ArticleArrhythmia & electrophysiology review2016
Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes.
Article in Arrhythmia & electrophysiology review, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 56 citations in OpenAlex.
- Advances in VT ablation for arrhythmogenic cardiomyopathy: evidence, mapping and ablation strategies, and predictive factors.Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing · 2026Review
- Genotype-phenotype correlations inFrontiers in cardiovascular medicine · 2026Article
- Molecular Pathogenesis of Arrhythmogenic Cardiomyopathy: Mechanisms and Therapeutic Perspectives.Biomolecules · 2025Review
- Transmembrane Protein 43: Molecular and Pathogenetic Implications in Arrhythmogenic Cardiomyopathy and Various Other Diseases.International journal of molecular sciences · 2025Review
- Hypermobile Ehlers-Danlos Syndrome: Diagnostic Challenges and the Role of Genetic Testing.Genes · 2025Article
- Right heart strain in arrhythmogenic right ventricular cardiomyopathy: implications for cardiovascular outcome.European heart journal. Cardiovascular Imaging · 2024Article
- Ventricular predominance in biventricular arrhythmogenic cardiomyopathy: Should new subtype criteria be recognized?Radiology case reports · 2024Article
- Prevention of Protease-Induced Degradation of Desmoplakin via Small Molecule Binding.Journal of personalized medicine · 2024Article
- Arrhythmogenic Cardiomyopathy: from Preclinical Models to Genotype-phenotype Correlation and Pathophysiology.Stem cell reviews and reports · 2023Review
- Article
- HumanizedCells · 2022Article
- Arrhythmogenic Cardiomyopathy: Exercise Pitfalls, Role of Connexin-43, and Moving beyond Antiarrhythmics.International journal of molecular sciences · 2022Review
- Pharmacologic Management for Ventricular Arrhythmias: Overview of Anti-Arrhythmic Drugs.Journal of clinical medicine · 2022Review
- Creating a 'Molecular Band-Aid'; Blocking an Exposed Protease Target Site in Desmoplakin.Journal of personalized medicine · 2021Article
- The Time Has Come to Explore Plasma Biomarkers in Genetic Cardiomyopathies.International journal of molecular sciences · 2021Review
- Phenotypic Variability of a PathogenicFrontiers in cardiovascular medicine · 2021Article
- ECG Abnormalities in Patients with Acute Exacerbation of Bronchiectasis and Factors Associated with High Probability of Abnormality.Pulmonary medicine · 2021Observational
- Distinct Myocardial Transcriptomic Profiles of Cardiomyopathies Stratified by the Mutant Genes.Genes · 2020Article
- Familial Arrhythmogenic Cardiomyopathy: Clinical Determinants of Phenotype Discordance and the Impact of Endurance Sports.Journal of clinical medicine · 2020Article
- Established and Emerging Mechanisms in the Pathogenesis of Arrhythmogenic Cardiomyopathy: A Multifaceted Disease.International journal of molecular sciences · 2020Review
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes. At later stages, heart failure may occur. Diagnosis is established with the 2010 Task Force Criteria (TFC). Modern imaging tools are crucial for ACM diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting functional and structural alternations. Of note, structural findings often become visible after electrical alterations, such as premature ventricular beats, ventricular fibrillation (VF) and ventricular tachycardia (VT). 12-lead ECG is important to assess for depolarisation and repolarisation abnormalities, including T-wave inversions as the most common ECG abnormality. Family history and the detection of causative mutations, mostly affecting the desmosome, have been incorporated in the TFC, and stress the importance of cascade family screening. Differential diagnoses include idiopathic right ventricular outflow tract (RVOT) VT, sarcoidosis, congenital heart disease, myocarditis, dilated cardiomyopathy, athlete's heart, Brugada syndrome and RV infarction. Therapeutic strategies include restriction from endurance and competitive sports, β-blockers, antiarrhythmic drugs, heart failure medication, implantable cardioverter-defibrillators and endocardial/epicardial catheter ablation.
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Registered trials
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