{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:50:25Z","timestamp":1753876225530,"version":"3.41.2"},"reference-count":8,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2022,10,28]],"date-time":"2022-10-28T00:00:00Z","timestamp":1666915200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Genetic abnormalities causing various arrhythmias including atrial arrhythmias, specialized cardiac conduction disorders, and malignant ventricular arrhythmias have been reported. However, it is sometimes difficult to diagnose and treat patients with various arrhythmias.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 49-year-old woman who underwent ablation of typical atrial flutter (AFL) at 31 years of age visited the emergency room due to a cardiopulmonary arrest. Her 12-lead electrocardiogram during sinus rhythm after resuscitation exhibited first-degree atrioventricular block with right bundle branch block and right axis deviation. No structural heart disease was evident on standard imaging screening. An implantation of a single-chamber implantable cardioverter defibrillator (ICD) was indicated. After the ICD implantation, she then experienced multiple ventricular fibrillation (VF) episodes. Radiofrequency catheter ablation of triggered ventricular premature contractions (VPCs) was performed but failed because the clinical VPCs could not be induced during the session. Although no pathogenic variants associated with Brugada syndrome or long-QT syndrome were found, a rare HCN4 variant, c.1209+2_1209+3insGAGT (rs786205418), was identified in a gene panel analysis. Because high-frequency clinical pacing was effective for suppressing the VF, the single-chamber ICD was upgraded to a dual-chamber ICD. Thereafter, high-rate pacing successfully prevented any further ventricular arrhythmias during the follow up.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>A clinical course with prominent wide QRS complexes and AFL in one\u2019s early 30s followed by sudden onset of a VF storm about 20 years later is extremely rare. Her clinical phenotype expression was possibly associated with a rare HCN4 variant; however, further study is needed to confirm whether this variant was pathological or not.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytac431","type":"journal-article","created":{"date-parts":[[2022,10,28]],"date-time":"2022-10-28T19:07:26Z","timestamp":1666984046000},"source":"Crossref","is-referenced-by-count":0,"title":["Case report of a ventricular fibrillation storm with a cardiac conduction disorder and HCN4 variant 18 years after ablation of atrial flutter"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0521-3008","authenticated-orcid":false,"given":"Toshihiro","family":"Nakamura","sequence":"first","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine , Kobe , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4276-479X","authenticated-orcid":false,"given":"Koji","family":"Fukuzawa","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine , Kobe , Japan"},{"name":"Section of Arrhythmia, Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine , Kusunoki-Cho 7-5-2, Chuoh-Ku, Kobe, Hyogo , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeshi","family":"Aiba","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center , Osaka , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1209-8896","authenticated-orcid":false,"given":"Seiko","family":"Ohno","sequence":"additional","affiliation":[{"name":"Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center , Osaka , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,10,29]]},"reference":[{"key":"2022110917583190500_ytac431-B1","doi-asserted-by":"crossref","first-page":"2242","DOI":"10.1016\/S0735-1097(00)00982-7","article-title":"Incidence and predictors of atrial flutter in the general population","volume":"36","author":"Granada","year":"2000","journal-title":"J Am College Cardiol"},{"key":"2022110917583190500_ytac431-B2","doi-asserted-by":"crossref","first-page":"1080","DOI":"10.1161\/CIRCULATIONAHA.107.189420","article-title":"Sleep apnea and cardiovascular disease: an American Heart Association\/American College of Cardiology Foundation Scientific Statement from the American Heart Association Council for High Blood Pressure Research Professional Education Committee, Council on Clinical Cardiology, Stroke Council, and Council on Cardiovascular Nursing. 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