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Risk of arrhythmia in type I Myotonic Dystrophy: the role of clinical and genetic variables
  1. Paola Cudia
  1. Muscular Pathology - Neuroimmunology Unit, IRCCS Foundation, Institute of Neurology, Italy
    1. Pia Bernasconi (pbernasconi{at}istituto-besta.it)
    1. Muscular Pathology - Neuroimmunology Unit, IRCCS Foundation, Institute of Neurology, Italy
      1. Roberta Chiodelli
      1. Arrhythmology Unit, Italy
        1. Fortunato Mangiola
        1. Centre for Neuromuscular Disease, UILDM, Rome, Italy
          1. Fulvio Bellocci
          1. Institute of Cardiology, Catholic University of the sacred Heart, Rome, Italy
            1. Antonio Dello Russo
            1. Institute of Cardiology, Catholic University of the sacred Heart, Rome, Italy
              1. Corrado Angelini
              1. Department of Neuroscience, University of Padua, Italy
                1. Vincenzo Romeo
                1. Department of Neuroscience, University of Padua, Italy
                  1. Paola Melancini
                  1. Department of Cardiac, Thoracic and Vascular sciences, University of Padua, Italy
                    1. Luisa Politano
                    1. Department of Experimental Medicine, Cardiomyology and Medical Genetics, 2nd University, Naples, Italy
                      1. Alberto Palladino
                      1. Department of Experimental Medicine, Cardiomyology and Medical Genetics, 2nd University, Naples, Italy
                        1. Gerardo Nigro
                        1. Department of Cardio-Thoracic Sciences,Unit of Arrhythmology, 2nd University, Naples, Italy
                          1. Gabriele Siciliano
                          1. Department of Neurosciences, University of Pisa, Italy
                            1. Michela Falorni
                            1. Department of Neurosciences, University of Pisa, Italy
                              1. Maria Grazia Bongiorni
                              1. Institute of Cardiology, Cisanello Hospital, Pisa, Italy
                                1. Chiara Falcone
                                1. Neuroepidemiology Unit, IRCCS Foundation, Institute of Neurology, Italy
                                  1. Renato Mantegazza
                                  1. Muscular Pathology - Neuroimmunology Unit, IRCCS Foundation, Institute of Neurology, Italy
                                    1. Lucia Ovidia Morandi, Dr (lmorandi{at}istituto-besta.it)
                                    1. Muscular Pathology - Neuroimmunology Unit, IRCCS Foundation, Institute of Neurology, Italy

                                      Abstract

                                      Objective: To examine the association between the presence of arrhythmia in type 1 myotonic dystrophy (DM1) and clinical-genetic variables, evaluating their role as predictors of the arrhythmic risk.

                                      Methods: Two hundred and forty five patients with genetically proven DM1 underwent clinical and non-invasive cardiological evaluation. Severity of muscular involvement was assessed according to the five point muscular disability rating score (MDRS). Data were analysed by univariate and multivariate models.

                                      Results: We examined 245 patients and found cardiac arrhythmias in 63 subjects ,40 of which required a device implant. Statistical analyses revealed that men had a more than double risk to develop arrhythmias compared to women (p = 0.018). The addition of each year of age caused an increased risk of arrhythmia equal to 3% (p=0.030). Subjects with MDRS 5 had a risk of arrhythmia 12 times higher than patients with MDRS 1-2 (p<0.001). Although all these variables were significantly associated with cardiac rhythm dysfunction, they had a low sensitivity for the prediction of the arrhythmic risk.

                                      Conclusion: Male sex, age and muscular disability are strongly associated with development of arrhythmia in DM1. However, all these variables are weak predictors of the arrhythmic risk. These results suggest that other factors may be involved in the development of cardiac conduction abnormalities in DM1.

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