Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent \"non-ischemic\" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.e., biventricular (ABVC) and arrhythmogenic left ventricular cardiomyopathy (ALVC). The ARVC phenotype together with these left-sided variants are now more appropriately named ACM. The lack of diagnostic criteria for the left ventricular (LV) phenotype has resulted in clinical under-recognition of ACM patients over the 4 decades since the disease discovery. In 2020, the \"Padua criteria\" were proposed for both right- and left-sided ACM phenotypes. The presently proposed criteria represent a refinement of the 2020 Padua criteria and have been developed by an expert European TF to improve the diagnosis of ACM with upgraded and internationally recognized criteria. The growing recognition of the diagnostic role of CMR has led to the incorporation of myocardial tissue characterization findings for detection of myocardial scar using the late‑gadolinium enhancement (LGE) technique to more fully characterize right, biventricular and left disease variants, whether genetic or acquired (phenocopies), and to exclude other \"non-scarring\" myocardial disease. The \"ring-like\' pattern of myocardial LGE/scar is now a recognized diagnostic hallmark of ALVC. Additional diagnostic criteria regarding LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias of LV origin are also provided. These proposed upgrading of diagnostic criteria represents a working framework to improve management of ACM patients.

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Cardiomyopathies are a group of heterogeneous diseases which can be caused by various factors (often genetic) and can lead to heart failure, arrhythmia and sudden death. Primary cardiomyopathies includes hereditary hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, mitochondrial cardiomyopathy, mixed (hereditary and acquired) dilated cardiomyopathy and restrictive cardiomyopathy, left ventricular densification insufficiency, and other unclassified cardiomyopathies. With the help of genomic technology, common mutations in the population have been identified. In vivo and in vitro study of such mutations has provided insight into the pathogenesis and treatment of these diseases. The compilation of this guideline is based on the consensus of basic and clinical research and guidelines from other countries, and has summarized the phenotype, diagnosis, treatment and consultation of various types of genetic cardiomyopathies with an aim to standardize the clinical management of patients.

Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

The article presents key points of published in March 2017, Guideline for the Evaluation and Management of Patients With Syncope (A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society). This document contains summary and analysis of evidence base and accumulated experience in this field and reflect further development of the problem of the management of adult and pediatric patients with suspected syncope. Traditionally, the basis of diagnostic algorithm of these patients has been primary examination, and in the present guideline one can find strengthening of the importance of anamnestic criteria, and supplementation of ECG criteria of arrhythmic syncope. At the same time, the diagnostic value of provocative tests has been revised, and the class of indications for these tests has been lowered. According to the guideline, along with diagnosis of the cause of fainting, risk stratifcation of short- and long-term adverse outcomes has a key value. The guideline summarizes latest achievements in the treatment of fainting in sarcoidosis, arrhythmogenic right ventricular dysplasia, primary arrhythmic heart diseases, orthostatic hypotension, and reflex-mediated syncope. Despite orientation on specifc properties of medical care in the USA, the guideline can be useful to practical physicians in choice of the best strategy of diagnosis and treatment of syncope in each individual case.

Ventricular arrhythmias are a feared complication of arrhythmogenic right ventricular dysplasia/cardiomyopathy. In 2015, an International Task Force Consensus Statement proposed a risk stratification algorithm for implantable cardioverter-defibrillator placement in arrhythmogenic right ventricular dysplasia/cardiomyopathy.
To evaluate performance of the algorithm, 365 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients were classified as having a Class I, IIa, IIb, or III indication per the algorithm at baseline. Survival free from sustained ventricular arrhythmia (VT/VF) in follow-up was the primary outcome. Incidence of ventricular fibrillation/flutter cycle length <240 ms was also assessed. Two hundred twenty-four (61%) patients had a Class I implantable cardioverter-defibrillator indication; 80 (22%), Class IIa; 54 (15%), Class IIb; and 7 (2%), Class III. During a median 4.2 (interquartile range, 1.7-8.4)-year follow-up, 190 (52%) patients had VT/VF and 60 (16%) had ventricular fibrillation/flutter. Although the algorithm appropriately differentiated risk of VT/VF, incidence of VT/VF was underestimated (observed versus expected: 29.6 [95% confidence interval, 25.2-34.0] versus >10%/year Class I; 15.5 [confidence interval 11.1-21.6] versus 1% to 10%/year Class IIa). In addition, the algorithm did not differentiate survival free from ventricular fibrillation/flutter between Class I and IIa patients (P=0.97) or for VT/VF in Class I and IIa primary prevention patients (P=0.22). Adding Holter results (<1000 premature ventricular contractions/24 hours) to International Task Force Consensus classification differentiated risks.
While the algorithm differentiates arrhythmic risk well overall, it did not distinguish ventricular fibrillation/flutter risks of patients with Class I and IIa implantable cardioverter-defibrillator indications. Limited differentiation was seen for primary prevention cases. As these are vital uncertainties in clinical decision-making, refinements to the algorithm are suggested prior to implementation.

Arrhythmogenic cardiomyopathy (AC) is a progressive disease with high risk of life-threatening ventricular arrhythmias. A genetic mutation is found in up to 50-60% of probands, mostly affecting desmosomal genes. Diagnosis of AC is made by a combination of data from different modalities including imaging, electrocardiogram, Holter monitoring, family history, genetic testing, and tissue properties. Being a progressive cardiomyopathy, repeated cardiac imaging is needed in AC patients. Repeated imaging is important also for risk assessment of ventricular arrhythmias. This expert consensus document gives clinical recommendations for how to use multi-modality imaging in the different aspects of AC disease, including diagnosis, family screening, follow-up, risk assessment, and differential diagnosis.

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