scholarly journals Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy

2021 ◽  
Vol 10 (18) ◽  
Author(s):  
Domenico Corrado ◽  
Alessandro Zorzi ◽  
Alberto Cipriani ◽  
Barbara Bauce ◽  
Riccardo Bariani ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Late Gadolinium Enhancement ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Task Force ◽  
Left Ventricular ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
New Criteria

Abstract Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and revised in 2010 by a Task Force. Although the Task Force criteria demonstrated a good accuracy for diagnosis of the original right ventricular phenotype (arrhythmogenic right ventricular cardiomyopathy), they lacked sensitivity for identification of the expanding phenotypic spectrum of ACM, which includes left‐sided variants and did not incorporate late‐gadolinium enhancement findings by cardiac magnetic resonance. The 2020 International criteria (“Padua criteria”) have been developed by International experts with the aim to improve the diagnosis of ACM by providing new criteria for the diagnosis of left ventricular phenotypic features. The key upgrade was the incorporation of tissue characterization findings by cardiac magnetic resonance for noninvasive detection of late‐gadolinium enhancement/myocardial fibrosis that are determinants for characterization of arrhythmogenic biventricular and left ventricular cardiomyopathy. The 2020 International criteria are heavily dependent on cardiac magnetic resonance, which has become mandatory to characterize the ACM phenotype and to exclude other diagnoses. New criteria regarding left ventricular depolarization and repolarization ECG abnormalities and ventricular arrhythmias of left ventricular origin were also provided. This article reviews the evolving approach to diagnosis of ACM, going back to the 1994 and 2010 International Task Force criteria and then grapple with the modern 2020 International criteria.

P1077Late gadolinium enhancement with cardiac magnetic resonance imaging demonstrates left ventricular involvement is under-recognized in arrhythmogenic right ventricular cardiomyopathy

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
S Mohamed ◽  
M Kunz ◽  
S Casey ◽  
W Katsiyiannis ◽  
R Abdelhadi ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Late Gadolinium Enhancement ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Fatty Tissue ◽  
Post Mortem ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement

Abstract Funding Acknowledgements The study is partially funded by Medtronic and the Minneapolis Heart Institute Foundation. Background/Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by replacement of the myocardium with fibrous and fatty tissue that may lead to an increased risk of ventricular arrhythmias and heart failure.  Although left ventricular (LV) and biventricular forms have been identified post-mortem resulting in the increased use of the term arrhythmogenic cardiomyopathy, there is only inclusion of right ventricular wall motion abnormalities in the taskforce diagnostic criteria. Purpose The aim of our study was to examine the utility of cardiac magnetic resonance (CMR) imaging in characterizing LV or biventricular involvement with late gadolinium enhancement (LGE) in a large cohort of patients with suspected ARVC. Methods Retrospective, single-institution, chart review of 76 patients diagnosed with ARVC between January 2009 and July 2019. Data collection and analysis included baseline demographics and parameters specific to diagnosis (definite, borderline, or possible) and risk stratification of ARVC based on 2019 modified taskforce criteria, as well as detailed CMR evaluation. Results Of the 76 patients with ARVC, 66 (87%) had at least one CMR with gadolinium administered. In that subset of patients, 27 (41%) had LGE. Of those with LGE, LV involvement was identified in 23 (85%) patients. The pattern of LGE was not localized to one myocardial region but demonstrated variable LV enhancement patterns including anterior, inferior, lateral, septal, basal, mid, apical, and from the sub-epicardium into the mid-myocardium. Conclusions   Left ventricular involvement reflected by LGE was identified in a high percentage of patients with suspected ARVC, and there was significant variation in the pattern of distribution in terms of region and depth of myocardial involvement.  While post-mortem examination of patients with ARVC demonstrates a high prevalence of left ventricular involvement, this study shows that CMR can consistently detect late gadolinium enhancement, and ARVC should be considered in the differential diagnosis for biventricular cardiomyopathy.  The identification of variable locations of LGE within the LV suggests there is more than one phenotype, and this imaging modality may help to clarify the implications of left ventricular involvement in disease progression.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

scholarly journals Utility of Cardiac Magnetic Resonance in the Evaluation of Unselected Patients with Possible Arrhythmogenic Right Ventricular Cardiomyopathy

2012 ◽  
Vol 6 ◽  
pp. CMC.S9996 ◽  
Author(s):  
Khang Li Looi ◽  
Colin Edwards ◽  
Hamish Hart ◽  
Jonathan P. Christiansen
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Fatty Infiltration ◽  
Venous Drainage ◽  
Ventricular Cardiomyopathy ◽  
Diagnostic Role ◽  
Characteristic Imaging

Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but important cause of sudden cardiac death. We investigated the role of cardiac magnetic resonance imaging (CMR) in the evaluation of patients with suspected ARVC referred by a general cardiology service. Methods Ninety-two patients (mean age 48 ± 15, 49% female), referred for CMR assessment of possible ARVC, were reviewed. CMR included both functional and tissue characteristic imaging. Results No patients had ARVC based on the 1994 Task Force Criteria (TFC) prior to CMR, but 4 met proposed Modified TFC; 15% met one major (±1 minor) TFC, 71% 1 or 2 minor TFC, and 14% no TFC. Reasons for CMR referral included symptomatic arrhythmia of likely RV origin (28%), Electrocardiogram/Holter abnormalities (28%), echocardiographic features suspicious of ARVC (19%), and family history of ARVC (8%). CMR findings strongly suggestive of ARVC were found in nine patients (10%), although only three were considered typical. Of these patients two met 1 major TFC and seven met 1 or 2 minor TFC. CMR findings included RV thinning, aneurysm, and diastolic out-pouching, but only 1 patient had definite fatty infiltration of the RV. Incidentally, CMR detected important, previously undiagnosed pathology, including anomalous pulmonary venous drainage (2 patients) and non-ischaemic cardiomyopathy (6%). CMR was normal in 63%, with minor abnormalities in 29%. Conclusions CMR may play an important diagnostic role in the evaluation of possible ARVC. Patients who do not meet TFC for diagnosis may have CMR features typical of ARVC. Additionally CMR may detect other hitherto undiagnosed structural or functional abnormalities that alter patient management. However the majority of patients referred have a low pretest probability of ARVC, and the rate of normal CMR scans is high.

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