{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T01:32:18Z","timestamp":1793410338587,"version":"4.5.0"},"reference-count":28,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2019,7,23]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>\n                      An accurate estimation of the risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with\n                      <jats:italic>LMNA<\/jats:italic>\n                      mutations is crucial to select candidates for implantable cardioverter-defibrillator implantation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We included 839 adult patients with LMNA mutations, including 660 from a French nationwide registry in the development sample, and 179 from other countries, referred to 5 tertiary centers for cardiomyopathies, in the validation sample. LTVTA was defined as (1) sudden cardiac death or (2) implantable cardioverter defibrillator\u2013treated or hemodynamically unstable VTA. The prognostic model was derived using the Fine-Gray regression model. The net reclassification was compared with current clinical practice guidelines. The results are presented as means (SD) or medians [interquartile range].<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>We included 444 patients, 40.6 (14.1) years of age, in the derivation sample and 145 patients, 38.2 (15.0) years, in the validation sample, of whom 86 (19.3%) and 34 (23.4%) experienced LTVTA over 3.6 [1.0\u20137.2] and 5.1 [2.0\u20139.3] years of follow-up, respectively. Predictors of LTVTA in the derivation sample were: male sex, nonmissense LMNA mutation, first degree and higher atrioventricular block, nonsustained ventricular tachycardia, and left ventricular ejection fraction (https:\/\/lmna-risk-vta.fr). In the derivation sample, C-index (95% CI) of the model was 0.776 (0.711\u20130.842), and the calibration slope 0.827. In the external validation sample, the C-index was 0.800 (0.642\u20130.959), and the calibration slope was 1.082 (95% CI, 0.643\u20131.522). A 5-year estimated risk threshold \u22657% predicted 96.2% of LTVTA and net reclassified 28.8% of patients with LTVTA in comparison with the guidelines-based approach.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>In comparison with the current standard of care, this risk prediction model for LTVTA in laminopathies significantly facilitated the choice of candidates for implantable cardioverter defibrillators.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Clinical Trial Registration:<\/jats:title>\n                    <jats:p>\n                      URL:\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/www.clinicaltrials.gov\">https:\/\/www.clinicaltrials.gov<\/jats:ext-link>\n                      . Unique identifier: NCT03058185.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.118.039410","type":"journal-article","created":{"date-parts":[[2019,6,3]],"date-time":"2019-06-03T05:02:48Z","timestamp":1559538168000},"page":"293-302","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":270,"title":["Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies"],"prefix":"10.1161","volume":"140","author":[{"given":"Karim","family":"Wahbi","sequence":"first","affiliation":[{"name":"APHP, Cochin Hospital, Cardiology Department, FILNEMUS, Centre de R\u00e9f\u00e9rence de Pathologie Neuromusculaire Nord\/Est\/Ile de France, Paris-Descartes, Sorbonne Paris Cit\u00e9 University (K.W., D.D.)."},{"name":"INSERM Unit 970, Paris Cardiovascular Research Centre (PARCC), France (K.W., C. Stalens, E.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabah","family":"Ben Yaou","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"},{"name":"Sorbonne Universit\u00e9s, INSERM UMRS 974, CNRS, UMR-7215, Center for Research in Myology, Myology Institute,(R.B.Y., G.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Gandjbakhch","sequence":"additional","affiliation":[{"name":"APHP, Institute of Cardiology (E.G., X.W., P.C.)"},{"name":"Sorbonne Universit\u00e9s, UPMC Univ Paris 06, INSERM 1166, Institute of Cardiometabolism and Nutrition (ICAN), France (E.G., P.C.)."},{"name":"Centre de R\u00e9f\u00e9rence des Maladies Cardiaques H\u00e9r\u00e9ditaires, Paris, France\u2020 (E.G., P.C.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Anselme","sequence":"additional","affiliation":[{"name":"Cardiology Department, University Hospital of Rouen, France (F.A.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Gossios","sequence":"additional","affiliation":[{"name":"Inherited Cardiovascular Diseases Unit, University College London &amp; St. Bartholomew\u2019s Hospital, United Kingdom\u2020 (T.G., K.S., P.E.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neal K.","family":"Lakdawala","sequence":"additional","affiliation":[{"name":"Cardiovascular Division, Department of Medicine, Brigham and Women\u2019s Hospital, Boston, MA (N.K.L., U.B.T.)