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2. Flecainide Is Associated With a Lower Incidence of Arrhythmic Events in a Large Cohort of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.

Author

Bergeman, Auke, Lieve, Krystien, Kallas, Dania, Bos, J, Rosés I Noguer, Ferran, Denjoy, Isabelle, Zorio, Esther, Kammeraad, Janneke, Peltenburg, Puck, Tobert, Katie, Aiba, Takeshi, Atallah, Joseph, Drago, Fabrizio, Batra, Anjan, Brugada, Ramon, Borggrefe, Martin, Clur, Sally-Ann, Cox, Moniek, Davis, Andrew, Dhillon, Santokh, Etheridge, Susan, Fischbach, Peter, Franciosi, Sonia, Haugaa, Kristina, Horie, Minoru, Johnsrude, Christopher, Kane, Austin, Krause, Ulrich, Kwok, Sit-Yee, LaPage, Martin, Ohno, Seiko, Probst, Vincent, Roberts, Jason, Robyns, Tomas, Sacher, Frederic, Semsarian, Christopher, Skinner, Jonathan, Swan, Heikki, Tavacova, Terezia, Tisma-Dupanovic, Svjetlana, Tfelt-Hansen, Jacob, Yap, Sing-Chien, Kannankeril, Prince, Leenhardt, Antoine, Till, Janice, Sanatani, Shubhayan, Tanck, Michael, Ackerman, Michael, Wilde, Arthur, and van der Werf, Christian

Subjects
catecholaminergic polymorphic ventricular tachycardia, sudden cardiac death, ventricular arrhythmias, Female, Humans, Adolescent, Male, Flecainide, Incidence, Cross-Over Studies, Tachycardia, Ventricular, Adrenergic beta-Antagonists, Defibrillators, Implantable, Death, Sudden, Cardiac
Abstract

BACKGROUND: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia. METHODS: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy. The study period was defined as the period in which background therapy (ie, beta-blocker type [beta1-selective or nonselective]), left cardiac sympathetic denervation, and implantable cardioverter defibrillator treatment status, remained unchanged within individual patients and was divided into pre-flecainide and on-flecainide periods. The primary end point was AEs, defined as sudden cardiac death, sudden cardiac arrest, appropriate implantable cardioverter defibrillator shock, and arrhythmic syncope. The association of flecainide with AE rates was assessed using a generalized linear mixed model assuming negative binomial distribution and random effects for patients. RESULTS: A total of 247 patients (123 [50%] females; median age at start of flecainide, 18 years [interquartile range, 14-29]; median flecainide dose, 2.2 mg/kg per day [interquartile range, 1.7-3.1]) were included. At baseline, all patients used a beta-blocker, 70 (28%) had an implantable cardioverter defibrillator, and 21 (9%) had a left cardiac sympathetic denervation. During a median pre-flecainide follow-up of 2.1 years (interquartile range, 0.4-7.2), 41 patients (17%) experienced 58 AEs (annual event rate, 5.6%). During a median on-flecainide follow-up of 2.9 years (interquartile range, 1.0-6.0), 23 patients (9%) experienced 38 AEs (annual event rate, 4.0%). There were significantly fewer AEs after initiation of flecainide (incidence rate ratio, 0.55 [95% CI, 0.38-0.83]; P=0.007). Among patients who were symptomatic before diagnosis or during the pre-flecainide period (n=167), flecainide was associated with significantly fewer AEs (incidence rate ratio, 0.49 [95% CI, 0.31-0.77]; P=0.002). Among patients with ≥1 AE on beta-blocker therapy (n=41), adding flecainide was also associated with significantly fewer AEs (incidence rate ratio, 0.25 [95% CI, 0.14-0.45]; P

Published
2023

11. Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report

Author

Corrado, Domenico, Anastasakis, Aris, Basso, Cristina, Bauce, Barbara, Blomström-Lundqvist, Carina, Bucciarelli-Ducci, Chiara, Cipriani, Alberto, De Asmundis, Carlo, Gandjbakhch, Estelle, Jiménez-Jáimez, Juan, Kharlap, Maria, McKenna, William J, Monserrat, Lorenzo, Moon, James, Pantazis, Antonis, Pelliccia, Antonio, Perazzolo Marra, Martina, Pillichou, Kalliopi, Schulz-Menger, Jeanette, Jurcut, Ruxandra, Seferovic, Petar, Sharma, Sanjay, Tfelt-Hansen, Jacob, Thiene, Gaetano, Wichter, Thomas, Wilde, Arthur, and Zorzi, Alessandro

