Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical…

GuidelineEuropace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology· Aug 2026

Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical Consensus Statement of the European Heart Rhythm Association of the ESC and the Association of Cardiovascular Nursing & Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology.

Georgia Sarquella-Brugada, Andrea Mazzanti, Anwar Baban … Elena Arbelo

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Pediatric CardiologyGuidelineClinical practice

Consensus statement provides guidance for very rare inherited arrhythmia syndromes.

Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical Consensus Statement of the European Heart Rhythm Association of the ESC and the Association of Cardiovascular Nursing & Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology.

Georgia Sarquella-Brugada … Elena Arbelo

Europace · Aug 2026

Background

Very rare and ultra-rare primary inherited arrhythmia syndromes (inherited arrhythmia syndromes) represent a heterogeneous group of disorders associated with a significant risk of sudden cardiac death, often manifesting from foetal life to early adulthood.

Methods

It underscores the need for specialized care, international collaboration, and prospective registries to improve evidence generation, risk stratification, and patient outcomes in this vulnerable population.

More results

Special attention is given to paediatric considerations, foetal diagnosis, and the role of multidisciplinary care.

The document also addresses arrhythmic risk in metabolic and cardiomyopathic conditions, as well as the importance of molecular autopsy and family screening in sudden unexplained death.

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Conclusion

This consensus document fills a critical gap by providing expert-driven, pragmatic guidance for the management of very rare inherited arrhythmia syndromes across the lifespan.

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