Natural history of cardiac involvement in myotonic dystrophy (Steinert’s Disease): A 13-year follow-up study
| Autor: | Antonio De Matteis, Antonio Mammarella, V. Paoletti, Giancarlo Labbadia, M. Paradiso, Stefania Basili, Giovanni Antonini, Manuela Di Franco, Antonino Musca, L. Donnarumma |
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| Rok vydání: | 2000 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Heart Diseases Heart disease Ventricular tachycardia Myotonic dystrophy Sudden death Bifascicular block Electrocardiography Internal medicine Humans Myotonic Dystrophy Medicine Pharmacology (medical) Child Aged business.industry Arrhythmias Cardiac General Medicine Middle Aged medicine.disease Myotonia Surgery Death Sudden Cardiac Echocardiography Heart failure cardiovascular system Cardiology Female business Complication Follow-Up Studies |
| Zdroj: | Advances in Therapy. 17:238-251 |
| ISSN: | 1865-8652 0741-238X |
| DOI: | 10.1007/bf02853163 |
| Popis: | Myotonic dystrophy (MD) is associated with a wide spectrum of cardiac abnormalities, but only a few longitudinal studies have investigated the natural course of heart disease in MD. To assess whether neuromuscular involvement significantly predicts cardiac disorders in MD, 83 patients with various grades of disease severity were enrolled in a 13-year follow-up study (mean, 60.6 +/- 37.8 months) that included periodic physical and instrumental cardiac examinations (standard and Holter electrocardiography, echocardiography). During follow-up, muscular disease worsened clinically in 9 patients (11%) whose baseline severity grade changed accordingly; only 3 of them demonstrated parallel worsening of cardiac disturbance, however, compared with a large number of patients who showed additional cardiac abnormalities. These included further worsening of pre-existing pathologic features (19/83) and the appearance de novo of serious arrhythmias and/or conduction defects (23/83). Pacemaker implantation was necessary in 11 of 83 patients (13.2%) who had symptomatic bradyarrhythmias, bifascicular block, and P-R prolongation with a His-to-ventricle interval exceeding 55 ms, as documented by electrophysiologic study. Eight (9.6%) patients died: 2 from noncardiac and 1 from unknown causes, 1 from heart failure, and 4 from sudden death closely related to documented ventricular tachycardia. The incidence and seriousness of arrhythmic and conduction disturbances correlated with the severity of the muscular involvement. Nevertheless, cardiac and muscular disease did not show a linear progression. Cardiac involvement generally worsened more rapidly than did skeletal muscle disease. |
| Databáze: | OpenAIRE |
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