Optimal management of syncope: the new ESC Guidelines and novel insights into its underlying causes

Optimal management of syncope: the new ESC Guidelines and novel insights into its underlying causes For the podcast associated with this article, please visit https://academic.oup.com/eurheartj/pages/Podcasts Syncope is an abrupt loss of consciousness that may either be transient or end in sudden death.1–4 Its causes are multiple and involve not only cardiac conditions, but also neurological conditions, among many other diseases. Thus, the diagnostic approach as well as the management has to be multidisciplinary.5 This is reflected by the composition of the Task Force of the ‘2018 ESC Guidelines for the diagnosis and management of syncope’ co-ordinated by Michele Brignole, which is truly multidisciplinary.6 The major changes are depicted in Figure 1. Figure 1 View largeDownload slide Changes in the most recent ESC Guidelines on syncope. Figure 1 View largeDownload slide Changes in the most recent ESC Guidelines on syncope. Syncope is not that uncommon during sports,7,8 in particular in those embarking on competitive sports, where it is associated with a wide spectrum of morphological and functional cardiac adaptations known as ‘the athlete’s heart’. These structural changes are usually mild compared with age- and gender-matched untrained individuals, but in some cases may be striking and overlap with cardiovascular disease such as hypertrophic,9 dilated,10 arrhythmogenic,11 or non-compaction cardiomyopathies.12 In a Current Opinion entitled ‘Recommendations for the indication and interpretation of cardiovascular imaging in the evaluation of the athlete’s heart’, Stefano Caselli and colleagues from the Institute of Sports Medicine and Science in Rome, Italy remind us that a correct diagnosis is of utmost importance, since these pathologies may be responsible for sports-related syncope and sudden cardiac death.7 Advances in imaging have improved diagnostics, allowing for the identification of a broader spectrum of pathological cardiovascular conditions occurring in athletes, further helping to differentiate benign adaptation from early manifestations of inherited cardiac diseases. An integrated and multimodality imaging approach is suggested to diagnose the most relevant pathological conditions in athletes. In patients with congenital heart disease, in particular, important factors for syncope or even sudden death are ventricular dysfunction or structural cardiac alterations.13 For instance, Ebstein anomaly can present with both right and left heart abnormalities; however, clinically useful predictors of sudden death have not been established. In their article, ‘Sudden death in patients with Ebstein anomaly’, Christopher J. Mcleod and colleagues from the Mayo Clinic in Rochester, Minnesota in the USA sought to characterize such risk factors in 968 Ebstein patients.14 The 10-, 50-, and 70-year incidences of sudden death were 0.8, 8.3, and 14.6%, respectively. Prior ventricular tachycardia, heart failure, tricuspid valve surgery, syncope, pulmonary stenosis, and haemoglobin >15 g/dL were predictors of sudden death. Thus, patients with Ebstein are at significant risk for sudden death. Such predictors can aid in risk stratification and potentially guide primary prevention with an implantable cardioverter defibrillator (ICD), as further discussed in an Editorial by Justin Thomas Tretter from the Cincinnati Children’s Hospital Medical Center in Ohio in the USA.15 In more than half of unexplained sudden cardiac arrests, a specific aetiology can be found by careful evaluation.16 In their article entitled ‘Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation’, Eloi Marijon et al. note that the characteristics and the extent to which such cases undergo a systematic thorough investigation in real-life practice are unknown.17 Among 18 622 out-of-hospital cardiac arrests, 717 survivors at hospital discharge fulfilled the definition of sudden cardiac arrest. Of those, 12.3% remained unexplained after ECG, echocardiography, and coronary angiography. Cardiac magnetic resonance imaging yielded the diagnosis in 3.5% of the cases, other investigations provided 2.4% additional diagnoses, and 6.8% were considered as idiopathic ventricular fibrillation (Figure 2). Among the latter, 16.3% benefited from a complete work-up including pharmacological testing. Younger patients and those admitted to university centres were more thoroughly investigated. Genetic testing and family screening were initiated in only 18.4% and 24.5%, respectively. Thus, complete investigations are carried out in a very low proportion of patients with