Published
2024
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13. An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

Author

Lamba, Avani, Roston, Thomas M., Peltenburg, Puck J., Kallas, Dania, Franciosi, Sonia, Lieve, Krystien V.V., Kannankeril, Prince J., Horie, Minoru, Ohno, Seiko, Brugada, Ramon, Aiba, Takeshi, Fischbach, Peter, Knight, Linda, Till, Jan, Kwok, Sit-Yee, Probst, Vincent, Backhoff, David, LaPage, Martin J., Batra, Anjan S., Drago, Fabrizio, Haugaa, Kristina, Krahn, Andrew D., Robyns, Tomas, Swan, Heikki, Tavacova, Terezia, van der Werf, Christian, Atallah, Joseph, Borggrefe, Martin, Rudic, Boris, Sarquella-Brugada, Georgia, Chorin, Ehud, Hill, Allison, Kammeraad, Janneke, Kamp, Anna, Law, Ian, Perry, James, Roberts, Jason D., Tisma-Dupanovic, Svjetlana, Semsarian, Christopher, Skinner, Jonathan R., Tfelt-Hansen, Jacob, Denjoy, Isabelle, Leenhardt, Antoine, Schwartz, Peter J., Ackerman, Michael J., Blom, Nico A., Wilde, Arthur A.M., and Sanatani, Shubhayan

Published
2024
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14. Exercise Capacity in Patients With Obstructive Hypertrophic Cardiomyopathy: SEQUOIA-HCM Baseline Characteristics and Study Design

Author

Coats, Caroline J., Maron, Martin S., Abraham, Theodore P., Olivotto, Iacopo, Lee, Matthew M.Y., Arad, Michael, Cardim, Nuno, Ma, Chang-Sheng, Choudhury, Lubna, Düngen, Hans-Dirk, Garcia-Pavia, Pablo, Hagège, Albert A., Lewis, Gregory D., Michels, Michelle, Oreziak, Artur, Owens, Anjali T., Tfelt-Hansen, Jacob, Veselka, Josef, Watkins, Hugh C., Heitner, Stephen B., Jacoby, Daniel L., Kupfer, Stuart, Malik, Fady I., Meng, Lisa, Wohltman, Amy, and Masri, Ahmad

Published
2024
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18. An International Multicenter Evaluation of Type 5 Long QT Syndrome

Author

Roberts, Jason D, Asaki, S Yukiko, Mazzanti, Andrea, Bos, J Martijn, Tuleta, Izabela, Muir, Alison R, Crotti, Lia, Krahn, Andrew D, Kutyifa, Valentina, Shoemaker, M Benjamin, Johnsrude, Christopher L, Aiba, Takeshi, Marcondes, Luciana, Baban, Anwar, Udupa, Sharmila, Dechert, Brynn, Fischbach, Peter, Knight, Linda M, Vittinghoff, Eric, Kukavica, Deni, Stallmeyer, Birgit, Giudicessi, John R, Spazzolini, Carla, Shimamoto, Keiko, Tadros, Rafik, Cadrin-Tourigny, Julia, Duff, Henry J, Simpson, Christopher S, Roston, Thomas M, Wijeyeratne, Yanushi D, El Hajjaji, Imane, Yousif, Maisoon D, Gula, Lorne J, Leong-Sit, Peter, Chavali, Nikhil, Landstrom, Andrew P, Marcus, Gregory M, Dittmann, Sven, Wilde, Arthur AM, Behr, Elijah R, Tfelt-Hansen, Jacob, Scheinman, Melvin M, Perez, Marco V, Kaski, Juan Pablo, Gow, Robert M, Drago, Fabrizio, Aziz, Peter F, Abrams, Dominic J, Gollob, Michael H, Skinner, Jonathan R, Shimizu, Wataru, Kaufman, Elizabeth S, Roden, Dan M, Zareba, Wojciech, Schwartz, Peter J, Schulze-Bahr, Eric, Etheridge, Susan P, Priori, Silvia G, and Ackerman, Michael J

