{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T17:06:34Z","timestamp":1784739994163,"version":"3.55.0"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2021,10,5]],"date-time":"2021-10-05T00:00:00Z","timestamp":1633392000000},"content-version":"vor","delay-in-days":4,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Calmodulinopathy is an emerging group of primary electrical disease with various, severe, and early onset phenotype. Sudden cardiac arrest (SCA)\/death can be the first symptom and current medical management seems insufficient to prevent recurrences. Implantable cardioverter-defibrillator (ICD) in the young is challenging and can be harmful.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>We report the management of two very young boys (aged 3.5 and 5.5 years old) who survived an SCA due to calmodulin mutation responsible of a catecholaminergic polymorphic ventricular tachycardia phenotype. In both case, SCA had an adrenergic trigger. Despite SCA, ICD implantation was denied by the parents. After thorough discussion with the family, the patients were managed with solely betablocker treatment and loop recorder implantation. At last follow-up of 30 and 23 months, respectively, there were no recurrence of any cardiac event.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>The benefits of ICD implantation at a very young age must be weighed against the risk complication. In the youngest, whom recreative activities are under constant supervision, the decision, jointly made with the parents, could be to postpone ICD.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab393","type":"journal-article","created":{"date-parts":[[2021,10,5]],"date-time":"2021-10-05T22:22:08Z","timestamp":1633472528000},"source":"Crossref","is-referenced-by-count":2,"title":["Challenging indication of cardioverter defibrillator implantation after sudden cardiac arrest in the very young: a case series of catecholaminergic polymorphic ventricular tachycardia secondary to <i>de novo<\/i> calmodulin p.Asn98Ser"],"prefix":"10.1093","volume":"5","author":[{"given":"Alice","family":"Maltret","sequence":"first","affiliation":[{"name":"H\u00f4pital Marie Lannelongue-M3C, GHPSJ, Universit\u00e9 Paris Saclay, service de cardiologie cong\u00e9nitale, Le Plessis-Robinson, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3203-6158","authenticated-orcid":false,"given":"Fatima Azzahrae","family":"Benaich","sequence":"additional","affiliation":[{"name":"M3C-Necker, H\u00f4pital Universitaire Necker-Enfants Malades, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0377-0807","authenticated-orcid":false,"given":"John","family":"Rendu","sequence":"additional","affiliation":[{"name":"Centre Hospitalier Universitaire Grenoble Alpes, Laboratoire de Biochimie et G\u00e9n\u00e9tique Mol\u00e9culaire, Univ. Grenoble Alpes, Inserm U1216, Grenoble Institut Neurosciences, Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Fressart","sequence":"additional","affiliation":[{"name":"Groupe Hospitalier Piti\u00e9-Salp\u00eatri\u00e8re, Service de Biochimie M\u00e9tabolique, Unit\u00e9 de Cardiog\u00e9n\u00e9tique et Myog\u00e9n\u00e9tique, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3005-1373","authenticated-orcid":false,"given":"Nathalie","family":"Roux-Buisson","sequence":"additional","affiliation":[{"name":"Centre Hospitalier Universitaire Grenoble Alpes, Laboratoire de Biochimie et G\u00e9n\u00e9tique Mol\u00e9culaire, Univ. Grenoble Alpes, Inserm U1216, Grenoble Institut Neurosciences, Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8722-5805","authenticated-orcid":false,"given":"Damien","family":"Bonnet","sequence":"additional","affiliation":[{"name":"M3C-Necker, H\u00f4pital Universitaire Necker-Enfants Malades, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1786-9461","authenticated-orcid":false,"given":"Isabelle","family":"Denjoy","sequence":"additional","affiliation":[{"name":"CNMR, Maladies Cardiaques H\u00e9r\u00e9ditaires Rares, Service de cardiologie, H\u00f4pital Bichat, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,10,5]]},"reference":[{"key":"2022052609542969500_ytab393-B1","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1161\/CIRCGENETICS.115.001370","article-title":"Whole-exome molecular autopsy after exertion-related sudden unexplained death in the young","volume":"9","author":"Anderson","year":"2016","journal-title":"Circ Cardiovasc Genet"},{"key":"2022052609542969500_ytab393-B2","doi-asserted-by":"crossref","first-page":"1677","DOI":"10.1016\/j.jacc.2017.10.053","article-title":"2017 AHA\/ACC\/HRS Guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: executive summary: a report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society","volume":"72","author":"Al-Khatib","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"2022052609542969500_ytab393-B3","doi-asserted-by":"crossref","first-page":"2964","DOI":"10.1093\/eurheartj\/ehz311","article-title":"Calmodulin mutations and life-threatening cardiac arrhythmias: insights from the International Calmodulinopathy Registry","volume":"40","author":"Crotti","year":"2019","journal-title":"Eur Heart J"},{"key":"2022052609542969500_ytab393-B4","first-page":"1601","article-title":"2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). 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