unexplained cardiac arrest. Standardized, systematic approaches need to be implemented, as outlined in an Editorial by Andrew D. Krahn from the University of Western Ontario in London, Ontario in Canada.18 Figure 2 View largeDownload slide Flow chart of the study. ACS, acute coronary syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SCA, sudden cardiac arrest; WPW, Wolff–Parkinson–White syndrome (from Waldmann V, Bougouin W, Karam N, Dumas F, Sharifzadehgan A, Gandjbakhch E, Algalarrondo V, Narayanan K, Zhao A, Amet D, Jost D, Geri G, Lamhaut L, Beganton F, Ludes B, Bruneval P, Plu I, Hidden-Lucet F, Albuisson J, Lavergne T, Piot O, Alonso C, Leenhardt A, Lellouche N, Extramiana F, Cariou A, Jouven X, Marijon E, on behalf Paris-SDEC investigators. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation. See pages 1981–1987). Figure 2 View largeDownload slide Flow chart of the study. ACS, acute coronary syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SCA, sudden cardiac arrest; WPW, Wolff–Parkinson–White syndrome (from Waldmann V, Bougouin W, Karam N, Dumas F, Sharifzadehgan A, Gandjbakhch E, Algalarrondo V, Narayanan K, Zhao A, Amet D, Jost D, Geri G, Lamhaut L, Beganton F, Ludes B, Bruneval P, Plu I, Hidden-Lucet F, Albuisson J, Lavergne T, Piot O, Alonso C, Leenhardt A, Lellouche N, Extramiana F, Cariou A, Jouven X, Marijon E, on behalf Paris-SDEC investigators. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation. See pages 1981–1987). Patients with aortic stenosis may also experience syncope, typically if it is severe and during abrupt physical stress.19 Among the different forms of aortic stenosis, those with a reduced valve area, preserved left ventricular ejection fraction, and low flow are currently defined using Doppler-echocardiography by a stroke volume index <35 mL/m2.20,21 However, the relationship between low flow and outcome is unclear, as pointed out by Christophe Tribouilloy and colleagues from Amiens Cedex 1 in Paris, France in their article ‘Impact of low stroke volume on mortality in patients with severe aortic stenosis and preserved left ventricular ejection fraction’.22 They analysed the relationship between low flow and mortality in 1450 patients with aortic valve area <1 cm² and preserved ejection fraction, and 1645 normal controls. Five-year survival was particularly low with a stroke volume index below 30 mL/m2. After adjustment for outcome predictors, including surgery, mortality risk remained considerable in this group. Similar mortality risk was observed for stroke volume index 30–35 mL/m2. The prognostic impact of a low stroke volume index was consistent in subgroups, including asymptomatic patients and patients with low-gradient severe aortic stenosis. Thus, low flow defined as a stroke volume index below 30 mL/m2 is an important outcome predictor in severe aortic stenosis with preserved left ventricular ejection fraction under medical and surgical management. Further studies are needed to test these values prospectively for risk stratification and decision-making, as further outlined in a thought-provoking Editorial by Victoria Delgado from the Leiden University Medical Center in the Netherlands.23 The incidence of new-onset conduction abnormalities requiring permanent pacemaker implantation after transcatheter aortic valve implantation or TAVI differs among different valve types and implantation techniques. A meta-analysis on this subject entitled ‘Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review’, is presented by Jeroen J. Bax and colleagues from the Leiden University Medical Center in the Netherlands.24 Of 1406 original articles, 348 with a total of 17 139 patients were examined. The incidence of implantation of a permanent pacemaker after the use of a new-generation TAVI prosthesis ranged between 2% and 36%. For the balloon-expandable prostheses early-generation SAPIEN device it ranged between 2.3% and 28.2%, and with the SAPIEN 3 between 4% and 24% (Figure 3). For the self-expandable prostheses, the permanent pacemaker implantation rates were higher with the early generation of CoreValve device (16.3–37.7%) and, despite a reduction in permanent pacemaker implantation rates with the new Evolut R, they remained relatively higher (14.7–26.7%). Patients at high risk were more frequently women, and had pre-existent conduction abnormalities, calcification of the left ventricular outflow tract, prior balloon valvuloplasty, and low implantation of the valve. Thus, the rate of permanent pacemaker implantation after TAVI with new-generation devices is highly variable. Specific recommendations should consider pre-existent conduction abnormalities and