary, because there are innumerable online open-access cardiovascular journals. Open table in a new tab AHA = American Heart Association; AMA = American Medical Association; NEJM = New England Journal of Medicine; na = not available. There are several ways of evaluating the quality of currently published cardiovascular journals and articles. The journals published by a recognized professional society (eg, American Heart Association, American College of Cardiology, American Physiological Society, and Heart Rhythm Society) bear a stamp of approval from each society with multiple expert peer reviewers of each submitted article. Several of these journals have been expanded by the recent addition of cardiovascular sub-journals: 5 by the Journal of the American College of Cardiology and 6 by Circulation (Table). These ancillary sub-journals were introduced because the parent journals reviewed more quality manuscripts than they could publish. Some non-society-sponsored journals are of high quality. The question is how does one evaluate the quality of the cardiovascular journals and the published articles in these journals? A relatively quantitative, well-appreciated measure of journal quality was developed by Eugene Garfield, the founder of the Institute of Scientific Information, with his introduction of the Impact Factor beginning in 1972.1Garfield E. Citation analysis as a tool in journal evaluation.Science. 1972; 178: 471-479Crossref PubMed Scopus (1887) Google Scholar As one can see in the Table, journals range from the highest impact factor value of 55.873 to journals with impact factors < 2.0. The impact factor of a journal is the average number of citations received per paper published in that journal during a 2-year period. The impact factor has its limitations, and it has been misused, with some journals publishing a large number of review articles that are frequently cited more than research reports. Editorials that reference published articles in the same journal can also increase the impact factor of a journal. In 2007 the European Association of Science Editors issued an official statement recommending “that journal impact factors are used only—and cautiously—for measuring and comparing the influence of entire journals, but not for the assessment of single papers, and certainly not for the assessment of researchers or research programs either directly or as a surrogate.”2European Association of Science Editors (EASE). Available at: http://www.ease.org.uk/sites/default/files/ease_statement_ifs_final.pdf. 2007. Accessed March 4, 2016.Google Scholar Overall, the impact factor has been helpful in roughly prioritizing the relative quality of the spectrum of published medical journals. There is a large number of online, low-quality, open-access cardiovascular-related journals, and the sources of these publications have been referred to as “predatory sites.”3Beall J. Predatory publishers are corrupting open access.Nature. 2012; 489: 179Crossref PubMed Scopus (497) Google Scholar Many of these journals do not have an impact factor, and in those that do, the impact factor is usually in the very low range. As of March 2015, the Directory of Open Access Journals database contained records for 10,000 online, open-access journals.4Adams C. (5 March 2015). SPARC* Directory of Open Access Journals introduces new standards to help community address quality concerns. Available at: http://sparcopen.org/news/2015/directory-of-open-access-journals-introduces-new-standards-to-help-community-address-quality-concerns/. Accessed April 14, 2015.Google Scholar Approximately 4 new online, open-access journals were added to the database each day in 2012, with larger daily numbers added in subsequent years. Currently, it is estimated that there are about 700 online, open-access cardiovascular publications on the Web. Cardiovascular clinicians and researchers receive many e-mails per day requesting submission of an article for one of the online, open-access, Web-based journals. The typical e-mail request indicates a broad array of topics for submission, including research articles, reviews, short communication, case reports, surveys, commentaries, essays, and editorials, with 2-week “reviews” and quick, online open-access publication on the Internet for a charge. It is estimated that 25% of open-access journals are predators.5Caplan A.L. The problem of publication-pollution denialism.Mayo Clin Proc. 2015; 90: 565-566Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar, 6Moher D. Moher E. Stop predatory publishers now: act collaboratively.Ann Intern Med. 2016; 164: 616-617Crossref PubMed Scopus (51) Google Scholar One open-access, Web-based journal that is not a predator is the Public Library of Science (PLOS), a nonprofit group publishing reviewed scientific articles since 2003 (impact factor of 3.234) for a charge of $1325. PLOS is not a cardiovascular journal, but it is an example of quality publishing that can be done on the Web with proper organization, structure, and leadership. Almost all of the published journals listed in the Table require a subscription to have access to printed articles. Most libraries at hospitals, medical schools, and universities provide free, open access to the major published journals for staff. Not all clinicians and researchers have institutional access to the journals, and new journal procedures have recently been introduced. For example, the Journal of the American College of Cardiology (JACC) sub-journal JACC: Basic to Translational Science, first published in 2016 (Table), is an online, open-access journal with high-level, peer-reviewed articles. For Web publication, there is a $4000 to $4200 publication charge to the primary author; but no costs to individual readers. Many of the standard cardiovascular journals offer the author of a published, printed article the option of also publishing it as an open-access article on the Internet for a significant charge, as high as $3000. Why would one choose to publish in an open-access, Web-based journal without the journal having a publishing history? Most of the predatory, open-access, Web-based, nonestablished journals are funded by financial charges to authors. In addition, the peer review process is frequently nonexistent, with articles that would not be accepted by standard journals. These are changing times in cardiovascular publications. We predict that the established cardiovascular journals and other specialty journals will move progressively to online publications with increased open access for interested clinicians and investigators. The charge to the author for on-line publication in the established journals is likely to be reduced over time because additional funding should be available through Web-based advertisements. The quality of the Web-based articles published by the established societies and those with a historical background should remain high in view of the peer-review process that will surely continue. We believe that more of the established journals will be progressively placed on the Web in the future.

Introduction: We have observed electrocardiographic (ECG) changes primarily in women during tilt table testing. Methods: We reviewed 12 lead ECGs during tilt studies between 2012 and 2016 for changes in ST segments and T waves during tilt table testing. Patients with distinctly abnormal baseline ECGs were excluded. Results: Of the 180 tilt studies, 117 (65%) were in women. There were 32 patients with ECG changes during tilting. Of these, 28 (87.5%) were in women with an average age of 45 years. None had a history of CAD or exertional chest pain. Echocardiograms were available in 21 of the 28 women with tilt induced ECG changes and all were normal. ECG changes during tilt table testing were found in 4/64 (6.25%) of men. The occurrence of ST-T wave changes during tilt testing was significantly higher among women compared to men, with a p value of 0.008. Of the 28 women with ECG changes during tilt, 11 had T wave inversions alone. ST segment depression alone was noted in 7 women. There were 10 women who had both ST segment depression and T wave inversions. Changes occurred immediately upon tilting in 6. In the remaining, they occurred at an average of 4.8 +/- 4 min after tilting. The slight increase in heart rate in patients with ECG changes was similar to that in the patients without new ECG changes. The ECG changes were not related to the presence of syncope. Conclusions: ECG changes during the testing was observed at a relatively high incidence primarily in women. The clinical significance of these repolarization changes during tilt testing is unknown. These ECG changes during tilt testing may correlate with the high incidence of false positive ECGs in women during exercise testing but do not necessarily indicate the presence of ischemic coronary disease. Additional research is needed to explain this phenomenon. (C) 2017 Elsevier Inc. All rights reserved.