Subjects
Genetics, Cardiovascular, Clinical Trials and Supportive Activities, Heart Disease, Clinical Research, Human Genome, 2.1 Biological and endogenous factors, Aetiology, Adolescent, Adult, Death, Sudden, Cardiac, Electric Countershock, Electrocardiography, Female, Heart Arrest, Humans, Long QT Syndrome, Male, Middle Aged, Penetrance, Potassium Channels, Voltage-Gated, Registries, arrhythmia, genetics, long QT syndrome, penetrance, sudden cardiac death, Cardiorespiratory Medicine and Haematology, Clinical Sciences, Public Health and Health Services, Cardiovascular System & Hematology
Abstract

BackgroundInsight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare KCNE1 variants implicated in LQT5 was sought through an international multicenter collaboration.MethodsPatients with either presumed autosomal dominant LQT5 (N = 229) or the recessive Type 2 Jervell and Lange-Nielsen syndrome (N = 19) were enrolled from 22 genetic arrhythmia clinics and 4 registries from 9 countries. KCNE1 variants were evaluated for ECG penetrance (defined as QTc >460 ms on presenting ECG) and genotype-phenotype segregation. Multivariable Cox regression was used to compare the associations between clinical and genetic variables with a composite primary outcome of definite arrhythmic events, including appropriate implantable cardioverter-defibrillator shocks, aborted cardiac arrest, and sudden cardiac death.ResultsA total of 32 distinct KCNE1 rare variants were identified in 89 probands and 140 genotype positive family members with presumed LQT5 and an additional 19 Type 2 Jervell and Lange-Nielsen syndrome patients. Among presumed LQT5 patients, the mean QTc on presenting ECG was significantly longer in probands (476.9±38.6 ms) compared with genotype positive family members (441.8±30.9 ms, P

Published
2020

21. GLP-1 increases heart rate by a direct action on the sinus node

Author

Lubberding, Anniek Frederike, primary, Veedfald, Simon, additional, Achter, Jonathan Samuel, additional, Nissen, Sarah Dalgas, additional, Soattin, Luca, additional, Sorrentino, Andrea, additional, Vega, Estefania Torres, additional, Linz, Benedikt, additional, Eggertsen, Caroline Harriet Eggert, additional, Mulvey, John, additional, Toräng, Signe, additional, Larsen, Sara Agnete, additional, Nissen, Anne, additional, Petersen, Lonnie Grove, additional, Bilir, Secil Erbil, additional, Bentzen, Bo Hjorth, additional, Rosenkilde, Mette Marie, additional, Hartmann, Bolette, additional, Lilleør, Thomas Nikolaj Bang, additional, Qazi, Saddiq, additional, Møller, Christian Holdflod, additional, Tfelt-Hansen, Jacob, additional, Sattler, Stefan Michael, additional, Jespersen, Thomas, additional, Holst, Jens Juul, additional, and Lundby, Alicia, additional

Published
2024
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22. Aficamten for Symptomatic Obstructive Hypertrophic Cardiomyopathy

Author

Maron, Martin S., primary, Masri, Ahmad, additional, Nassif, Michael E., additional, Barriales-Villa, Roberto, additional, Arad, Michael, additional, Cardim, Nuno, additional, Choudhury, Lubna, additional, Claggett, Brian, additional, Coats, Caroline J., additional, Düngen, Hans-Dirk, additional, Garcia-Pavia, Pablo, additional, Hagège, Albert A., additional, Januzzi, James L., additional, Lee, Matthew M.Y., additional, Lewis, Gregory D., additional, Ma, Chang-Sheng, additional, Michels, Michelle, additional, Olivotto, Iacopo, additional, Oreziak, Artur, additional, Owens, Anjali T., additional, Spertus, John A., additional, Solomon, Scott D., additional, Tfelt-Hansen, Jacob, additional, van Sinttruije, Marion, additional, Veselka, Josef, additional, Watkins, Hugh, additional, Jacoby, Daniel L., additional, Heitner, Stephen B., additional, Kupfer, Stuart, additional, Malik, Fady I., additional, Meng, Lisa, additional, Wohltman, Amy, additional, and Abraham, Theodore P., additional

Published
2024
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25. Sex Differences in the Prognosis of Non-Sustained Ventricular Tachycardia Detected on Holter Recording

