Journal article
Long-Term Arrhythmic and Nonarrhythmic Outcomes of Lamin A/C Mutation Carriers
S Kumar, SH Baldinger, E Gandjbakhch, P Maury, JM Sellal, AFA Androulakis, X Waintraub, P Charron, A Rollin, P Richard, WG Stevenson, CJ Macintyre, CY Ho, T Thompson, JK Vohra, JM Kalman, K Zeppenfeld, F Sacher, UB Tedrow, NK Lakdawala
Journal of the American College of Cardiology | ELSEVIER SCIENCE INC | Published : 2016
Abstract
Background Mutations in LMNA are variably expressed and may cause cardiomyopathy, atrioventricular block (AVB), or atrial arrhythmias (AAs) and ventricular arrhythmias (VA). Detailed natural history studies of LMNA-associated arrhythmic and nonarrhythmic outcomes are limited, and the prognostic significance of the index cardiac phenotype remains uncertain. Objectives This study sought to describe the arrhythmic and nonarrhythmic outcomes of LMNA mutation carriers and to assess the prognostic significance of the index cardiac phenotype. Methods The incidence of AVB, AA, sustained VA, left ventricular systolic dysfunction (LVD) (= left ventricular ejection fraction ≤50%), and end-stage heart f..
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Funding Acknowledgements
From the <SUP>a</SUP>Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts; <SUP>b</SUP>Department of Cardiology, Inselspital, Bern University Hospital, Bern, Switzerland; <SUP>c</SUP>Hopital Pitie-Salpetriere, Assistance Publique Hopitaux De Paris (AP-HP), Department of Cardiology, Paris, France; <SUP>d</SUP>Toulouse University Hospital, Rangueil, Toulouse, France; <SUP>e</SUP>Hopital Cardiologique du Haut-Leveque (CHU), Bordeaux-Pessac, France; <SUP>f</SUP>L'Institut de Rythmologie et Modelisation Cardiaque (LIRYC), Bordeaux, France; <SUP>g</SUP>Institut Hospitalo-Universitaire (IHU), Bordeaux, France; <SUP>h</SUP>Centre Hospitalier Universitaire de Nancy, Nancy, France; <SUP>i</SUP>Department of Cardiology, Leiden University Medical Centre, Leiden, the Netherlands; <SUP>j</SUP>Centre de Reference Maladies Cardiaques Hereditaires, Institute for Cardiometabolism and Nutrition (ICAN), Paris, France; <SUP>k</SUP>Universite de Versailles-Saint Quentin, Hopital Ambroise Pare, AP-HP, Boulogne-Billancourt, France; <SUP>l</SUP>Cardiomyogenetics, Department of Biochemistry and INSERM U582, University Hospital Pitie-Salpetriere, AP-HP, Paris, France; <SUP>m</SUP>Department of Genetic Medicine, The Royal Melbourne Hospital and University of Melbourne, Melbourne, Victoria, Australia; and the <SUP>n</SUP>Department of Cardiology, Division of Medicine, The Royal Melbourne Hospital and University of Melbourne, Melbourne, Victoria, Australia. Dr. Kumar is a recipient of the Neil Hamilton Fairley Overseas Research scholarship co-funded by the National Health and Medical Research Council and the National Heart Foundation of Australia; and the Bushell Travelling Fellowship funded by the Royal Australasian College of Physicians. Dr. Lakdawala has received support from the O'Hare Family Foundation directed toward research on cardiolaminopathy. Dr. Gandjbakhch has received consultant fees from Sorin, Medtronic, Boston Scientific, and Bayer. Dr. Stevenson has intellectual property and a patent for needle ablation consigned to Brigham and Women's Hospital. Dr. Sacher has received speaker honoraria from St. Jude Medical. Dr. Tedrow is on the faculty of St. Jude Medical and Boston Scientific. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.