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Войти в исследования Laminopathies: natural history and risk prediction of heart failure.
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Charron Philippe, Proukhnitzky Julie, Ben Yaou Rabah, Richard Pascale, Dembélé Mohamed, Urtis Mario, Gossios Thomas, Kumar Saurabh, Savvatis Konstantinos, Stojkovic Tanya, Anselme Frédéric, Maury Philippe, Gandjbakhch Estelle, Martins Raphaël, Sacher Frédéric, Trochu Jean-Noel, Rouanet Stéphanie, Lejeune Julie, Moubarak Ghassan, Fayssoil Abdallah, Marijon Eloi, Laforêt Pascal, Béhin Anthony, Leonard-Louis Sarah, Sole Guilhem, Labombarda Fabien, Metay Corinne, Quijano-Roy Susana, Dabaj Ivana, Klug Didier, Habib Gilbert, Vantyghem Marie-Christine, Chevalier Philippe, Salort-Campana Emmanuelle, Sellal Jean-Marc, Waintraub Xavier, Zeppenfeld Katja, Amin Ahmad S, Kramarenko Daria R, Pinto Yigal M, Landstrom Andrew, Serio Alessandra, Chikhaoui Cathy, Combes Nicolas, Barnerias Christine, Bécane Henri-Marc, Bieth Eric, Boccara Franck, Bonnet Damien, Bouhour Françoise, Brehin Anne-Claire, Cintas Pascal, Roubille François, Lamblin Nicolas, de Groote Pascal, Winum Pierre-Francois, Piriou Nicolas, Réant Patricia, De Sandre-Giovannoli Annachiara, Masingue Marion, Desguerre Isabelle, Durigneux Julien, Echaniz-Laguna Andoni, Eschalier Romain, Ferreiro Ana, Fradin Mélanie, Gaborit Bénédicte, Gay Arnaud, Hagège Albert, Isapof Arnaud, Jeru Isabelle, Lagrue Emmanuelle, Laugel Vincent, Lazarus Arnaud, Leturcq France, Magot Armelle, Manel Véronique, Mercier Sandra, Meune Christophe, Michaud Maud, Minot-Myhié Marie-Christine, Nadaj-Pakleza Aleksandra, Péréon Yann, Petit Florence, Praline Julien, Rollin Anne, Sarret Catherine, Taithe Frederic, Tard Céline, Tiffreau Vincent, Fauchier Laurent, Vatier Camille, Walther-Louvier Ulrike, Schurr Benjamin, Bobin Pierre, El Hachmi Mohamed, Billon Clarisse, Fontaine Bertrand, Vigouroux Corinne, Lakdawala Neal K, Arbustini Eloisa, Elliott Perry, Bonne Gisèle, Wahbi Karim
Аннотация
METHODS: From a population of 660 patients enrolled in the French LMNA nationwide registry, 470 adults were included in the derivation cohort. An independent international validation cohort included 245 additional patients. Baseline characteristics at genetic testing were assessed and the cumulative incidence of the primary endpoint HF-major adverse cardiac events (HF-MACE) was calculated, defined as HF hospitalization, HF-related death, mechanical circulatory support, or heart transplantation. Predictors of HF-MACE were studied after excluding patients with left ventricular ejection fraction (LVEF) <30% at baseline using a Fine-Gray competing risk model, adjusted hazard ratio (aHR) with 95% confidence interval (CI), and Harrell's concordance (C-) index. A secondary composite endpoint, without hospitalization, was also studied.
RESULTS: Among 470 patients of the derivation cohort, HF-MACE occurred in 65 over a median follow-up of 7.1 years (interquartile range: 3.4-12.1). Four independent predictors of HF-MACE were identified: male sex (aHR 1.86; 95% CI 1.060-3.290), LVEF <50% (aHR 2.18; 95% CI 1.080-4.400), missense variants in head and rod domains (aHR 2.91; 95% CI 1.110-7.630), and complete left bundle branch block (aHR 2.99; 95% CI 1.400-6.400). The C-index of the model was 0.750 (95% CI 0.720-0.780) in the derivation cohort and 0.758 (95% CI 0.720-0.800) in the validation cohort. The 5-year cumulative incidence of HF-MACE was 1.5% (95% CI 0.6-3.6), 5.0% (95% CI 1.8-8.2), and 22.0% (95% CI 15.6-28.4) among patients with 0, 1, and ≥2 risk factors, respectively. In patients with LVEF <30% at baseline, the 1-year incidence of HF-MACE was 50%, and those patients were excluded from the risk score.
CONCLUSIONS: The first prediction model for severe HF events in adult laminopathies was developed, which may facilitate early and optimal preventive management.
CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov Unique identifier: NCT03058185.