anatomical factors to reduce the risk of permanent pacemaker implantation. Figure 3 View largeDownload slide Histograms showing the permanent pacemaker implantation rates after transcatheter aortic valve implantation using new-generation prostheses (from van Rosendael PJ, Delgado V, Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review. See pages 2003–2013). Figure 3 View largeDownload slide Histograms showing the permanent pacemaker implantation rates after transcatheter aortic valve implantation using new-generation prostheses (from van Rosendael PJ, Delgado V, Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review. See pages 2003–2013). The editors hope that readers of this issue of the European Heart Journal will find it of interest. References 1 Osafo N , Goel K , Geske J. An unusual cause of syncope while gardening . Eur Heart J 2016 ; 37 : 1552 . Google Scholar CrossRef Search ADS PubMed 2 Wahbi K , Babuty D , Probst V , Wissocque L , Labombarda F , Porcher R , Becane HM , Lazarus A , Behin A , Laforet P , Stojkovic T , Clementy N , Dussauge AP , Gourraud JB , Pereon Y , Lacour A , Chapon F , Milliez P , Klug D , Eymard B , Duboc D. Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1 . Eur Heart J 2017 ; 38 : 751 – 758 . Google Scholar PubMed 3 Genereux P , Pibarot P , Redfors B , Mack MJ , Makkar RR , Jaber WA , Svensson LG , Kapadia S , Tuzcu EM , Thourani VH , Babaliaros V , Herrmann HC , Szeto WY , Cohen DJ , Lindman BR , McAndrew T , Alu MC , Douglas PS , Hahn RT , Kodali SK , Smith CR , Miller DC , Webb JG , Leon MB. Staging classification of aortic stenosis based on the extent of cardiac damage . Eur Heart J 2017 ; 38 : 3351 – 3358 . Google Scholar CrossRef Search ADS PubMed 4 Baumgartner H , Falk V , Bax JJ , De Bonis M , Hamm C , Holm PJ , Iung B , Lancellotti P , Lansac E , Rodriguez Munoz D , Rosenhek R , Sjogren J , Tornos Mas P , Vahanian A , Walther T , Wendler O , Windecker S , Zamorano JL. 2017 ESC/EACTS Guidelines for the management of valvular heart disease . Eur Heart J 2017 ; 38 : 2739 – 2791 . Google Scholar CrossRef Search ADS PubMed 5 Costantino G , Sun BC , Barbic F , Bossi I , Casazza G , Dipaola F , McDermott D , Quinn J , Reed MJ , Sheldon RS , Solbiati M , Thiruganasambandamoorthy V , Beach D , Bodemer N , Brignole M , Casagranda I , Del Rosso A , Duca P , Falavigna G , Grossman SA , Ippoliti R , Krahn AD , Montano N , Morillo CA , Olshansky B , Raj SR , Ruwald MH , Sarasin FP , Shen WK , Stiell I , Ungar A , Gert van Dijk J , van Dijk N , Wieling W , Furlan R. Syncope clinical management in the emergency department: a consensus from the first international workshop on syncope risk stratification in the emergency department . Eur Heart J 2016 ; 37 : 1493 – 1498 . Google Scholar CrossRef Search ADS PubMed 6 Brignole M , Moya A , de Lange FJ , Deharo JC , Elliott PM , Fanciulli A , Fedorowski A , Furlan R , Kenny RA , Martin A , Probst V , Reed MJ , Rice CP , Sutton R , Ungar A , van Dijk JG. 2018 ESC Guidelines for the diagnosis and management of syncope . Eur Heart J 2018 ; 39 : 1883 – 1948 . 7 Pelliccia A , Caselli S , Sharma S , Basso C , Bax JJ , Corrado D , D’Andrea A , D’Ascenzi F , Di Paolo FM , Edvardsen T , Gati S , Galderisi M , Heidbuchel H , Nchimi A , Nieman K , Papadakis M , Pisicchio C , Schmied C , Popescu BA , Habib G , Grobbee D , Lancellotti P. European Association of Preventive Cardiology (EAPC) and European Association of Cardiovascular Imaging (EACVI) joint position statement: recommendations for the indication and interpretation of cardiovascular imaging in the evaluation of the athlete’s heart . Eur Heart J 2018 ; 39 : 1949 – 1969 . 8 Sharma S , Drezner JA , Baggish A , Papadakis M , Wilson MG , Prutkin JM , La Gerche A , Ackerman MJ , Borjesson M , Salerno JC , Asif IM , Owens DS , Chung EH , Emery MS , Froelicher VF , Heidbuchel H , Adamuz C , Asplund CA , Cohen G , Harmon KG , Marek JC , Molossi S , Niebauer J , Pelto HF , Perez MV , Riding NR , Saarel T , Schmied CM , Shipon DM , Stein R , Vetter VL , Pelliccia A , Corrado D. International recommendations for electrocardiographic interpretation in athletes . Eur Heart J 2018 ; 39 : 1466 – 1480 . Google Scholar CrossRef Search ADS PubMed 9 Charron P , Elliott PM , Gimeno JR , Caforio ALP , Kaski JP , Tavazzi L , Tendera M , Maupain C , Laroche C , Rubis P , Jurcut R , Calo L , Helio TM , Sinagra G , Zdravkovic M , Kavoliuniene A , Felix SB , Grzybowski J , Losi MA , Asselbergs FW , Garcia-Pinilla JM , Salazar-Mendiguchia J , Mizia-Stec K , Maggioni AP. The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies . Eur Heart J 2018 ; 39 : 1784 – 1793 . Google Scholar CrossRef Search ADS