Author

Christiansen, Philip Bonde, primary, Larsen, Bjørn Strøier, additional, Hadad, Rakin, additional, Nielsen, Olav Wendelboe, additional, Dominguez Vall-Lamora, Maria Helena, additional, Prescott, Eva, additional, Galatius, Søren, additional, Rasmusen, Hanne Kruuse, additional, Davidsen, Ulla, additional, Karlsen, Finn Michael, additional, Højberg, Søren, additional, Bang, Casper N., additional, Schramm, Tina Ken, additional, Tfelt-Hansen, Jacob, additional, and Sajadieh, Ahmad, additional

Published
2024
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26. A comprehensive evaluation of the genetic architecture of sudden cardiac arrest

Author

Ashar, Foram N, Mitchell, Rebecca N, Albert, Christine M, Newton-Cheh, Christopher, Brody, Jennifer A, Müller-Nurasyid, Martina, Moes, Anna, Meitinger, Thomas, Mak, Angel, Huikuri, Heikki, Junttila, M Juhani, Goyette, Philippe, Pulit, Sara L, Pazoki, Raha, Tanck, Michael W, Blom, Marieke T, Zhao, XiaoQing, Havulinna, Aki S, Jabbari, Reza, Glinge, Charlotte, Tragante, Vinicius, Escher, Stefan A, Chakravarti, Aravinda, Ehret, Georg, Coresh, Josef, Li, Man, Prineas, Ronald J, Franco, Oscar H, Kwok, Pui-Yan, Lumley, Thomas, Dumas, Florence, McKnight, Barbara, Rotter, Jerome I, Lemaitre, Rozenn N, Heckbert, Susan R, O’Donnell, Christopher J, Hwang, Shih-Jen, Tardif, Jean-Claude, VanDenburgh, Martin, Uitterlinden, André G, Hofman, Albert, Stricker, Bruno HC, de Bakker, Paul IW, Franks, Paul W, Jansson, Jan-Hakan, Asselbergs, Folkert W, Halushka, Marc K, Maleszewski, Joseph J, Tfelt-Hansen, Jacob, Engstrøm, Thomas, Salomaa, Veikko, Virmani, Renu, Kolodgie, Frank, Wilde, Arthur AM, Tan, Hanno L, Bezzina, Connie R, Eijgelsheim, Mark, Rioux, John D, Jouven, Xavier, Kääb, Stefan, Psaty, Bruce M, Siscovick, David S, Arking, Dan E, and Sotoodehnia, Nona

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Prevention, Heart Disease, Human Genome, Genetics, Clinical Research, Heart Disease - Coronary Heart Disease, Cardiovascular, 2.1 Biological and endogenous factors, Aetiology, Good Health and Well Being, Arrhythmias, Cardiac, Body Mass Index, Coronary Artery Disease, Death, Sudden, Cardiac, Female, Genome-Wide Association Study, Heart Conduction System, Humans, Male, Mendelian Randomization Analysis, Polymorphism, Single Nucleotide, Risk Assessment, Risk Factors, Sex Factors, Sudden cardiac arrest, Genome-wide association study, Mendelian randomization, Cardiorespiratory Medicine and Haematology, Cardiovascular System & Hematology, Cardiovascular medicine and haematology, Clinical sciences
Abstract

AimsSudden cardiac arrest (SCA) accounts for 10% of adult mortality in Western populations. We aim to identify potential loci associated with SCA and to identify risk factors causally associated with SCA.Methods and resultsWe carried out a large genome-wide association study (GWAS) for SCA (n = 3939 cases, 25 989 non-cases) to examine common variation genome-wide and in candidate arrhythmia genes. We also exploited Mendelian randomization (MR) methods using cross-trait multi-variant genetic risk score associations (GRSA) to assess causal relationships of 18 risk factors with SCA. No variants were associated with SCA at genome-wide significance, nor were common variants in candidate arrhythmia genes associated with SCA at nominal significance. Using cross-trait GRSA, we established genetic correlation between SCA and (i) coronary artery disease (CAD) and traditional CAD risk factors (blood pressure, lipids, and diabetes), (ii) height and BMI, and (iii) electrical instability traits (QT and atrial fibrillation), suggesting aetiologic roles for these traits in SCA risk.ConclusionsOur findings show that a comprehensive approach to the genetic architecture of SCA can shed light on the determinants of a complex life-threatening condition with multiple influencing factors in the general population. The results of this genetic analysis, both positive and negative findings, have implications for evaluating the genetic architecture of patients with a family history of SCA, and for efforts to prevent SCA in high-risk populations and the general community.

