{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T03:37:51Z","timestamp":1747193871754,"version":"3.40.5"},"reference-count":47,"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":[[2023,2]]},"abstract":"<jats:sec>\n            <jats:title>Background:<\/jats:title>\n            <jats:p>Arrhythmogenic right ventricular cardiomyopathy (ARVC), a rare inherited disease, causes ventricular tachycardia, sudden cardiac death, and heart failure (HF). We investigated ARVC clinical features, genetic findings, natural history, and the occurrence of life-threatening arrhythmic events (LTAEs), HF death, or heart transplantation (HF-death\/HTx) to identify risk factors.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods:<\/jats:title>\n            <jats:p>The clinical course of 111 consecutive patients with definite ARVC, predictors of LTAE, HF-death\/HTx, and combined events were analyzed in the entire cohort and in a subgroup of 40 patients without sustained ventricular arrhythmia before diagnosis.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results:<\/jats:title>\n            <jats:p>\n              The 5-year cumulative probability of LTAE was 30% and HF-death\/HTx was 10%. Predictors of HF-death\/HTx were reduced right ventricle ejection fraction (HR: 0.93;\n              <jats:italic>P<\/jats:italic>\n              =0.010), HF symptoms (HR: 4.37;\n              <jats:italic>P<\/jats:italic>\n              =0.010), epsilon wave (HR: 4.99;\n              <jats:italic>P<\/jats:italic>\n              =0.015), and number of leads with low QRS voltage (HR: 1.28;\n              <jats:italic>P<\/jats:italic>\n              =0.001). Each additional lead with low QRS voltage increased the risk of HF-death\/HTx by 28%. Predictors of LTAE were prior syncope (HR: 1.81;\n              <jats:italic>P<\/jats:italic>\n              =0.040), number of leads with T wave inversion (HR: 1.17;\n              <jats:italic>P<\/jats:italic>\n              =0.039), low QRS voltage (HR: 1.12;\n              <jats:italic>P<\/jats:italic>\n              =0.021), younger age (HR: 0.97;\n              <jats:italic>P<\/jats:italic>\n              =0.006), and prior ventricular arrhythmia\/ventricular fibrillation (HR: 2.45;\n              <jats:italic>P<\/jats:italic>\n              =0.012). Each additional lead with low QRS voltage increased the risk of LTAE by 17%. In patients without ventricular arrhythmia before clinical diagnosis of ARVC, the number of leads with low QRS voltage (HR: 1.68;\n              <jats:italic>P<\/jats:italic>\n              =0.023) was independently associated with HF-death\/HTx.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions:<\/jats:title>\n            <jats:p>Our study demonstrated the characteristics of a specific cohort with a high prevalence of arrhythmic burden at presentation, male predominance, younger age and HF severe outcomes. Our main results suggest that the presence and extension of low QRS voltage can be a risk predictor for HF-death\/HTx in ARVC patients, regardless of the arrhythmic risk. This study can contribute to the global ARVC risk stratification, adding new insights to the international current scientific knowledge.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circep.122.011391","type":"journal-article","created":{"date-parts":[[2023,1,31]],"date-time":"2023-01-31T20:00:20Z","timestamp":1675195220000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical Features, Genetic Findings, and Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy: Data From a Brazilian Cohort"],"prefix":"10.1161","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1061-8982","authenticated-orcid":true,"given":"Nat\u00e1lia","family":"Quintella Sangiorgi Olivetti","sequence":"first","affiliation":[{"name":"Arrhythmia Unit (N.Q.S.O., L.S., G.D.P., D.T.H., P.V., C.H., C.P., T.C.W., S.C.B., V.B.L., M.S., F.d.C.D.)."},{"name":"Laboratory of Genetics and Molecular Cardiology (LGMC) (N.Q.S.O., F.W., M.L.P.d.C., J.E.K., A.d.C.P.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3679-5349","authenticated-orcid":true,"given":"Luciana","family":"Sacilotto","sequence":"additional","affiliation":[{"name":"Arrhythmia Unit (N.Q.S.O., L.S., G.D.P., D.T.H., P.V., C.H., C.P., T.C.W., S.C.B., V.B.L., M.S., F.d.C.D.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0007-4862","authenticated-orcid":true,"given":"Fanny","family":"Wulkan","sequence":"additional","affiliation":[{"name":"Laboratory of Genetics and Molecular Cardiology (LGMC) (N.Q.S.O., F.W., M.L.P.d.C., J.E.K., A.d.C.P.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9831-3392","authenticated-orcid":true,"given":"Gabrielle","family":"D\u2019Arezzo Pessente","sequence":"additional","affiliation":[{"name":"Arrhythmia Unit (N.Q.S.O., L.S., G.D.P., D.T.H., P.V., C.H., C.P., T.C.W., S.C.B., V.B.L., M.S., F.d.C.D.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4769-8031","authenticated-orcid":true,"given":"Mariana","family":"Lombardi Peres de Carvalho","sequence":"additional","affiliation":[{"name":"Laboratory of Genetics and Molecular Cardiology (LGMC) (N.Q.S.O., F.W., M.L.P.d.C., J.E.K., A.d.C.P.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1028-9568","authenticated-orcid":true,"given":"Danilo","family":"Moleta","sequence":"additional","affiliation":[{"name":"Echocardiogram Imaging Unit (D.B.M., M.L.C.V.)."},{"name":"Echocardiogram Imaging Unit, Hospital Israelita Albert Einstein. 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