Heart failure & cardiomyopathies

Arrhythmogenic cardiomyopathy diagnosis and management: a systematic review of clinical practice guidelines and recommendations with insights for future research

Eur Heart J Qual Care Clin OutcomesPublished systematic review of the most relevant guidelines and recommendations for arrhythmogenic cardiomyopathy (ACM) diagnosis and management

Authors' conclusion

Bridging these gaps could help guiding future research and guideline development towards improving patient outcomes.

Study context

Population
Clinical practice guidelines and expert consensus statements on arrhythmogenic cardiomyopathy (ACM)documents meeting inclusion criteria · guidelines two · consensus statements three
Outcome
Areas of agreement across documents
Methodological detailsMEDLINE, EMBASE, and resources from scientific societies over the last 10 years
  • MEDLINE, EMBASE, and resources from scientific societies over the last 10 years

Pooled result

As reported in the abstract

  • Key areas of agreement included the critical role of cardiac imaging for initial diagnosis and ongoing monitoring, genetic testing in index patients, ventricular arrhythmia management, catheter ablation indications, heart failure treatment strategies, and exercise recommendations

Further pooled results

Areas of difference across documents

Significant differences were found in definitions and diagnostic criteria for ACM, interpretation of phenocopies, management of family members, and criteria for implantable cardioverter defibrillator implantation
Additional discrepancies emerged regarding the role of multidisciplinary teams, non-cardiac surgical considerations, atrial fibrillation management, and reproductive issues

Evidence gaps

Considerable gaps in evidence, especially in areas such as the management and follow-up of patients with potential or borderline ACM diagnoses, as well as the care of their relatives
The clinical implications of genetic findings, along with the clinical management of left-dominant, biventricular phenotypes, and hot phases of disease, are also insufficiently addressed
Lack of externally validated risk assessment tools to guide clinical decision-making

Original publication

Arrhythmogenic cardiomyopathy diagnosis and management: a systematic review of clinical practice guidelines and recommendations with insights for future research. Eur Heart J Qual Care Clin Outcomes. Published 19 May 2025. doi:10.1093/ehjqcco/qcaf029

Summarised from the published abstract; every number is checked against it

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