Published
2018

27. European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) Expert Consensus Statement on the State of Genetic Testing for Cardiac Diseases

Author

Wilde, Arthur A.M., Semsarian, Christopher, Márquez, Manlio F., Sepehri Shamloo, Alireza, Ackerman, Michael J., Ashley, Euan A., Sternick, Eduardo Back, Barajas-Martinez, Héctor, Behr, Elijah R., Bezzina, Connie R., Breckpot, Jeroen, Charron, Philippe, Chockalingam, Priya, Crotti, Lia, Gollob, Michael H., Lubitz, Steven, Makita, Naomasa, Ohno, Seiko, Ortiz-Genga, Martín, Sacilotto, Luciana, Schulze-Bahr, Eric, Shimizu, Wataru, Sotoodehnia, Nona, Tadros, Rafik, Ware, James S., Winlaw, David S., Kaufman, Elizabeth S., Aiba, Takeshi, Bollmann, Andreas, Choi, Jong-Il, Dalal, Aarti, Darrieux, Francisco, Giudicessi, John, Guerchicoff, Mariana, Hong, Kui, Krahn, Andrew D., MacIntyre, Ciorsti, Mackall, Judith A., Mont, Lluís, Napolitano, Carlo, Ochoa, Juan Pablo, Peichl, Petr, Pereira, Alexandre C., Schwartz, Peter J., Skinner, Jon, Stellbrink, Christoph, Tfelt-Hansen, Jacob, and Deneke, Thomas

Published
2022
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29. Genome-wide association analyses identify new Brugada syndrome risk loci and highlight a new mechanism of sodium channel regulation in disease susceptibility

Author

Barc, Julien, Tadros, Rafik, Glinge, Charlotte, Chiang, David Y., Jouni, Mariam, Simonet, Floriane, Jurgens, Sean J., Baudic, Manon, Nicastro, Michele, Potet, Franck, Offerhaus, Joost A., Walsh, Roddy, Choi, Seung Hoan, Verkerk, Arie O., Mizusawa, Yuka, Anys, Soraya, Minois, Damien, Arnaud, Marine, Duchateau, Josselin, Wijeyeratne, Yanushi D., Muir, Alison, Papadakis, Michael, Castelletti, Silvia, Torchio, Margherita, Ortuño, Cristina Gil, Lacunza, Javier, Giachino, Daniela F., Cerrato, Natascia, Martins, Raphaël P., Campuzano, Oscar, Van Dooren, Sonia, Thollet, Aurélie, Kyndt, Florence, Mazzanti, Andrea, Clémenty, Nicolas, Bisson, Arnaud, Corveleyn, Anniek, Stallmeyer, Birgit, Dittmann, Sven, Saenen, Johan, Noël, Antoine, Honarbakhsh, Shohreh, Rudic, Boris, Marzak, Halim, Rowe, Matthew K., Federspiel, Claire, Le Page, Sophie, Placide, Leslie, Milhem, Antoine, Barajas-Martinez, Hector, Beckmann, Britt-Maria, Krapels, Ingrid P., Steinfurt, Johannes, Winkel, Bo Gregers, Jabbari, Reza, Shoemaker, Moore B., Boukens, Bas J., Škorić-Milosavljević, Doris, Bikker, Hennie, Manevy, Federico C., Lichtner, Peter, Ribasés, Marta, Meitinger, Thomas, Müller-Nurasyid, Martina, Veldink, Jan H., van den Berg, Leonard H., Van Damme, Philip, Cusi, Daniele, Lanzani, Chiara, Rigade, Sidwell, Charpentier, Eric, Baron, Estelle, Bonnaud, Stéphanie, Lecointe, Simon, Donnart, Audrey, Le Marec, Hervé, Chatel, Stéphanie, Karakachoff, Matilde, Bézieau, Stéphane, London, Barry, Tfelt-Hansen, Jacob, Roden, Dan, Odening, Katja E., Cerrone, Marina, Chinitz, Larry A., Volders, Paul G., van de Berg, Maarten P., Laurent, Gabriel, Faivre, Laurence, Antzelevitch, Charles, Kääb, Stefan, Arnaout, Alain Al, Dupuis, Jean-Marc, Pasquie, Jean-Luc, Billon, Olivier, Roberts, Jason D., Jesel, Laurence, Borggrefe, Martin, Lambiase, Pier D., Mansourati, Jacques, Loeys, Bart, Leenhardt, Antoine, Guicheney, Pascale, Maury, Philippe, Schulze-Bahr, Eric, Robyns, Tomas, Breckpot, Jeroen, Babuty, Dominique, Priori, Silvia G., Napolitano, Carlo, de Asmundis, Carlo, Brugada, Pedro, Brugada, Ramon, Arbelo, Elena, Brugada, Josep, Mabo, Philippe, Behar, Nathalie, Giustetto, Carla, Molina, Maria Sabater, Gimeno, Juan R., Hasdemir, Can, Schwartz, Peter J., Crotti, Lia, McKeown, Pascal P., Sharma, Sanjay, Behr, Elijah R., Haissaguerre, Michel, Sacher, Frédéric, Rooryck, Caroline, Tan, Hanno L., Remme, Carol A., Postema, Pieter G., Delmar, Mario, Ellinor, Patrick T., Lubitz, Steven A., Gourraud, Jean-Baptiste, Tanck, Michael W., George, Jr., Alfred L., MacRae, Calum A., Burridge, Paul W., Dina, Christian, Probst, Vincent, Wilde, Arthur A., Schott, Jean-Jacques, Redon, Richard, and Bezzina, Connie R.