PubMed 10 Haas J , Frese KS , Peil B , Kloos W , Keller A , Nietsch R , Feng Z , Muller S , Kayvanpour E , Vogel B , Sedaghat-Hamedani F , Lim WK , Zhao X , Fradkin D , Kohler D , Fischer S , Franke J , Marquart S , Barb I , Li DT , Amr A , Ehlermann P , Mereles D , Weis T , Hassel S , Kremer A , King V , Wirsz E , Isnard R , Komajda M , Serio A , Grasso M , Syrris P , Wicks E , Plagnol V , Lopes L , Gadgaard T , Eiskjaer H , Jorgensen M , Garcia-Giustiniani D , Ortiz-Genga M , Crespo-Leiro MG , Deprez RH , Christiaans I , van Rijsingen IA , Wilde AA , Waldenstrom A , Bolognesi M , Bellazzi R , Morner S , Bermejo JL , Monserrat L , Villard E , Mogensen J , Pinto YM , Charron P , Elliott P , Arbustini E , Katus HA , Meder B. Atlas of the clinical genetics of human dilated cardiomyopathy . Eur Heart J 2015 ; 36 : 1123 – 1135 . Google Scholar CrossRef Search ADS PubMed 11 Akdis D , Saguner AM , Shah K , Wei C , Medeiros-Domingo A , von Eckardstein A , Luscher TF , Brunckhorst C , Chen HSV , Duru F. Sex hormones affect outcome in arrhythmogenic right ventricular cardiomyopathy/dysplasia: from a stem cell derived cardiomyocyte-based model to clinical biomarkers of disease outcome . Eur Heart J 2017 ; 38 : 1498 – 1508 . Google Scholar CrossRef Search ADS PubMed 12 Sedaghat-Hamedani F , Haas J , Zhu F , Geier C , Kayvanpour E , Liss M , Lai A , Frese K , Pribe-Wolferts R , Amr A , Li DT , Samani OS , Carstensen A , Bordalo DM , Muller M , Fischer C , Shao J , Wang J , Nie M , Yuan L , Hassfeld S , Schwartz C , Zhou M , Zhou Z , Shu Y , Wang M , Huang K , Zeng Q , Cheng L , Fehlmann T , Ehlermann P , Keller A , Dieterich C , Streckfuss-Bomeke K , Liao Y , Gotthardt M , Katus HA , Meder B. Clinical genetics and outcome of left ventricular non-compaction cardiomyopathy . Eur Heart J 2017 ; 38 : 3449 – 3460 . Google Scholar CrossRef Search ADS PubMed 13 Oliver JM , Gallego P , Gonzalez AE , Garcia-Hamilton D , Avila P , Yotti R , Ferreira I , Fernandez-Aviles F. Risk factors for excess mortality in adults with congenital heart diseases . Eur Heart J 2017 ; 38 : 1233 – 1241 . Google Scholar PubMed 14 Attenhofer Jost CH , Tan NY , Hassan A , Vargas ER , Hodge DO , Dearani JA , Connolly H , Asirvatham SJ , McLeod CJ. Sudden death in patients with Ebstein anomaly . Eur Heart J 2018 ; 39 : 1970 – 1977 . 15 Tretter JT. Sudden death in Ebstein’s anomaly: are we killing our patients with surgery? Eur Heart J 2018 ; 39 : 1978 – 1980 . Google Scholar CrossRef Search ADS 16 Sutherland GR. Sudden cardiac death: the pro-arrhythmic interaction of an acute loading with an underlying substrate . Eur Heart J 2017 ; 38 : 2986 – 2994 . Google Scholar PubMed 17 Waldmann V , Bougouin W , Karam N , Dumas F , Sharifzadehgan A , Gandjbakhch E , Algalarrondo V , Narayanan K , Zhao A , Amet D , Jost D , Geri G , Lamhaut L , Beganton F , Ludes B , Bruneval P , Plu I , Hidden-Lucet F , Albuisson J , Lavergne T , Piot O , Alonso C , Leenhardt A , Lellouche N , Extramiana F , Cariou A , Jouven X , Marijon E. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation . Eur Heart J 2018 ; 39 : 1981 – 1987 . Google Scholar CrossRef Search ADS 18 Cheung CC, , Krahn AD. The importance of a comprehensive evaluation of survivors of cardiac arrest . Eur Heart J 2018 ; 39 : 1988 – 1991 . 19 Reddy YNV , Nishimura RA. Evaluating the severity of aortic stenosis: a re-look at our current ‘gold standard’ measurements . Eur Heart J 2018 ; doi: 10.1093/eurheartj/ehy224. 20 Clavel MA , Magne J , Pibarot P. Low-gradient aortic stenosis . Eur Heart J 2016 ; 37 : 2645 – 2657 . Google Scholar CrossRef Search ADS PubMed 21 Doi S , Ohno Y , Nakazawa G , Ikari Y. Uncommon cause of paradoxical low-flow low-gradient severe aortic stenosis: easy to underestimate, difficult to diagnose . Eur Heart J 2016 ; 37 : 2678 . Google Scholar CrossRef Search ADS PubMed 22 Rusinaru D , Bohbot Y , Ringle A , Marechaux S , Diouf M , Tribouilloy C. Impact of low stroke volume on mortality in patients with severe aortic stenosis and preserved left ventricular ejection fraction . Eur Heart J 2018 ; 39 : 1992 – 1999 . 23 Delgado V , Bax JJ. Left ventricular stroke volume in severe aortic stenosis and preserved left ventricular ejection fraction: prognostic relevance . Eur Heart J 2018 ; 39 : 2000 – 2002 . Google Scholar CrossRef Search ADS 24 van Rosendael PJ , Delgado V , Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review . Eur Heart J 2018 ; 39 : 2003 – 2013 . Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal Oxford University Press

Optimal management of syncope: the new ESC Guidelines and novel insights into its underlying causes

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Abstract