."},{"name":"Aix Marseille University, INSERM, GMGF; Department of Medical Genetics, Childrens\u2019 Hospital La Timone, France (A.D.S.-G., N.L.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Stalens","sequence":"additional","affiliation":[{"name":"INSERM Unit 970, Paris Cardiovascular Research Centre (PARCC), France (K.W., C. Stalens, E.J.)."},{"name":"Medical Affairs Department, AFM-Telethon, Evry, France (C. Stalens)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Sacher","sequence":"additional","affiliation":[{"name":"Centre de reference des maladies rythmiques h\u00e9r\u00e9ditaires, Bordeaux University Hospital (CHU), IHU Liryc, Electrophysiology and Heart Modeling Institute, fondation Bordeaux Universit\u00e9, Univ. Bordeaux, INSERM U1045, France (F.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Babuty","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Fran\u00e7ois Rabelais, Cardiology Department, CHU Tours, France (D. Babuty)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Noel","family":"Trochu","sequence":"additional","affiliation":[{"name":"INSERM, UMR1087, Universit\u00e9 de Nantes, L\u2019Institut du Thorax, CHU de Nantes, CIC, Centre de r\u00e9f\u00e9rence pour la prise en charge des maladies rythmiques h\u00e9r\u00e9ditaires de Nantes, France\u2020 (J.-N.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghassan","family":"Moubarak","sequence":"additional","affiliation":[{"name":"Department of Electrophysiology and Pacing, InParys Clinical Research Group, Clinique Ambroise Par\u00e9, Neuilly-sur-Seine, France (G.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kostantinos","family":"Savvatis","sequence":"additional","affiliation":[{"name":"Inherited Cardiovascular Diseases Unit, University College London &amp; St. Bartholomew\u2019s Hospital, United Kingdom\u2020 (T.G., K.S., P.E.)."},{"name":"William Harvey Research Institute, Queen Mary University London, United Kingdom (K.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rapha\u00ebl","family":"Porcher","sequence":"additional","affiliation":[{"name":"APHP, H\u00f4tel-Dieu Hospital, Centre d\u2019Epid\u00e9miologie Clinique, INSERM U1153, Universit\u00e9 Paris Descartes - Sorbonne Paris Cit\u00e9, France (R.P.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Lafor\u00eat","sequence":"additional","affiliation":[{"name":"APHP, H\u00f4pital Raymond Poincar\u00e9, Centre de R\u00e9f\u00e9rence des maladies neuromusculaires Nord-Est-\u00cele de France, Garches (P.L., A.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdallah","family":"Fayssoil","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"},{"name":"APHP, H\u00f4pital Raymond Poincar\u00e9, Centre de R\u00e9f\u00e9rence des maladies neuromusculaires Nord-Est-\u00cele de France, Garches (P.L., A.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eloi","family":"Marijon","sequence":"additional","affiliation":[{"name":"H\u00f4pital Europ\u00e9en Georges Pompidou, D\u00e9partement de Cardiologie, Unit\u00e9 de Rythmologie, Paris, France (E.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanya","family":"Stojkovic","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"B\u00e9hin","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Leonard-Louis","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilhem","family":"Sole","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des maladies neuromusculaires AOC, H\u00f4pital Pellegrin, CHU Bordeaux, France (G.S., X.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabien","family":"Labombarda","sequence":"additional","affiliation":[{"name":"Cardiology Department, University Hospital of Caen, France (F.L.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascale","family":"Richard","sequence":"additional","affiliation":[{"name":"APHP, UF Cardiog\u00e9n\u00e9tique et Myog\u00e9n\u00e9tique, Centre de G\u00e9n\u00e9tique, GH Piti\u00e9 Salp\u00eatri\u00e8re, Paris, France (P.R., C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Metay","sequence":"additional","affiliation":[{"name":"APHP, UF Cardiog\u00e9n\u00e9tique et Myog\u00e9n\u00e9tique, Centre de G\u00e9n\u00e9tique, GH Piti\u00e9 Salp\u00eatri\u00e8re, Paris, France (P.R., C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Quijano-Roy","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Service de Neurologie, R\u00e9animation et R\u00e9education Pediatriques, H\u00f4pital Raymond Poincar\u00e9, Garches, France; UMR 1179 INSERM, Universit\u00e9 Versailles Saint-Quentin\u2013en-Yvelines, Montigny-le-Bretonneux (S.Q.