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2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic

Jeffrey A Towbin1, William J McKenna2, Dominic J Abrams3

  • 1Le Bonheur Children's Hospital, Memphis, Tennessee; University of Tennessee Health Science Center, Memphis, Tennessee.

Heart Rhythm
|May 13, 2019
PubMed

Insights

Arrhythmogenic cardiomyopathy (ACM) is a complex heart muscle disorder with diverse causes, including genetic and inflammatory conditions. This consensus provides clinical guidance on evaluating and managing ACM, focusing on exercise recommendations for arrhythmogenic right ventricular cardiomyopathy.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic cardiomyopathy (ACM) encompasses a range of myocardial disorders not caused by ischemia, hypertension, or valvular disease.
  • It includes conditions like arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction.
  • ACM shares overlapping phenotypes with other cardiomyopathies, notably dilated cardiomyopathy presenting with arrhythmias.

Purpose of the Study:

  • To provide clinicians with expert guidance on the evaluation and management of arrhythmogenic cardiomyopathy (ACM).
  • To offer clinically relevant information on the genetics and disease mechanisms underlying ACM.
  • To develop evidence-based recommendations for exercise in arrhythmogenic right ventricular cardiomyopathy using PICO questions.

Main Methods:

  • Utilized PICO (Population, Intervention, Comparison, Outcome) questions to assess contemporary evidence.
  • Conducted a systematic literature search with evidence tables.
  • Developed recommendations based on expert consensus, literature review, and clinical experience, using the ACC/AHA Class of Recommendation and Level of Evidence system.

Main Results:

  • Recommendations for ACM evaluation and management were developed.
  • Specific guidance on exercise in arrhythmogenic right ventricular cardiomyopathy was formulated.
  • The consensus statement integrates genetic insights and disease mechanisms.

Conclusions:

  • ACM is a heterogeneous group of disorders requiring comprehensive evaluation and management strategies.
  • Understanding the genetic basis of ACM is crucial for elucidating disease triggers and pathways.
  • This expert consensus provides a framework for evidence-based clinical practice in ACM.

Keywords:
Arrhythmogenic cardiomyopathyArrhythmogenic left ventricular cardiomyopathyArrhythmogenic right ventricular cardiomyopathyCascade family screeningCatheter ablationDiagnosis of arrhythmogenic cardiomyopathyDisease mechanismsElectrophysiologyExercise restrictionGenetic testingGenetic variantsICD decisionsLeft ventricular noncompactionRisk stratificationTreatment of arrhythmogenic cardiomyopathy

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