For the podcast associated with this article, please visit https://academic.oup.com/eurheartj/pages/Podcasts Syncope is an abrupt loss of consciousness that may either be transient or end in sudden death.1–4 Its causes are multiple and involve not only cardiac conditions, but also neurological conditions, among many other diseases. Thus, the diagnostic approach as well as the management has to be multidisciplinary.5 This is reflected by the composition of the Task Force of the ‘2018 ESC Guidelines for the diagnosis and management of syncope’ co-ordinated by Michele Brignole, which is truly multidisciplinary.6 The major changes are depicted in Figure 1. Figure 1 View largeDownload slide Changes in the most recent ESC Guidelines on syncope. Figure 1 View largeDownload slide Changes in the most recent ESC Guidelines on syncope. Syncope is not that uncommon during sports,7,8 in particular in those embarking on competitive sports, where it is associated with a wide spectrum of morphological and functional cardiac adaptations known as ‘the athlete’s heart’. These structural changes are usually mild compared with age- and gender-matched untrained individuals, but in some cases may be striking and overlap with cardiovascular disease such as hypertrophic,9 dilated,10 arrhythmogenic,11 or non-compaction cardiomyopathies.12 In a Current Opinion entitled ‘Recommendations for the indication and interpretation of cardiovascular imaging in the evaluation of the athlete’s heart’, Stefano Caselli and colleagues from the Institute of Sports Medicine and Science in Rome, Italy remind us that a correct diagnosis is of utmost importance, since these pathologies may be responsible for sports-related syncope and sudden cardiac death.7 Advances in imaging have improved diagnostics, allowing for the identification of a broader spectrum of pathological cardiovascular conditions occurring in athletes, further helping to differentiate benign adaptation from early manifestations of inherited cardiac diseases. An integrated and multimodality imaging approach is suggested to diagnose the most relevant pathological conditions in athletes. In patients with congenital heart disease, in particular, important factors for syncope or even sudden death are ventricular dysfunction or structural cardiac alterations.13 For instance, Ebstein anomaly can present with both right and left heart abnormalities; however, clinically useful predictors of sudden death have not been established. In their article, ‘Sudden death in patients with Ebstein anomaly’, Christopher J. Mcleod and colleagues from the Mayo Clinic in Rochester, Minnesota in the USA sought to characterize such risk factors in 968 Ebstein patients.14 The 10-, 50-, and 70-year incidences of sudden death were 0.8, 8.3, and 14.6%, respectively. Prior ventricular tachycardia, heart failure, tricuspid valve surgery, syncope, pulmonary stenosis, and haemoglobin >15 g/dL were predictors of sudden death. Thus, patients with Ebstein are at significant risk for sudden death. Such predictors can aid in risk stratification and potentially guide primary prevention with an implantable cardioverter defibrillator (ICD), as further discussed in an Editorial by Justin Thomas Tretter from the Cincinnati Children’s Hospital Medical Center in Ohio in the USA.15 In more than half of unexplained sudden cardiac arrests, a specific aetiology can be found by careful evaluation.16 In their article entitled ‘Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation’, Eloi Marijon et al. note that the characteristics and the extent to which such cases undergo a systematic thorough investigation in real-life practice are unknown.17 Among 18 622 out-of-hospital cardiac arrests, 717 survivors at hospital discharge fulfilled the definition of sudden cardiac arrest. Of those, 12.3% remained unexplained after ECG, echocardiography, and coronary angiography. Cardiac magnetic resonance imaging yielded the diagnosis in 3.5% of the cases, other investigations provided 2.4% additional diagnoses, and 6.8% were considered as idiopathic ventricular fibrillation (Figure 2). Among the latter, 16.3% benefited from a complete work-up including pharmacological testing. Younger patients and those admitted to university centres were more thoroughly investigated. Genetic testing and family screening were initiated in only 18.4% and 24.5%, respectively. Thus, complete investigations are carried out in a very low proportion of patients with unexplained cardiac arrest. Standardized, systematic approaches need to be implemented, as outlined in an Editorial by Andrew D. Krahn from the University of Western Ontario in London, Ontario in Canada.18 Figure 2 View largeDownload slide Flow chart of the study. ACS, acute coronary syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SCA, sudden cardiac arrest; WPW, Wolff–Parkinson–White syndrome (from Waldmann V, Bougouin W, Karam N, Dumas F, Sharifzadehgan A, Gandjbakhch E, Algalarrondo V, Narayanan K, Zhao A, Amet D, Jost D, Geri G, Lamhaut L, Beganton F, Ludes B, Bruneval P, Plu I, Hidden-Lucet F, Albuisson J, Lavergne T, Piot O, Alonso C, Leenhardt A, Lellouche N, Extramiana F, Cariou A, Jouven X, Marijon E, on behalf Paris-SDEC investigators. