Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy

Published: May 2019
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  • Key Points
  • Graded Treatment Recommendations
    • Who to study
    • Limitations of genetic testing
    • Family history
    • Age-related penetrance of disease in at-risk relatives
    • Cascade cardiac investigations
    • Cascade genetic testing
    • Risk stratification and ICD decisions
    • Medical therapies for right ventricular failure
    • Antithrombotic therapy in ACM
    • Arrhythmia management
    • Role of catheter ablation
    • Exercise and other ACM
    • Amyloidosis
    • Left ventricular noncompaction treatment
  • Tables
    • Classification of likelihood of pathogenicity of a variant
    • Different methods for screening genes
    • Minimal set of genes to be prioritized in ACM
    • Diagnostic criteria for left ventricular noncompaction
  • Figures
    • Algorithm to consider the presence of ACM
    • Arrhythmogenic cardiomyopathy
    • Common pathway and genetic mutations in a patient with ACM according to the predominant ventricular dysfunction
    • Cytoskeletal protein complexes within the cardiomyocyte costamere and Z-disk
    • Modified Task Force criteria for ARVC – diagnostic categories major and minor criteria
    • Representative 12-lead ECG obtained from ARVC patients with IRBBB and CRBBB
    • Cumulative prevalence of disease expression in family members at risk of ARVC
    • Genetic testing algorithm
    • Summary of cascade family screening algorithm
    • Implantable cardioverter defibrillator algorithm
    • Algorithm for ventricular dysfunction and antithrombotic medical therapy in individuals with arrhythmogenic cardiomyopathy
    • Medical therapy algorithm for arrhythmias
    • Catheter ablation algorithm for individuals with ACM
    • Exercise algorithm for individuals with ARVC
    • METS associated with common types of endurance exercise
    • Disease mechanisms for ACM
    • Amyloidosis arrhythmia treatment algorithm
    • Diagnosis and risk stratification of LVNC (a) and family and genetic evaluation of LVNC (b)
    • LVNC treatment algorithm
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The Heart Rhythm Society (HRS) is a leading resource on cardiac pacing and electrophysiology. This specialty organization represents medical, allied health, and science professionals from more than 70 countries who specialize in cardiac rhythm disorders.

Description

This pocket guide attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. This pocket guide should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool.

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