A 34-year longitudinal study on long-term cardiac outcomes in DM1 patients with normal ECG at baseline at an Italian clinical centre
| Autor: | Marco Testa, Laura Fionda, Giovanni Antonini, Fiammetta Vanoli, Stefania Morino, Elisabetta Bucci, Nicola Vanacore, Nadia Attalla El Halabieh, Giulia Pignatelli, Loretta Licchelli, Tiziana De Santis, Annalisa Botta, Matteo Garibaldi, Erica Gabriele, Antonella Di Pasquale, Alessandra Frattari |
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| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities Longitudinal study medicine.medical_specialty Adolescent 030204 cardiovascular system & hematology Severity of Illness Index Sudden cardiac death Cohort Studies 03 medical and health sciences Electrocardiography Young Adult 0302 clinical medicine CTG expansion Cardiac Conduction System Disease Internal medicine Cardiac conduction Prevalence Medicine Humans Myotonic Dystrophy cardiovascular diseases myotonic dystrophy type 1 ctg expansion cardiac conduction and/ or rhythm abnormalities sudden cardiac death prevalence incidence risk factors Neuroradiology Proportional Hazards Models Cardiac conduction and/or rhythm abnormalities Incidence Myotonic dystrophy type 1 Risk factors business.industry Incidence (epidemiology) Arrhythmias Cardiac Middle Aged medicine.disease Neurology Settore MED/03 - Genetica Medica Italy Cohort Cardiology Settore MED/26 - Neurologia Female Neurology (clinical) Age of onset business 030217 neurology & neurosurgery Atrial flutter |
| Popis: | Cardiac conduction and/or rhythm abnormalities (CCRA) are the most frequent and life-threatening complications in DM1. In order to determine prevalence, incidence, characteristics, age of onset and predictors of CCRA, CCRA progression and sudden cardiac death (SCD) in DM1, we collected ECG/24hECG-Holter data from a yearly updated 34-year database of a cohort of 103 DM1 patients without cardiac abnormalities at baseline, followed for at least 1 year. Fifty-five patients developed CCRA [39 developed conduction abnormalities (CCA) and 16 rhythm abnormalities (CRA)], which progressed in 22. Nine had SCD. Risk and incidence of CCRA amounted to 53.4 and 6.83% person-years (CCA: 37.9 and 4.8%; CRA 15.5 and 2%), respectively; risk and incidence of SCD amounted to 8.74 and 0.67% person-years, respectively. CTG expansion represented a predictor of CCRA incidence (HR 1.10, p = 0.04), CCRA progression (HR 1.28, p = 0.001) and SCD (HR 1.39, p = 0.002). MIRS progression during follow-up was associated with CCRA prevalence (OR 5.82, p = 0.004); older age and larger CTG expansion to SCD prevalence (OR 2.67, p = 0.012; OR 1.54, p = 0.005). Age of CCRA onset and CCRA progression was significantly lower in patients with larger CTG expansion and in those with MIRS progression. Age when SCD occurred was significantly lower in patients with larger CTG expansion. Amongst recorded cardiac abnormalities, both atrial flutter (OR 8.70; p = 0.031) and paroxysmal supraventricular tachycardia (OR 8.67; p = 0.040) were associated with SCD. Although all DM1patients may develop cardiac abnormalities at any time in their life, patients older than 30 years with larger CTG expansion and MIRS progression in particular should be carefully monitored via periodical ECG. |
| Databáze: | OpenAIRE |
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