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation. See pages 1981–1987). Figure 2 View largeDownload slide Flow chart of the study. ACS, acute coronary syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SCA, sudden cardiac arrest; WPW, Wolff–Parkinson–White syndrome (from Waldmann V, Bougouin W, Karam N, Dumas F, Sharifzadehgan A, Gandjbakhch E, Algalarrondo V, Narayanan K, Zhao A, Amet D, Jost D, Geri G, Lamhaut L, Beganton F, Ludes B, Bruneval P, Plu I, Hidden-Lucet F, Albuisson J, Lavergne T, Piot O, Alonso C, Leenhardt A, Lellouche N, Extramiana F, Cariou A, Jouven X, Marijon E, on behalf Paris-SDEC investigators. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation. See pages 1981–1987). Patients with aortic stenosis may also experience syncope, typically if it is severe and during abrupt physical stress.19 Among the different forms of aortic stenosis, those with a reduced valve area, preserved left ventricular ejection fraction, and low flow are currently defined using Doppler-echocardiography by a stroke volume index <35 mL/m2.20,21 However, the relationship between low flow and outcome is unclear, as pointed out by Christophe Tribouilloy and colleagues from Amiens Cedex 1 in Paris, France in their article ‘Impact of low stroke volume on mortality in patients with severe aortic stenosis and preserved left ventricular ejection fraction’.22 They analysed the relationship between low flow and mortality in 1450 patients with aortic valve area <1 cm² and preserved ejection fraction, and 1645 normal controls. Five-year survival was particularly low with a stroke volume index below 30 mL/m2. After adjustment for outcome predictors, including surgery, mortality risk remained considerable in this group. Similar mortality risk was observed for stroke volume index 30–35 mL/m2. The prognostic impact of a low stroke volume index was consistent in subgroups, including asymptomatic patients and patients with low-gradient severe aortic stenosis. Thus, low flow defined as a stroke volume index below 30 mL/m2 is an important outcome predictor in severe aortic stenosis with preserved left ventricular ejection fraction under medical and surgical management. Further studies are needed to test these values prospectively for risk stratification and decision-making, as further outlined in a thought-provoking Editorial by Victoria Delgado from the Leiden University Medical Center in the Netherlands.23 The incidence of new-onset conduction abnormalities requiring permanent pacemaker implantation after transcatheter aortic valve implantation or TAVI differs among different valve types and implantation techniques. A meta-analysis on this subject entitled ‘Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review’, is presented by Jeroen J. Bax and colleagues from the Leiden University Medical Center in the Netherlands.24 Of 1406 original articles, 348 with a total of 17 139 patients were examined. The incidence of implantation of a permanent pacemaker after the use of a new-generation TAVI prosthesis ranged between 2% and 36%. For the balloon-expandable prostheses early-generation SAPIEN device it ranged between 2.3% and 28.2%, and with the SAPIEN 3 between 4% and 24% (Figure 3). For the self-expandable prostheses, the permanent pacemaker implantation rates were higher with the early generation of CoreValve device (16.3–37.7%) and, despite a reduction in permanent pacemaker implantation rates with the new Evolut R, they remained relatively higher (14.7–26.7%). Patients at high risk were more frequently women, and had pre-existent conduction abnormalities, calcification of the left ventricular outflow tract, prior balloon valvuloplasty, and low implantation of the valve. Thus, the rate of permanent pacemaker implantation after TAVI with new-generation devices is highly variable. Specific recommendations should consider pre-existent conduction abnormalities and anatomical factors to reduce the risk of permanent pacemaker implantation. Figure 3 View largeDownload slide Histograms showing the permanent pacemaker implantation rates after