{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T22:14:38Z","timestamp":1790720078535,"version":"4.1.0"},"reference-count":26,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Familial ST-depression syndrome is an inherited disease characterized by persistent, nonischemic ST-deviations, and risk of arrhythmias and heart failure. We aimed at further characterizing the ECG, arrhythmias, and structural characteristics associated with this novel syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Retrospective analysis of data from consecutive families with familial ST-depression Syndrome in Denmark. ECG features, prevalence and type of arrhythmias, occurrence of systolic dysfunction, and medium-term outcome were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Forty affected individuals (43% men; mean age at diagnosis 49.1 years) from 14 apparently unrelated families with \u22652 affected members were included. Autosomal dominant inheritance was observed in all families. The ECG phenotype seemed to develop in prepuberty and the ST-deviations were persistent and most pronounced in leads V4\/V5\/II, respectively. Serial ECG analyses showed stable to slow progression of the ECG phenotype. Exercise accentuated the ST-deviations with a maximum difference between rest\/stress (mean) of \u2212117 \u03bcV in lead V5. During a mean follow-up of 9.3\u00b17.1 years 5 (13%) patients developed sustained ventricular arrhythmias or (aborted) sudden cardiac death, 10 (25%) developed atrial fibrillation, 2 (5%) other supraventricular arrhythmias, and 10 (25%) were diagnosed with left ventricular ejection fraction \u226450%. The ventricular arrhythmias were polymorphic with relatively short-coupled premature ventricular contractions at onset (300\u2013360 ms); no QT prolongation was observed. Seven patients had at least one catheter ablation; 5 for supraventricular arrhythmias and 2 for ventricular arrhythmias. Males experienced more arrhythmic end points than females (\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.01).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>The familial ST-depression ECG phenotype is stable to slowly progressive after medium-term follow-up. Clinically, both supra- and ventricular arrhythmias are common; as are some degree of left ventricular systolic dysfunction. Familial ST-depression represent a novel inherited cause of polymorphic ventricular tachycardia.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circep.121.010688","type":"journal-article","created":{"date-parts":[[2022,3,31]],"date-time":"2022-03-31T11:07:56Z","timestamp":1648724876000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":14,"title":["Electrocardiographic Findings, Arrhythmias, and Left Ventricular Involvement in Familial ST-Depression Syndrome"],"prefix":"10.1161","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6855-1496","authenticated-orcid":true,"given":"Alex H\u00f8rby","family":"Christensen","sequence":"first","affiliation":[{"name":"The Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark &amp; Department of Clinical Medicine (A.H.C., C.R.V., J.T.-H., S.P., H.B.), The Heart Centre, Rigshospitalet, University of Copenhagen."},{"name":"Department of Cardiology, Herlev-Gentofte Hospital (A.H.C., A.P., K.K.I.), Copenhagen University Hospital."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0834-206X","authenticated-orcid":true,"given":"Christoffer Rasmus","family":"Vissing","sequence":"additional","affiliation":[{"name":"The Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark &amp; Department of Clinical Medicine (A.H.C., C.R.V., J.T.-H., S.P., H.B.), The Heart Centre, Rigshospitalet, University of Copenhagen."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0225-8657","authenticated-orcid":true,"given":"Adrian","family":"Pietersen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Herlev-Gentofte Hospital (A.H.C., A.P., K.K.I.), Copenhagen University Hospital."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3895-9316","authenticated-orcid":true,"given":"Jacob","family":"Tfelt-Hansen","sequence":"additional","affiliation":[{"name":"The Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark &amp; Department of Clinical Medicine (A.H.C., C.R.V., J.T.-H., S.P., H.B.), The Heart Centre, Rigshospitalet, University of Copenhagen."},{"name":"Department of Forensic Medicine, Faculty of Medical Sciences (J.T.-H.), The Heart Centre, Rigshospitalet, University of Copenhagen."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5622-9347","authenticated-orcid":true,"given":"Thomas","family":"Hartvig Lindk\u00e6r Jensen","sequence":"additional","affiliation":[{"name":"Department of Pathology, Rigshospitalet (T.H.L.J.), Copenhagen University Hospital."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7149-4613","authenticated-orcid":true,"given":"Steen","family":"Pehrson","sequence":"additional","affiliation":[{"name":"The Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark &amp; Department of Clinical Medicine (A.H.C., C.R.V., J.T.-H., S.P., H.B.), The Heart Centre, Rigshospitalet, University of Copenhagen."