2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary

Towbin, Jeffrey A.; McKenna, William J.; Abrams, Dominic J.; Ackerman, Michael J.; Calkins, Hugh; Darrieux, Francisco; Daubert, James P.; Chillou, Christian de; DePasquale, E.C.; Desai, Milind Y.; Estes, N.A. Mark; Hua, Wei; Indik, Julia H.; Ingles, Jodie; James, Cynthia; John, Roy M.; Judge, Daniel P.; Keegan, Roberto; Krahn, Andrew D.; Link, Mark S.; Marcus, Frank I.; McLeod, Christopher J.; Mestroni, Luisa; Priori, Silvia G.; Saffitz, Jeffrey E.; Sanatani, Shubhayan; Shimizu, Wataru; Tintelen, J. Peter van; Wilde, Arthur A.M.; Zaręba, Wojciech

doi:10.1016/j.hrthm.2019.09.019

Cited by 151 publications

(117 citation statements)

References 124 publications

(11 reference statements)

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“…The latter, along with cardiac sodium channel NAv1.5 (SCN5A), filamin C (FLNC) and desmoplakin (DSP) mutations, is associated with a more aggressive disease and an arrhythmogenic phenotype [36]. This 'arrhythmogenic DCM' can occur in up to one third of DCM patients and overlaps with the current concept of arrhythmogenic cardiomyopathy [37].…”

Section: Dilated Cardiomyopathymentioning

confidence: 90%

Cardiovascular Magnetic Resonance and Sport Cardiology: a Growing Role in Clinical Dilemmas

Maestrini

Torlasco

Hughes

et al. 2020

J. of Cardiovasc. Trans. Res.

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Exercise training induces morphological and functional cardiovascular adaptation known as the "athlete's heart" with changes including dilatation, hypertrophy, and increased stroke volume. These changes may overlap with pathological appearances. Distinguishing athletic cardiac remodelling from cardiomyopathy is important and is a frequent medical dilemma. Cardiac magnetic resonance (CMR) has a role in clinical care as it can refine discrimination of health from a disease where ECG and echocardiography alone have left or generated uncertainty. CMR can more precisely assess cardiac structure and function as well as characterise the myocardium detecting key changes including myocardial scar and diffuse fibrosis. In this review, we will review the role of CMR in sports cardiology.

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Section: Dilated Cardiomyopathymentioning

confidence: 90%

Cardiovascular Magnetic Resonance and Sport Cardiology: a Growing Role in Clinical Dilemmas

Maestrini

Torlasco

Hughes

et al. 2020

J. of Cardiovasc. Trans. Res.

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“…Furthermore, a growing body of data has shown genetic and clinical overlaps between LD-ACM and a subset of dilated cardiomyopathy (DCM) (so called arrhythmogenic-DCM, a-DCM) which presents with increased arrhythmogenic risk exceeding the degree of the morphological anomalies and systolic dysfunction [ 74 , 76 , 77 , 78 ]. On the basis of these observations, an expert panel of the Heart Rhythm Society (HRS) has proposed to include a-DCM, ARVC and LD-ACM in a common nosological entity whose hallmark is the electrical instability [ 11 ]. However this classification of ACM has yet to be agreed upon by all the experts in the field.…”

Section: Genetics Clinical and Histological Hallmarksmentioning

confidence: 99%

“…Life threatening arrhythmias and sudden cardiac death (SCD) are the main clinical characteristics of ACM and often manifest in the early stages of the disease, before the occurrence of the histological abnormalities and cardiac dysfunction [ 3 , 11 ]. However, early diagnosis is challenging due to the nonspecific clinical presentation of the disease [ 3 ]; for this reason, ACM remains a leading cause of SCD, particularly in the young and athletes [ 1 , 3 , 4 , 11 , 12 ]. Presently there is no causal therapy and the main goals of treatment are to control symptoms, delay the progression toward heart failure, and to prevent SCD with implantable cardioverter defibrillators (ICDs) [ 1 , 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Established and Emerging Mechanisms in the Pathogenesis of Arrhythmogenic Cardiomyopathy: A Multifaceted Disease