transcatheter aortic valve implantation using new-generation prostheses (from van Rosendael PJ, Delgado V, Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review. See pages 2003–2013). Figure 3 View largeDownload slide Histograms showing the permanent pacemaker implantation rates after transcatheter aortic valve implantation using new-generation prostheses (from van Rosendael PJ, Delgado V, Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review. See pages 2003–2013). The editors hope that readers of this issue of the European Heart Journal will find it of interest. References 1 Osafo N , Goel K , Geske J. An unusual cause of syncope while gardening . Eur Heart J 2016 ; 37 : 1552 . Google Scholar CrossRef Search ADS PubMed 2 Wahbi K , Babuty D , Probst V , Wissocque L , Labombarda F , Porcher R , Becane HM , Lazarus A , Behin A , Laforet P , Stojkovic T , Clementy N , Dussauge AP , Gourraud JB , Pereon Y , Lacour A , Chapon F , Milliez P , Klug D , Eymard B , Duboc D. Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1 . Eur Heart J 2017 ; 38 : 751 – 758 . Google Scholar PubMed 3 Genereux P , Pibarot P , Redfors B , Mack MJ , Makkar RR , Jaber WA , Svensson LG , Kapadia S , Tuzcu EM , Thourani VH , Babaliaros V , Herrmann HC , Szeto WY , Cohen DJ , Lindman BR , McAndrew T , Alu MC , Douglas PS , Hahn RT , Kodali SK , Smith CR , Miller DC , Webb JG , Leon MB. Staging classification of aortic stenosis based on the extent of cardiac damage . Eur Heart J 2017 ; 38 : 3351 – 3358 . Google Scholar CrossRef Search ADS PubMed 4 Baumgartner H , Falk V , Bax JJ , De Bonis M , Hamm C , Holm PJ , Iung B , Lancellotti P , Lansac E , Rodriguez Munoz D , Rosenhek R , Sjogren J , Tornos Mas P , Vahanian A , Walther T , Wendler O , Windecker S , Zamorano JL. 2017 ESC/EACTS Guidelines for the management of valvular heart disease . Eur Heart J 2017 ; 38 : 2739 – 2791 . Google Scholar CrossRef Search ADS PubMed 5 Costantino G , Sun BC , Barbic F , Bossi I , Casazza G , Dipaola F , McDermott D , Quinn J , Reed MJ , Sheldon RS , Solbiati M , Thiruganasambandamoorthy V , Beach D , Bodemer N , Brignole M , Casagranda I , Del Rosso A , Duca P , Falavigna G , Grossman SA , Ippoliti R , Krahn AD , Montano N , Morillo CA , Olshansky B , Raj SR , Ruwald MH , Sarasin FP , Shen WK , Stiell I , Ungar A , Gert van Dijk J , van Dijk N , Wieling W , Furlan R. Syncope clinical management in the emergency department: a consensus from the first international workshop on syncope risk stratification in the emergency department . Eur Heart J 2016 ; 37 : 1493 – 1498 . Google Scholar CrossRef Search ADS PubMed 6 Brignole M , Moya A , de Lange FJ , Deharo JC , Elliott PM , Fanciulli A , Fedorowski A , Furlan R , Kenny RA , Martin A , Probst V , Reed MJ , Rice CP , Sutton R , Ungar A , van Dijk JG. 2018 ESC Guidelines for the diagnosis and management of syncope . Eur Heart J 2018 ; 39 : 1883 – 1948 . 7 Pelliccia A , Caselli S , Sharma S , Basso C , Bax JJ , Corrado D , D’Andrea A , D’Ascenzi F , Di Paolo FM , Edvardsen T , Gati S , Galderisi M , Heidbuchel H , Nchimi A , Nieman K , Papadakis M , Pisicchio C , Schmied C , Popescu BA , Habib G , Grobbee D , Lancellotti P. European Association of Preventive Cardiology (EAPC) and European Association of Cardiovascular Imaging (EACVI) joint position statement: recommendations for the indication and interpretation of cardiovascular imaging in the evaluation of the athlete’s heart . Eur Heart J 2018 ; 39 : 1949 – 1969 . 8 Sharma S , Drezner JA , Baggish A , Papadakis M , Wilson MG , Prutkin JM , La Gerche A , Ackerman MJ , Borjesson M , Salerno JC , Asif IM , Owens DS , Chung EH , Emery MS , Froelicher VF , Heidbuchel H , Adamuz C , Asplund CA , Cohen G , Harmon KG , Marek JC , Molossi S , Niebauer J , Pelto HF , Perez MV , Riding NR , Saarel T , Schmied CM , Shipon DM , Stein R , Vetter VL , Pelliccia A , Corrado D. International recommendations for electrocardiographic interpretation in athletes . Eur Heart J 2018 ; 39 : 1466 – 1480 . Google Scholar CrossRef Search ADS PubMed 9 Charron P , Elliott PM , Gimeno JR , Caforio ALP , Kaski JP , Tavazzi L , Tendera M , Maupain C , Laroche C , Rubis P , Jurcut R , Calo L , Helio TM , Sinagra G , Zdravkovic M , Kavoliuniene A , Felix SB , Grzybowski J , Losi MA , Asselbergs FW , Garcia-Pinilla JM , Salazar-Mendiguchia J , Mizia-Stec K , Maggioni AP. The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies . Eur Heart J 2018 ; 39 : 1784 – 1793 . Google Scholar CrossRef Search ADS PubMed 10 Haas J , Frese KS , Peil B , Kloos W , Keller A , Nietsch R , Feng Z , Muller S , Kayvanpour E , Vogel B , Sedaghat-Hamedani F , Lim WK , Zhao X , Fradkin D , Kohler D , Fischer S , Franke J , Marquart S , Barb I , Li DT , Amr A , Ehlermann P , Mereles D , Weis T , Hassel S , Kremer A , King V , Wirsz E , Isnard R , Komajda M , Serio A , Grasso M , Syrris P , Wicks E , Plagnol V , Lopes L , Gadgaard T , Eiskjaer H , Jorgensen M , Garcia-Giustiniani D , Ortiz-Genga M , Crespo-Leiro MG , Deprez RH , Christiaans I , van Rijsingen IA , Wilde AA , Waldenstrom A , Bolognesi M , Bellazzi R , Morner S , Bermejo JL , Monserrat L , Villard E , Mogensen J , Pinto YM , Charron P , Elliott P , Arbustini E , Katus HA , Meder B. Atlas of the clinical genetics of human dilated cardiomyopathy . Eur Heart J 2015 ; 36 : 1123 – 1135 . Google Scholar CrossRef Search ADS PubMed 11 Akdis D , Saguner AM , Shah K , Wei C , Medeiros-Domingo A , von Eckardstein A , Luscher TF , Brunckhorst C , Chen HSV , Duru F. Sex hormones affect outcome in arrhythmogenic right ventricular cardiomyopathy/dysplasia: from a stem cell derived cardiomyocyte-based model to clinical biomarkers of disease outcome . Eur Heart J 2017 ; 38 : 1498 – 1508 . Google Scholar CrossRef Search ADS PubMed 12 Sedaghat-Hamedani F , Haas J , Zhu F , Geier C , Kayvanpour E , Liss M , Lai A , Frese K , Pribe-Wolferts R , Amr A , Li DT , Samani OS , Carstensen A , Bordalo DM , Muller M , Fischer C , Shao J , Wang J , Nie M , Yuan L , Hassfeld S , Schwartz C , Zhou M , Zhou Z , Shu Y , Wang M , Huang K , Zeng Q , Cheng L , Fehlmann T , Ehlermann P , Keller A , Dieterich C , Streckfuss-Bomeke K , Liao Y , Gotthardt M , Katus HA , Meder B. Clinical genetics and outcome of left ventricular non-compaction cardiomyopathy . Eur Heart J 2017 ; 38 : 3449 – 3460 . Google Scholar CrossRef Search ADS PubMed 13 Oliver JM , Gallego P , Gonzalez AE , Garcia-Hamilton D , Avila P , Yotti R , Ferreira I , Fernandez-Aviles F. Risk factors for excess mortality in adults with congenital heart diseases . Eur Heart J 2017 ; 38 : 1233 – 1241 . Google Scholar PubMed 14 Attenhofer Jost CH , Tan NY , Hassan A , Vargas ER , Hodge DO , Dearani JA , Connolly H , Asirvatham SJ , McLeod CJ. Sudden death in patients with Ebstein anomaly . Eur Heart J 2018 ; 39 : 1970 – 1977 . 15 Tretter JT. Sudden death in Ebstein’s anomaly: are we killing our patients with surgery? Eur Heart J 2018 ; 39 : 1978 – 1980 . Google Scholar CrossRef Search ADS 16 Sutherland GR. Sudden cardiac death: the pro-arrhythmic interaction of an acute loading with an underlying substrate . Eur Heart J 2017 ; 38 : 2986 – 2994 . Google Scholar PubMed 17 Waldmann V , Bougouin W , Karam N , Dumas F , Sharifzadehgan A , Gandjbakhch E , Algalarrondo V , Narayanan K , Zhao A , Amet D , Jost D , Geri G , Lamhaut L , Beganton F , Ludes B , Bruneval P , Plu I , Hidden-Lucet F , Albuisson J , Lavergne T , Piot O , Alonso C , Leenhardt A , Lellouche N , Extramiana F , Cariou A , Jouven X , Marijon E. Characteristics and clinical assessment of unexplained sudden cardiac arrest in the real-world setting: focus on idiopathic ventricular fibrillation . Eur Heart J 2018 ; 39 : 1981 – 1987 . Google Scholar CrossRef Search ADS 18 Cheung CC, , Krahn AD. The importance of a comprehensive evaluation of survivors of cardiac arrest . Eur Heart J 2018 ; 39 : 1988 – 1991 . 19 Reddy YNV , Nishimura RA. Evaluating the severity of aortic stenosis: a re-look at our current ‘gold standard’ measurements . Eur Heart J 2018 ; doi: 10.1093/eurheartj/ehy224. 20 Clavel MA , Magne J , Pibarot P. Low-gradient aortic stenosis . Eur Heart J 2016 ; 37 : 2645 – 2657 . Google Scholar CrossRef Search ADS PubMed 21 Doi S , Ohno Y , Nakazawa G , Ikari Y. Uncommon cause of paradoxical low-flow low-gradient severe aortic stenosis: easy to underestimate, difficult to diagnose . Eur Heart J 2016 ; 37 : 2678 . Google Scholar CrossRef Search ADS PubMed 22 Rusinaru D , Bohbot Y , Ringle A , Marechaux S , Diouf M , Tribouilloy C. Impact of low stroke volume on mortality in patients with severe aortic stenosis and preserved left ventricular ejection fraction . Eur Heart J 2018 ; 39 : 1992 – 1999 . 23 Delgado V , Bax JJ. Left ventricular stroke volume in severe aortic stenosis and preserved left ventricular ejection fraction: prognostic relevance . Eur Heart J 2018 ; 39 : 2000 – 2002 . Google Scholar CrossRef Search ADS 24 van Rosendael PJ , Delgado V , Bax JJ. Pacemaker implantation rate after transcatheter aortic valve implantation with early and new-generation devices: a systematic review . Eur Heart J 2018 ; 39 : 2003 – 2013 . Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

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European Heart JournalOxford University Press

Published: May 31, 2018

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