-R., I. Dabaj)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivana","family":"Dabaj","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Service de Neurologie, R\u00e9animation et R\u00e9education Pediatriques, H\u00f4pital Raymond Poincar\u00e9, Garches, France; UMR 1179 INSERM, Universit\u00e9 Versailles Saint-Quentin\u2013en-Yvelines, Montigny-le-Bretonneux (S.Q.-R., I. Dabaj)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Didier","family":"Klug","sequence":"additional","affiliation":[{"name":"Cardiologie A, University Hospital, Lille, France (D.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Christine","family":"Vantyghem","sequence":"additional","affiliation":[{"name":"CHU Lille, Endocrinology, Diabetology and Metabolism, Univ Lille, Inserm, UMR 1190 -Translational research in diabetes; EGID European Genomic Institute for Diabetes, France (M.-C.V.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Chevalier","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9s, UPMC Univ Paris 06, INSERM 1166, Institute of Cardiometabolism and Nutrition (ICAN), France (E.G., P.C.)."},{"name":"Centre de R\u00e9f\u00e9rence des Maladies Cardiaques H\u00e9r\u00e9ditaires, Paris, France\u2020 (E.G., P.C.)."},{"name":"Service de Cardiologie, H\u00f4pital Est, Lyon, France\u2020 (P.C.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Ambrosi","sequence":"additional","affiliation":[{"name":"Department of Cardiology, La Timone Hospital, Aix-Marseille Universit\u00e9, France (P.A.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Salort","sequence":"additional","affiliation":[{"name":"APHM, Centre de r\u00e9f\u00e9rence des maladies neuromusculaires PACA-R\u00e9union-Rh\u00f4ne Alpes, H\u00f4pital Timone; Aix Marseille Universit\u00e9, Inserm UMR_S 910, GMGF, France (E.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Sadoul","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Institut Lorrain du Coeur et des Vaisseaux, CHU Nancy-Brabois, Vandoeuvre les Nancy Cedex, France (N.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Waintraub","sequence":"additional","affiliation":[{"name":"APHP, Institute of Cardiology (E.G., X.W., P.C.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khadija","family":"Chikhaoui","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence de pathologie neuromusculaire Paris-Est, FILNEMUS, Myology Institute, Neurology Department (R.B.Y., A.F., T.S., A.B., S.L.-L., K.C., H.-M.B., B.E.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Mabo","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Inserm, LTSI - UMR 1099, France (P. Mabo, R.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Combes","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Rythmologie, Clinique Pasteur, Toulouse, France (N.C.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Maury","sequence":"additional","affiliation":[{"name":"University Hospital Rangueil, Cardiology department; Unit\u00e9 Inserm U1048, Toulouse, France (P. Maury)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Marc","family":"Sellal","sequence":"additional","affiliation":[{"name":"D\u00e9partement de Cardiologie, Centre Hospitalier Universitaire de Nancy; INSERM-IADI U1254, Vand\u0153uvre l\u00e8s-Nancy, France (J.-M.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Usha B.","family":"Tedrow","sequence":"additional","affiliation":[{"name":"Cardiovascular Division, Department of Medicine, Brigham and Women\u2019s Hospital, Boston, MA (N.K.L., U.B.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan M.","family":"Kalman","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Division of Medicine (J.M.K., J.V.), The Royal Melbourne Hospital and University of Melbourne, Victoria, Australia."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jitendra","family":"Vohra","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Division of Medicine (J.M.K., J.V.), The Royal Melbourne Hospital and University of Melbourne, Victoria, Australia."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander F.A.","family":"Androulakis","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Leiden University Medical Centre, the Netherlands (A.G.A.A., K.Z.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Zeppenfeld","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Leiden University Medical Centre, the Netherlands (A.G.A.A., K.Z.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tina","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine (T.T.), The Royal Melbourne Hospital and University of Melbourne, Victoria, Australia."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Barnerias","sequence":"additional","affiliation":[{"name":"AP-HP, Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, service de neurologie p\u00e9diatrique, H\u00f4pital Necker, GH Necker-Enfants malades, Paris, France (C.B., I. 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