Gao

Puthenvedu

Lombardi

et al. 2020

IJMS

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Arrhythmogenic cardiomyopathy (ACM) is a heritable myocardial disease that manifests with cardiac arrhythmias, syncope, sudden cardiac death, and heart failure in the advanced stages. The pathological hallmark of ACM is a gradual replacement of the myocardium by fibroadiposis, which typically starts from the epicardium. Molecular genetic studies have identified causal mutations predominantly in genes encoding for desmosomal proteins; however, non-desmosomal causal mutations have also been described, including genes coding for nuclear proteins, cytoskeleton componentsand proteins involved in excitation-contraction coupling. Despite the poor prognosis, currently available treatments can only partially control symptoms and to date there is no effective therapy for ACM. Inhibition of the canonical Wnt/β-catenin pathway and activation of the Hippo and the TGF-β pathways have been implicated in the pathogenesis of ACM. Yet, our understanding of the molecular mechanisms involved in the development of the disease and the cell source of fibroadiposis remains incomplete. Elucidation of the pathogenesis of the disease could facilitate targeted approaches for treatment. In this manuscript we will provide a comprehensive review of the proposed molecular and cellular mechanisms of the pathogenesis of ACM, including the emerging evidence on abnormal calcium homeostasis and inflammatory/autoimmune response. Moreover, we will propose novel hypothesis about the role of epicardial cells and paracrine factors in the development of the phenotype. Finally, we will discuss potential innovative therapeutic approaches based on the growing knowledge in the field.

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“…Arytmogenní kardiomyopatie je onemocnění řazené nově do široké skupiny arytmogenních kardiomyopatií, která dle doporučení Heart Rhythm Society (HRS) z roku 2019 zahrnují všechny kardiomyopatie s klinickou arytmickou prezentací. 31 Ve své klasické formě s dominantním pravostranným postižením je ARVC většinou autosomálně dominantně dědičné onemocnění s nekompletní penetrancí, jehož genetická podstata spočívá v mutaci genů pro desmosomy a jim příbuzné proteiny, které zajišťují mezibuněčná spojení. Porucha vede k postupnému nahrazování svaloviny pravé komory fi brolipomatózní tkání, která se stává arytmogenním substrátem s rizikem rozvoje fatální arytmie (obr.…”

Section: Arytmogenní Kardiomyopatie Pravé Komoryunclassified

“…3). 31,32 Prevalence ARVC je odhadována na 1 : 5 000, muži jsou postiženi třikrát častěji. V klinickém obraze dominují arytmické příznaky -palpitace, synkopy či náhlá srdeční smrt.…”

Section: Arytmogenní Kardiomyopatie Pravé Komoryunclassified

(Right ventricle in athletes)

Jeníšová¹,

Marek²,

Havránek³

et al. 2020

Cor Vasa

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Pravá komora (PK) hraje klíčovou roli v kardiovaskulárním výkonu sportovců, přesto je oproti levé komoře opomíjeným tématem. Arytmogenní kardiomyopatie postihující často dominantně PK je jednou z nejčastějších příčin úmrtí vrcholových sportovců především v evropských zemích. Screening a diferenciální diagnostika patologie PK u vrcholových sportovců může být obtížná. Pravá komora prodělává v rámci fyziologické adaptace na zátěž významné změny včetně dilatace a odlišení fyziologických změn od kardiovaskulární patologie vyžaduje specifi cká kritéria. Cílem článku je shrnutí poznatků o fyziologické adaptaci PK u sportovců, tak jak se projevují na EKG a v zobrazovacích metodách. Dále jsou probírána specifi ka diferenciální diagnózy u sportovců s fyziologickou remodelací PK zejména proti arytmogenní kardiomyopatii PK.

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2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary

Cited by 151 publications

References 124 publications

Cardiovascular Magnetic Resonance and Sport Cardiology: a Growing Role in Clinical Dilemmas

Cardiovascular Magnetic Resonance and Sport Cardiology: a Growing Role in Clinical Dilemmas

Established and Emerging Mechanisms in the Pathogenesis of Arrhythmogenic Cardiomyopathy: A Multifaceted Disease

(Right ventricle in athletes)

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