{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T23:11:23Z","timestamp":1790896283477,"version":"4.1.0"},"reference-count":145,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T00:00:00Z","timestamp":1787875200000},"content-version":"vor","delay-in-days":27,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,8,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Very rare and ultra-rare primary inherited arrhythmia syndromes (IAS) represent a heterogeneous group of disorders associated with a significant risk of sudden cardiac death, often manifesting from foetal life to early adulthood. Current guidelines primarily address more common IAS and provide limited, non-specific recommendations for these rare entities, particularly in paediatric populations. This European Heart Rhythm Association Clinical Consensus Statement, developed in collaboration with the Association of Cardiovascular Nursing and Allied Professions and endorsed by the Association for European Paediatric and Congenital Cardiology, integrates available evidence with expert opinion. Recommendations were formulated through structured discussion and voting, following ESC consensus methodology, with a focus on clinically actionable gene-disease associations. The document provides a comprehensive framework for the diagnosis and management of very rare IAS, including calmodulinopathies, Andersen\u2013Tawil syndrome, Timothy syndrome, TRDN-related disease, calcium release deficiency syndrome, and other atypical channelopathies. It highlights age-specific clinical presentations, the importance of genetic testing, and tailored therapeutic strategies, including pharmacological treatments, left cardiac sympathetic denervation, and selective use of implantable cardioverter-defibrillators. 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. This consensus document fills a critical gap by providing expert-driven, pragmatic guidance for the management of very rare IAS across the lifespan. 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.<\/jats:p>","DOI":"10.1093\/europace\/euag184","type":"journal-article","created":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T12:45:31Z","timestamp":1787921131000},"source":"Crossref","is-referenced-by-count":1,"title":["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 &amp; Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6857-8904","authenticated-orcid":false,"given":"Georgia","family":"Sarquella-Brugada","sequence":"first","affiliation":[{"name":"Pediatric Arrhythmias, Genetic Cardiology and Sudden Death, Institut de Recerca Sant Joan de D\u00e9u (IRSJD) , Santa Rosa 39-57, Esplugues de Llobregat, Barcelona 08950 ,","place":["Spain"]},{"name":"Arrhythmia, Inherited Cardiac Diseases and Sudden Death Unit, Department of Cardiology, Hospital Sant Joan de D\u00e9u Barcelona , Passeig Sant Joan de D\u00e9u 2, Esplugues de Llobregat, Barcelona 08950 ,","place":["Spain"]},{"name":"Departament de Pediatria, Facultat de Medicina i Ci\u00e8ncies M\u00e8diques, Universitat de Barcelona , Barcelona ,","place":["Spain"]},{"name":"Medical Sciences Department, School of Medicine, University of Girona , Girona ,","place":["Spain"]},{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart\u2014ERN GUARD-Heart"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0208-2172","authenticated-orcid":false,"given":"Andrea","family":"Mazzanti","sequence":"additional","affiliation":[{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart\u2014ERN GUARD-Heart"},{"name":"Molecular Cardiology, IRCCS Salvatore Maugeri Foundation , Pavia ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9428-4934","authenticated-orcid":false,"given":"Anwar","family":"Baban","sequence":"additional","affiliation":[{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart\u2014ERN GUARD-Heart"},{"name":"Genetic Department, Mediclinic Middle East Hospitals , Dubai Healthcare City, Building 37 PO Box 505004, Dubai ,","place":["UAE"]},{"name":"Cardiogenetic Centre Rare Diseases and Medical Genetics Units Bambino Ges\u00f9 Children Hospital & Research Institute IRCCS , Rome ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5298-5276","authenticated-orcid":false,"given":"Oscar","family":"Campuzano","sequence":"additional","affiliation":[{"name":"Medical Sciences Department, School of Medicine, University of Girona , Girona ,","place":["Spain"]},{"name":"Institut d'Investigaci\u00f3 Biom\u00e8dica de Girona Dr. Josep Trueta , Salt ,","place":["Spain"]},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red. 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