Published
2022
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30. Harmonization of the definition of sudden cardiac death in longitudinal cohorts of the European Sudden Cardiac Arrest network – towards Prevention, Education, and New Effective Treatments (ESCAPE-NET) consortium

Author

Warming, Peder Emil, Ågesen, Frederik Nybye, Lynge, Thomas Hadberg, Jabbari, Reza, Smits, Robin L.A., van Valkengoed, Irene G.M., Welten, Sabrina J.G.C., van der Heijden, Amber A., Elders, Petra J., Blom, Marieke T., Jouven, Xavier, Schwartz, Peter J., Albert, Christine M., Beulens, Joline W., Rutters, Femke, Tan, Hanno L., Empana, Jean-Philippe, and Tfelt-Hansen, Jacob

Published
2022
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32. Understanding the incidence of atrial fibrillation and stroke in hypertrophic cardiomyopathy patients: insights from Danish nationwide registries.

Author

Zörner, Christopher R, Schjerning, Anne-Marie, Jensen, Morten Kvistholm, Christensen, Alex Hørby, Tfelt-Hansen, Jacob, Tønnesen, Jacob, Riis-Vestergaard, Lise Da, Middelfart, Charlotte, Rasmussen, Peter Vibe, Gislason, Gunnar, and Hansen, Morten Lock

Abstract

Aims The treatment of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) can be challenging since AF aggravates symptoms and increases the risk of stroke. Which factors contribute to the development of AF and stroke in HCM remains unknown. The aim of this study was to determine the incidence of AF and stroke in HCM patients and identify the risk factors. Methods and results Using Danish national registries, all HCM patients from 2005 to 2018 were included. The association between HCM, incident AF, and stroke was investigated using multivariable Cox proportional hazards analysis. Cumulative incidences were calculated using the Aalen–Johansen estimator. Among the 3367 patients without prevalent AF, 24% reached the endpoint of incident AF with death as a competing risk. Median follow-up time was 4 years. Atrial fibrillation incidence was equal between sexes and increased for patients with ischaemic heart disease [IHD; hazard ratio (HR) 1.33, 95% confidence interval (CI) 1.08–1.63], hypertension (HT) (HR 1.36, 95% CI 1.14–1.67), and obstructive HCM (HR 1.27, 95% CI 1.05–1.52). Seven per cent developed stroke, with no difference detected stratifying for the presence of AF. Sub-analysis revealed that when AF was treated with oral anticoagulants (OACs), stroke was less likely (HR 0.4, 95% CI 0.18–0.86, P = 0.02). However, 34% of patients were not receiving adequate anticoagulation following AF diagnosis. Conclusion Obstructive HCM, HT, and IHD were associated with increased risk of AF. Prevalent AF alone was not predictive of stroke; however, AF patients treated with OAC were significantly less likely to develop stroke, suggesting that this development is driven by the protective effect of OAC. Despite this, 34% of patients did not receive OAC. [ABSTRACT FROM AUTHOR]

