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

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.2019.05.007

Cited by 522 publications

(627 citation statements)

References 546 publications

(673 reference statements)

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“…This further emphasized the fact that despite the improved diagnostic accuracy with the objective criteria of the revised TFC, a careful clinical and diagnostic evaluation is critical for the clinicians during the evaluation for ARVC. This issue is also recognized by the 2019 HRS Expert Consensus Statement on Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy, while discussing the role of CMR in the diagnosis of ARVC . The guidelines explicitly mentioned that regional RV wall motion abnormality is essential for minor criteria along with RV enlargement and the importance of CMR expertize.…”

Section: Discussionmentioning

confidence: 99%

“…It is important for cardiologists to recognize that merely establishment of the diagnosis of ARVC does not automatically warrant ICD implantation. Tools to stratify sudden death risk are now available . The most recent contribution to this topic is the development of a personalized risk calculator that clinicians can use to estimate the annual and 5 year risk of a sustained ventricular in a patient with ARVC on the basis of simple criteria, such as age, sex, prior syncope, PVC frequency, and NSVT …”

Section: Discussionmentioning

confidence: 99%

“…Shown in Table 3 discussing the role of CMR in the diagnosis of ARVC. 15 The guidelines explicitly mentioned that regional RV wall motion abnormality is essential for minor criteria along with RV enlargement and the importance of CMR expertize.…”

Section: Final Diagnosis: Tfc Evaluation and Icd Explantsmentioning

confidence: 99%

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Misdiagnosis of ARVC leading to inappropriate ICD implant and subsequent ICD removal – lessons learned

Sharma

Assis

James

et al. 2019

Cardiovasc electrophysiol

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Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited progressive cardiomyopathy characterized by frequent life‐threatening arrhythmias. The diagnosis of ARVC is challenging and is on the basis of a set of major and minor criteria as described by the modified Task Force Criteria (TFC). We report our clinical experience in a series of patients who were misdiagnosed with ARVC and subsequently underwent removal of their implantable cardioverter defibrillator (ICD) after a re‐evaluation at our center. Methods and results We studied 12 patients who were misdiagnosed with ARVC and had ICD implantation before our assessment. All patients had a repeat evaluation and were scored according to TFC before ICD removal. Cardiac magnetic resonance imaging (CMR) studies performed at outside institutions during the initial evaluation were reported abnormal and classified as meeting major TFC in ninety percent of patients. The most common abnormality reported was fatty infiltration of the right ventricular (RV) free wall and/or presence of focal intra‐myocardial fat in six patients (50%). On re‐evaluation, none of these findings fulfilled the TFC for the diagnosis. Conclusion This study demonstrated that high dependence on misinterpretation of CMR along with a misunderstanding of the TFC evaluation are the main reasons for the misdiagnosis of ARVC. Despite the updated criteria for almost a decade, this study reminds that the diagnosis of ARVC is complex and hence careful TFC evaluation and consideration of multiple cardiac test results should be the focused approach for clinicians when confronted with suspected ARVC patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Misdiagnosis of ARVC leading to inappropriate ICD implant and subsequent ICD removal – lessons learned

Sharma

Assis

James

et al. 2019

Cardiovasc electrophysiol

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“…All these specific risk factors for SCD in AC have extensively been re-discussed in the more recent Consensus Document for AC in 2019 and once again no specific recommendations are provided for the pediatric age (72). According to the authors, in case of primary prevention, an ICD should be evaluated by means of a risk score based on the presence of major or minor risk markers.…”

Section: Arrhythmogenic Cardiomyopathymentioning

confidence: 99%

Sudden Cardiac Death in Children Affected by Cardiomyopathies: An Update on Risk Factors and Indications at Transvenous or Subcutaneous Implantable Defibrillators

2020

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In the present paper, we will discuss the main cardiomyopathies affecting children with a specific focus on risk stratification and prevention of sudden cardiac death (SCD). We will discuss the main clinical features of hypertrophic cardiomyopathy (HCM), dilated and restrictive cardiomyopathies, left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (AC), always highlighting their peculiarities in the pediatric age. Since sudden cardiac death may be the first manifestation of the disease, even in children, the identification of the specific underlying condition and of risk factors are pivotal to carry out the appropriate preventing strategies. ICD recommendations in children are similar to adults, but supporting evidences are not so solid, being based on registries or single center studies. Furthermore, children and young patients are most likely to manifest long term complications related to an implanted ICD, and this should be taken into account when evaluating the risk benefit ratio. In this perspective, subcutaneous ICDs (S-ICDs) could carry an advantage; however, they cannot be considered in small children for technical reasons. Data on effectiveness and safety of S-ICDs in a pediatric population is still lacking, although some limited experiences are reported and will be discussed in the current review.

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“…Furthermore, all HCM and ACM genes are usually included in diagnostic DCM panels, as a certain degree of genetic and phenotypic overlap can be present ( Table 1). The most significant DCM genes, which should be always tested are: TTN, LMNA, MYH7, TNNT2, BAG3, RBM20, TNNC1, TNNI3, TPM1, SCN5A, PLN, and FLNC [26,27]; while there are a number of other minor genes that have been associated with DCM, but should be screened only in selected cases [28]. Among the major genes, TTN truncating mutations are the most common cause of DCM, occurring iñ 25% of familial cases of DCM and in 18% of sporadic cases [29].…”

Section: Depicting the Genetic Background Of Inherited Hfmentioning

confidence: 99%

Novel Basic Science Insights to Improve the Management of Heart Failure: Review of the Working Group on Cellular and Molecular Biology of the Heart of the Italian Society of Cardiology

Ameri

Schiattarella

Crotti

et al. 2020

IJMS

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Despite important advances in diagnosis and treatment, heart failure (HF) remains a syndrome with substantial morbidity and dismal prognosis. Although implementation and optimization of existing technologies and drugs may lead to better management of HF, new or alternative strategies are desirable. In this regard, basic science is expected to give fundamental inputs, by expanding the knowledge of the pathways underlying HF development and progression, identifying approaches that may improve HF detection and prognostic stratification, and finding Int.

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

Cited by 522 publications

References 546 publications

Misdiagnosis of ARVC leading to inappropriate ICD implant and subsequent ICD removal – lessons learned

Misdiagnosis of ARVC leading to inappropriate ICD implant and subsequent ICD removal – lessons learned

Sudden Cardiac Death in Children Affected by Cardiomyopathies: An Update on Risk Factors and Indications at Transvenous or Subcutaneous Implantable Defibrillators

Novel Basic Science Insights to Improve the Management of Heart Failure: Review of the Working Group on Cellular and Molecular Biology of the Heart of the Italian Society of Cardiology

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