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1192-532X","authenticated-orcid":true,"given":"Finn Lund","family":"Henriksen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Odense University Hospital (F.L.H., N.C.F.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2876-7388","authenticated-orcid":true,"given":"Niels Christian Foldager","family":"Sandgaard","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Odense University Hospital (F.L.H., N.C.F.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0504-8487","authenticated-orcid":true,"given":"Kasper Karmark","family":"Iversen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Herlev-Gentofte Hospital (A.H.C., A.P., K.K.I.), Copenhagen University Hospital."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1802-4302","authenticated-orcid":true,"given":"Henrik Kj\u00e6rulf","family":"Jensen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Aarhus University Hospital (H.K.J.)."},{"name":"Department of Clinical Medicine, Health, Aarhus University, Aarhus, Denmark (H.K.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9477-5322","authenticated-orcid":true,"given":"Morten Salling","family":"Olesen","sequence":"additional","affiliation":[{"name":"Laboratory for Molecular Cardiology (M.S.O.), The Heart Centre, Rigshospitalet, University of Copenhagen."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0563-7049","authenticated-orcid":true,"given":"Henning","family":"Bundgaard","sequence":"additional","affiliation":[{"name":"The Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark &amp; Department of Clinical Medicine (A.H.C., C.R.V., J.T.-H., S.P., H.B.), The Heart Centre, Rigshospitalet, University of Copenhagen."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(57)90079-0"},{"key":"e_1_3_3_3_2","first-page":"656","article-title":"[rare cardiac arrythmias of the pediatric age. ii. syncopal attacks due to paroxysmal ventricular fibrillation. (presentation of 1st case in italian pediatric literature)].","volume":"45","author":"Romano C","year":"1963","unstructured":"Romano, C, Gemme, G, Pongiglione, R. [rare cardiac arrythmias of the pediatric age. ii. syncopal attacks due to paroxysmal ventricular fibrillation. (presentation of 1st case in italian pediatric literature)]. Clin Pediatr (Bologna). 1963;45:656\u2013683.","journal-title":"Clin Pediatr (Bologna)"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(92)90253-j"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000085071.28695.C4"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1159\/000047299"},{"key":"e_1_3_3_7_2","first-page":"28","article-title":"Catecholaminergic-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases.","volume":"40","author":"Coumel P","year":"1978","unstructured":"Coumel, P, Fidelle, J, Lucet, V, Attuel, P, Bouvrain, Y. Catecholaminergic-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases. Br Heart J. 1978;40(supplement):28\u201337.","journal-title":"Br Heart J"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/12618"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8167.2000.tb00743.x"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1807668"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2021.03.313"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehab275"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/pace.13408"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacep.2020.03.010"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2014.12.023"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp252"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCEP.107.737882"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.520999"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000144458.58660.BB"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1329"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2014.05.045"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8159.2009.02179.x"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8167.2008.01282.x"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCEP.113.000444"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.gene.2015.08.062"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.02.009"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1249\/JSR.0b013e3182874abb"}],"container-title":["Circulation: Arrhythmia and Electrophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCEP.121.010688","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,12]],"date-time":"2024-05-12T21:38:53Z","timestamp":1715549933000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCEP.121.010688"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4]]},"references-count":26,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,4]]}},"alternative-id":["10.1161\/CIRCEP.121.010688"],"URL":"https:\/\/doi.org\/10.1161\/circep.121.010688","relation":{},"ISSN":["1941-3149","1941-3084"],"issn-type":[{"value":"1941-3149","type":"print"},{"value":"1941-3084","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4]]},"assertion":[{"value":"2021-11-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-31","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}