[Diagnosis of arrhythmogenic cardiomyopathy: 20 years of progress and innovation]
Domenico Corrado1, Alessandro Zorzi1, Barbara Bauce1
1Dipartimento di Scienze Cardio-Toraco-Vascolari e Sanità Pubblica, Università Degli Studi, Padova.
Insights
Arrhythmogenic cardiomyopathy (CMA) diagnosis has evolved significantly. Recent European Task Force criteria (2023) refine diagnosis by incorporating left ventricular variants and cardiac MRI for scarring, improving understanding of this cardiac disease.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Arrhythmogenic cardiomyopathy (CMA) is a challenging cardiac disease defined by ventricular scarring and electrical instability.
- Current diagnostic methods rely on criteria sets, as no single test is definitive.
- Previous criteria (1994, 2010) primarily focused on right ventricular involvement.
Purpose of the Study:
- To review the historical evolution of diagnostic criteria for arrhythmogenic cardiomyopathy from 1994 to 2023.
- To highlight advancements in understanding CMA's pathobiology and morpho-functional characteristics.
- To analyze the impact of new criteria, including the 2020 Padua and 2023 European Task Force criteria.
Main Methods:
- Historical review of diagnostic criteria for arrhythmogenic cardiomyopathy.
- Analysis of changes and progress in diagnostic approaches over 20 years.
- Examination of the role of cardiac magnetic resonance imaging in characterizing myocardial scarring.
Main Results:
- Diagnostic criteria have evolved from focusing on right ventricular involvement to including left ventricular variants.
- The 2020 Padua criteria and 2023 European Task Force criteria represent significant advancements.
- Introduction of the "scarring/arrhythmogenic cardiomyopathy" concept enhances understanding of diverse phenotypes.
Conclusions:
- The diagnostic landscape for CMA has substantially improved, reflecting increased knowledge of its pathobiology.
- The 2023 European Task Force criteria offer a more comprehensive approach to diagnosing CMA.
- Recognizing diverse phenotypes and the role of myocardial scarring is crucial for accurate CMA diagnosis and management.
Abstract:
Arrhythmogenic cardiomyopathy (CMA) is a cardiac disease characterized by non-ischemic ventricular scarring and electrical instability. The diagnosis of CMA still remains challenging today and requires the use of a set of criteria, since no single diagnostic test represents the gold standard. The first diagnostic criteria were defined and disseminated in 1994 and then revised in 2010, focusing mainly on right ventricular involvement. In 2019, an international panel of experts identified the limitations of the previous diagnostic criteria. The 2020 Padua criteria included a specific pathway for the diagnosis of left ventricular variants and emphasized the need for the use of cardiac magnetic resonance imaging in the characterization of myocardial scarring. These criteria were further refined and published in 2023 as European Task Force (TF) criteria, thus gaining international recognition.Exploring the history of CMA and its diagnosis, in this review we analyze the changes and progress in the 20 years that have occurred from the first version of the criteria in 1994 to the latest in European TF of 2023, highlighting the evolution of our knowledge of the pathobiology and morpho-functional characteristics of the disease. One of the most relevant updates is undoubtedly the introduction of the concept of "scarring/arrhythmogenic cardiomyopathy", a definition that enhances the main features of the pathology and emphasizes the multiplicity of phenotypes and clinical presentations independent of etiology.
Related Concept Videos
01:30Cardiomyopathy II: Dilated Cardiomyopathy
01:29Cardiomyopathy V: Interprofessional Care
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
01:30Dysrhythmias V: Evaluating Dysrhythmias
01:27Myocarditis II: Clinical features and Diagnostic Tests