Published
2024
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34. Baseline Characteristics of Patients in SEQUOIA-HCM: A Phase 3 Trial of Aficamten in Obstructive Hypertrophic Cardiomyopathy

Author

Maron, Martin S., primary, Abraham, Theodore P., additional, Coats, Caroline J., additional, Olivotto, Iacopo, additional, Lee, Matthew M.Y., additional, Arad, Michael, additional, Cardim, Nuno, additional, Ma, Chang-Sheng, additional, Choudhury, Lubna, additional, Düngen, Hans-Dirk, additional, Garcia-Pavia, Pablo, additional, Hagege, Albert, additional, Lewis, Gregory D., additional, Michels, Michelle, additional, Oreziak, Artur, additional, Owens, Anjali T., additional, Tfelt-Hansen, Jacob, additional, Veselka, Josef, additional, Watkins, Hugh C., additional, Heitner, Stephen B., additional, Jacoby, Daniel L., additional, Kupfer, Stuart, additional, Malik, Fady I., additional, Meng, Lisa, additional, Wohltman, Amy, additional, and Masri, Ahmad, additional

Published
2024
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39. 2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families

Author

Stiles, Martin K., Wilde, Arthur A.M., Abrams, Dominic J., Ackerman, Michael J., Albert, Christine M., Behr, Elijah R., Chugh, Sumeet S., Cornel, Martina C., Gardner, Karen, Ingles, Jodie, James, Cynthia A., Jimmy Juang, Jyh-Ming, Kääb, Stefan, Kaufman, Elizabeth S., Krahn, Andrew D., Lubitz, Steven A., MacLeod, Heather, Morillo, Carlos A., Nademanee, Koonlawee, Probst, Vincent, Saarel, Elizabeth V., Sacilotto, Luciana, Semsarian, Christopher, Sheppard, Mary N., Shimizu, Wataru, Skinner, Jonathan R., Tfelt-Hansen, Jacob, and Wang, Dao Wu

Published
2021
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40. Common and rare susceptibility genetic variants predisposing to Brugada syndrome in Thailand

Author

Makarawate, Pattarapong, Glinge, Charlotte, Khongphatthanayothin, Apichai, Walsh, Roddy, Mauleekoonphairoj, John, Amnueypol, Montawatt, Prechawat, Somchai, Wongcharoen, Wanwarang, Krittayaphong, Rungroj, Anannab, Alisara, Lichtner, Peter, Meitinger, Thomas, Tjong, Fleur V.Y., Lieve, Krystien V.V., Amin, Ahmad S., Sahasatas, Dujdao, Ngarmukos, Tachapong, Wichadakul, Duangdao, Payungporn, Sunchai, Sutjaporn, Boosamas, Wandee, Pharawee, Poovorawan, Yong, Tfelt-Hansen, Jacob, Tanck, Michael W.T., Tadros, Rafik, Wilde, Arthur A.M., Bezzina, Connie R., Veerakul, Gumpanart, and Nademanee, Koonlawee

Published
2020
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42. Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC—endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society

Author

Lenarczyk, Radosław, Zeppenfeld, Katja, Tfelt-Hansen, Jacob, Heinzel, Frank R, Deneke, Thomas, Ene, Elena, Meyer, Christian, Wilde, Arthur, Arbelo, Elena, Jędrzejczyk-Patej, Ewa, Sabbag, Avi, Stühlinger, Markus, Biase, Luigi di, Vaseghi, Marmar, Ziv, Ohad, Bautista-Vargas, William-Fernando, Kumar, Saurabh, Namboodiri, Narayanan, Henz, Benhur Davi, and Montero-Cabezas, Jose

