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Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Application of current diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy in every day clinical
Eleanor C Wicks, Perry M Elliott1
1The Heart Hospital, 16-18 Westmoreland Street, London, W1G 8PH. eleanor.wicks@nhs.net.
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart condition causing sudden death. This review examines revised diagnostic criteria for ARVC, highlighting the need for ongoing improvements in accuracy.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease linked to sudden cardiac death, ventricular arrhythmias, and heart failure.
- It primarily affects the desmosome, with mutations in desmosomal protein genes found in about 50% of patients, leading to fibrofatty replacement of cardiomyocytes and ventricular dilation.
Purpose of the Study:
- To review the application of revised clinical diagnostic criteria for ARVC in practice.
- To highlight the necessity for continued refinement of these criteria to enhance their diagnostic sensitivity and specificity.
Main Methods:
- Review of current literature and clinical practice guidelines regarding ARVC diagnosis.
- Analysis of the revised diagnostic criteria, considering pathology, genetics, and clinical presentation.
Main Results:
- The revised criteria provide a framework for ARVC diagnosis, integrating multiple factors.
- Application in practice reveals areas where sensitivity and specificity can be further improved.
Conclusions:
- The current revised criteria for diagnosing arrhythmogenic right ventricular cardiomyopathy are a significant step forward.
- Continuous modification and validation are essential to optimize the diagnostic accuracy for this inherited cardiac condition.
Abstract:
Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias and cardiac failure. It is primarily a disease of the desmosome, with mutations in desmosomal protein genes identified in approximately 50% of affected individuals. Mutations result in fibrofatty replacement of cardiomyocytes, aneurysm formation and dilatation of the right and often the left ventricle. The clinical diagnosis of ARVC is based upon complex criteria that consider pathology, genetics and clinical presentation. This review describes the application of the revised criteria for ARVC in everyday practice and illustrates the requirement for continued modification to improve their sensitivity and specificity.
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