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Scarring/arrhythmogenic cardiomyopathy. | LitMetric

AI Article Synopsis

  • Arrhythmogenic cardiomyopathy (S/ACM) is a heart muscle disease affecting both the right and left ventricles, characterized by loss of heart muscle tissue replaced by scar tissue, which increases the risk of dangerous heart rhythms and impacts heart function.
  • The term S/ACM includes various genetic or acquired conditions that show similar scarring features, and the updated "Padua criteria" help improve diagnosis by incorporating advanced imaging techniques.
  • Treatment focuses on managing arrhythmias and heart failure, with implantable cardioverter defibrillators (ICDs) being the only proven life-saving option, though choosing the right patients for this therapy poses significant challenges.

Article Abstract

The designation of 'arrhythmogenic cardiomyopathy' reflects the evolving concept of a heart muscle disease affecting not only the right ventricle (ARVC) but also the left ventricle (LV), with phenotypic variants characterized by a biventricular (BIV) or predominant LV involvement (ALVC). Herein, we use the term 'scarring/arrhythmogenic cardiomyopathy (S/ACM)' to emphasize that the disease phenotype is distinctively characterized by loss of ventricular myocardium due to myocyte death with subsequent fibrous or fibro-fatty scar tissue replacement. The myocardial scarring predisposes to potentially lethal ventricular arrhythmias and underlies the impairment of systolic ventricular function. S/ACM is an 'umbrella term' which includes a variety of conditions, either genetic or acquired (mostly post-inflammatory), sharing the typical 'scarring' phenotypic features of the disease. Differential diagnoses include 'non-scarring' heart diseases leading to either RV dilatation from left-to-right shunt or LV dilatation/dysfunction from a dilated cardiomyopathy. The development of 2020 upgraded criteria ('Padua criteria') for diagnosis of S/ACM reflected the evolving clinical experience with the expanding spectrum of S/ACM phenotypes and the advances in cardiac magnetic resonance (CMR) imaging. The Padua criteria aimed to improve the diagnosis of S/ACM by incorporation of CMR myocardial tissue characterization findings. Risk stratification of S/ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other ECG or imaging parameters may have a role. Medical therapy is crucial for treatment of ventricular arrhythmias and heart failure. Implantable cardioverter defibrillator (ICD) is the only proven life-saving treatment, despite its significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10132624PMC
http://dx.doi.org/10.1093/eurheartjsupp/suad017DOI Listing

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