Abstract

Electrical storm (ES) is a state of electrical instability, manifesting as recurrent ventricular arrhythmias (VAs) over a short period of time (three or more episodes of sustained VA within 24 h, separated by at least 5 min, requiring termination by an intervention). The clinical presentation can vary, but ES is usually a cardiac emergency. Electrical storm mainly affects patients with structural or primary electrical heart disease, often with an implantable cardioverter-defibrillator (ICD). Management of ES requires a multi-faceted approach and the involvement of multi-disciplinary teams, but despite advanced treatment and often invasive procedures, it is associated with high morbidity and mortality. With an ageing population, longer survival of heart failure patients, and an increasing number of patients with ICD, the incidence of ES is expected to increase. This European Heart Rhythm Association clinical consensus statement focuses on pathophysiology, clinical presentation, diagnostic evaluation, and acute and long-term management of patients presenting with ES or clustered VA. [ABSTRACT FROM AUTHOR]

Published
2024
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46. Toxicology Screening in Sports-Related Sudden Cardiac Death

Author

Hansen, Carl J., primary, Svane, Jesper, additional, Palsøe, Marie K., additional, Isbister, Julia C., additional, Paratz, Elizabeth, additional, Molina, Pilar, additional, Morentin, Benito, additional, Winkel, Bo G., additional, La Gerche, André, additional, Linnet, Kristian, additional, Banner, Jytte, additional, Lucena, Joaquin, additional, Semsarian, Christopher, additional, and Tfelt-Hansen, Jacob, additional

Published
2023
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47. Exercise Capacity in Patients With Obstructive Hypertrophic Cardiomyopathy

Author

Coats, Caroline J., primary, Maron, Martin S., additional, Abraham, Theodore P., additional, Olivotto, Iacopo, additional, Lee, Matthew M.Y., additional, Arad, Michael, additional, Cardim, Nuno, additional, Ma, Chang-Sheng, additional, Choudhury, Lubna, additional, Düngen, Hans-Dirk, additional, Garcia-Pavia, Pablo, additional, Hagège, Albert A., additional, Lewis, Gregory D., additional, Michels, Michelle, additional, Oreziak, Artur, additional, Owens, Anjali T., additional, Tfelt-Hansen, Jacob, additional, Veselka, Josef, additional, Watkins, Hugh C., additional, Heitner, Stephen B., additional, Jacoby, Daniel L., additional, Kupfer, Stuart, additional, Malik, Fady I., additional, Meng, Lisa, additional, Wohltman, Amy, additional, and Masri, Ahmad, additional

Published
2023
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48. Discovery of plasma proteins associated with ventricular fibrillation during first ST-elevation myocardial infarction via proteomics

Author

Stampe, Niels Kjær, primary, Ottenheijm, Maud Eline, additional, Drici, Lylia, additional, Wewer Albrechtsen, Nicolai J, additional, Nielsen, Annelaura Bach, additional, Christoffersen, Christina, additional, Warming, Peder Emil, additional, Engstrøm, Thomas, additional, Winkel, Bo Gregers, additional, Jabbari, Reza, additional, Tfelt-Hansen, Jacob, additional, and Glinge, Charlotte, additional

Published
2023
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49. Ethnic differences in patients with Brugada syndrome and arrhythmic events: New insights from Survey on Arrhythmic Events in Brugada Syndrome

Author

Milman, Anat, Andorin, Antoine, Postema, Pieter G., Gourraud, Jean-Baptiste, Sacher, Frederic, Mabo, Philippe, Kim, Sung-Hwan, Maeda, Shingo, Takahashi, Yoshihide, Kamakura, Tsukasa, Aiba, Takeshi, Conte, Giulio, Juang, Jimmy J.M., Leshem, Eran, Michowitz, Yoav, Fogelman, Rami, Hochstadt, Aviram, Mizusawa, Yuka, Giustetto, Carla, Arbelo, Elena, Huang, Zhengrong, Corrado, Domenico, Delise, Pietro, Allocca, Giuseppe, Takagi, Masahiko, Wijeyeratne, Yanushi D., Mazzanti, Andrea, Brugada, Ramon, Casado-Arroyo, Ruben, Champagne, Jean, Calo, Leonardo, Sarquella-Brugada, Georgia, Jespersen, Camilla H., Tfelt-Hansen, Jacob, Veltmann, Christian, Priori, Silvia G., Behr, Elijah R., Yan, Gan-Xin, Brugada, Josep, Gaita, Fiorenzo, Wilde, Arthur A.M., Brugada, Pedro, Kusano, Kengo F., Hirao, Kenzo, Nam, Gi-Byoung, Probst, Vincent, and Belhassen, Bernard

Published
2019
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