Background: Conduction and ventricular arrhythmic disturbances in myotonic dystrophy (MD) are the main causes of cardiac morbidity and mortality. Methods: To better define the natural history of cardiac involvement and the relationship with severity grade of neuromuscular disease, we followed up 83 MD patients (56 men and 27 women; mean age 37±14 years) for a mean period of 61±38 months (range 2-156 months). Pre-study evaluation included physical examination, 12-lead electrocardiogram, 24-h Holter monitoring, and echocardiography. Results: During the study, cardiac critical events (CCE) occurred in 20 patients (24%), five of whom died of sudden death related to documented ventricular tachycardia, 12 of whom had pacemaker implantation and three of whom experienced ventricular tachyarrhythmias. The incidence of CCE appeared to be related to disease severity; in fact, CCE progressively increased from class I to class III (2% vs. 17% vs. 78%, respectively, P<0.001). Moreover, 8% of the patients experienced CCE in spite of low-grade muscular involvement. Conclusions: Critical cardiac events are significantly associated with the more severe forms of neuromuscular disease, while in some patients cardiac involvement in MD worsens independently from skeletal muscle disease. Copyright (C) 1999 Elsevier Science B.V.

Cardiac critical events in myotonic dystrophy: Relationship with the severity of neuromuscular disease / Mammarella, Antonio; Michele, Paradiso; Vincenzo, Paoletti; Basili, Stefania; Antonini, Giovanni; Antonio De Matteis, ; Cardarello, Cristiano M.; Lucia, Donnarumma; Labbadia, Giancarlo; Antonino, Musca. - In: EUROPEAN JOURNAL OF INTERNAL MEDICINE. - ISSN 0953-6205. - 10:4(1999), pp. 198-201. [10.1016/s0953-6205(99)00050-3]

Cardiac critical events in myotonic dystrophy: Relationship with the severity of neuromuscular disease

MAMMARELLA, Antonio;BASILI, Stefania;Giovanni Antonini;LABBADIA, Giancarlo;
1999

Abstract

Background: Conduction and ventricular arrhythmic disturbances in myotonic dystrophy (MD) are the main causes of cardiac morbidity and mortality. Methods: To better define the natural history of cardiac involvement and the relationship with severity grade of neuromuscular disease, we followed up 83 MD patients (56 men and 27 women; mean age 37±14 years) for a mean period of 61±38 months (range 2-156 months). Pre-study evaluation included physical examination, 12-lead electrocardiogram, 24-h Holter monitoring, and echocardiography. Results: During the study, cardiac critical events (CCE) occurred in 20 patients (24%), five of whom died of sudden death related to documented ventricular tachycardia, 12 of whom had pacemaker implantation and three of whom experienced ventricular tachyarrhythmias. The incidence of CCE appeared to be related to disease severity; in fact, CCE progressively increased from class I to class III (2% vs. 17% vs. 78%, respectively, P<0.001). Moreover, 8% of the patients experienced CCE in spite of low-grade muscular involvement. Conclusions: Critical cardiac events are significantly associated with the more severe forms of neuromuscular disease, while in some patients cardiac involvement in MD worsens independently from skeletal muscle disease. Copyright (C) 1999 Elsevier Science B.V.
1999
bradyarrhythmias; myotonic dystrophy (steinert's disease); pacemaker implantation; sudden death; ventricular tachyarrhythmias
01 Pubblicazione su rivista::01a Articolo in rivista
Cardiac critical events in myotonic dystrophy: Relationship with the severity of neuromuscular disease / Mammarella, Antonio; Michele, Paradiso; Vincenzo, Paoletti; Basili, Stefania; Antonini, Giovanni; Antonio De Matteis, ; Cardarello, Cristiano M.; Lucia, Donnarumma; Labbadia, Giancarlo; Antonino, Musca. - In: EUROPEAN JOURNAL OF INTERNAL MEDICINE. - ISSN 0953-6205. - 10:4(1999), pp. 198-201. [10.1016/s0953-6205(99)00050-3]
File allegati a questo prodotto
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/124382
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 0
  • ???jsp.display-item.citation.isi??? ND
social impact