{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T12:21:22Z","timestamp":1763036482545},"reference-count":36,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2010,4]]},"abstract":"<jats:p>\n            <jats:bold>\n              <jats:italic>Background\u2014<\/jats:italic>\n            <\/jats:bold>\n            Arrhythmogenic Right Ventricular Dysplasia\/Cardiomyopathy (ARVD\/C) Diagnostic Task Force Criteria (TFC) proposed in 1994 are highly specific but lack sensitivity. A new international task force modified criteria to improve diagnostic yield. A comparison of diagnosis by 1994 TFC versus newly proposed criteria in 3 patient groups was conducted.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>Methods and Results\u2014<\/jats:italic>\n            <\/jats:bold>\n            In new TFC, scoring by major and minor criteria is maintained. Structural abnormalities are quantified and TFC highly specific for ARVD\/C upgraded to major. Furthermore, new criteria are added: terminal activation duration of QRS \u226555 ms, ventricular tachycardia with left bundle-branch block morphology and superior axis, and genetic criteria. Three groups were studied: (1) 105 patients with proven ARVD\/C according to 1994 TFC, (2) 89 of their family members, and (3) 39 patients with probable ARVD\/C (ie, 3 points by 1994 TFC). All were screened for pathogenic mutations in desmosomal genes. Three ARVD\/C patients did not meet the new sharpened criteria on structural abnormalities and thereby did not fulfill new TFC. In 62 of 105 patients with proven ARVD\/C, mutations were found: 58 in the gene encoding Plakophilin2 (\n            <jats:italic>PKP2<\/jats:italic>\n            ), 3 in Desmoglein2, 3 in Desmocollin2, and 1 in Desmoplakin. Three patients had bigenic involvement. Ten additional relatives (11%) fulfilled new TFC: 9 (90%) were female, and all carried\n            <jats:italic>PKP2<\/jats:italic>\n            mutations. No relatives lost diagnosis by application of new TFC. Of patients with probable ARVD\/C, 25 (64%) fulfilled new TFC: 8 (40%) women and 14 (56%) carrying pathogenic mutations.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>Conclusions\u2014<\/jats:italic>\n            <\/jats:bold>\n            In this first study applying new TFC to patients suspected of ARVD\/C, 64% of probable ARVD\/C patients and 11% of family members were additionally diagnosed. ECG criteria and pathogenic mutations especially contributed to new diagnosis. Newly proposed TFC have a major impact in increasing diagnostic yield of ARVD\/C.\n          <\/jats:p>","DOI":"10.1161\/circep.109.927202","type":"journal-article","created":{"date-parts":[[2010,3,10]],"date-time":"2010-03-10T03:24:58Z","timestamp":1268191498000},"page":"126-133","update-policy":"http:\/\/dx.doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":59,"title":["Arrhythmogenic Right Ventricular Dysplasia\/Cardiomyopathy Diagnostic Task Force Criteria"],"prefix":"10.1161","volume":"3","author":[{"given":"Moniek G.P.J.","family":"Cox","sequence":"first","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jasper J.","family":"van der Smagt","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maartje","family":"Noorman","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ans C.","family":"Wiesfeld","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul G.A.","family":"Volders","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irene M.","family":"van Langen","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douwe E.","family":"Atsma","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dennis","family":"Dooijes","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arjan C.","family":"Houweling","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Loh","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luc","family":"Jordaens","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yvonne","family":"Arens","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maarten J.","family":"Cramer","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pieter A.","family":"Doevendans","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. Peter","family":"van Tintelen","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur A.M.","family":"Wilde","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard N.W.","family":"Hauer","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology, Medical Genetics, and Medical Physiology (M.G.P.J.C., J.J.v.d.S., M.N., D.D., P.L., M.J.C., P.A.D., R.N.W.H.), University Medical Center Utrecht; the Departments of Cardiology and Medical Genetics (A.C.W., I.M.v.L., P.v.T.), University Medical Center Groningen, University of Groningen; Maastricht University Medical Center (P.G.A.V., Y.A.); Academic Medical Center Amsterdam (A.A.M.W.); University Medical Center Leiden (D.E.A.); VU University Medical Center..."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.65.2.7053899"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(97)00332-X"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.94.5.983"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.71.3.215"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02379-5"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1086\/344208"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1461"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.105.568642"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.105.609719"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.105.561654"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.105.583674"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1086\/509122"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)02307-0"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1054-8807(03)00107-8"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2004.01.001"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehl095"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/circresaha.107.154252"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1341.030"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI27751"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.59.2.758993"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.88.3.8353918"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.75.6.1014"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(96)00216-7"},{"key":"e_1_3_2_24_2","unstructured":"MarcusFI McKennaWJ SherillD BassoC BauceB BluemkeDA CalkinsH CorradoDA CoxMGPJ DaubertJD FontaineG GearK HauerRNW NavaA PicardMH ProtonotariosN SaffitzJE Yoerger SanbornDM SteinbergJS TandriHM ThieneG TowbinJA TsatsopoulouA WichterT ZarebaW.Diagnosis of arrhythmogenic right ventricular cardiomyopathy\/dysplasie (ARVC\/D):proposed modifications of the Task Force Criteria.Circulation.2010;121:In press."},{"key":"e_1_3_2_25_2","first-page":"399","volume":"42","year":"1993","unstructured":"Fontaine G, Umemura J, Di Donna P, Tsezana R, Cannat JJ, Frank R. 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