The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): Long-term outcomes

V. A. Sansone, E. Brigonzi, B. Schoser, S. Villani, M. Gaeta, G. De Ambroggi, F. Bandera, L. De Ambroggi, G. Meola

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Background: Frequency and severity of cardiac involvement in DM2 are still controversial. The aims of our study were to determine the frequency and progression of cardiac and muscle involvement in a relatively large cohort of patients with DM2 throughout Italy and Germany and to provide long-term outcomes in this disorder. Methods: 104 DM2 and 117 DM1 patients underwent baseline and follow-up assessments of, ECG, 24 h Holter monitoring, 2D echocardiography and electrophysiological study (EPS) when appropriate, and manual muscle strength testing (mean follow-up: 7.4±4.1 for DM2 and 5.7±4 years for DM1). Results: Overall, 10% of DM2 patients vs 31% of DM1 patients had PR≥200 ms and 17% of DM2 patients vs 48% of DM1 patients had QRSD≥100 ms. Six patients with DM2 vs 28 patients with DM1 required PM/ICD implantations. DM2 patients were stronger than DM1 patients at baseline, but muscle strength worsened significantly over time (p

Original languageEnglish
Pages (from-to)1147-1153
Number of pages7
JournalInternational Journal of Cardiology
Volume168
Issue number2
DOIs
Publication statusPublished - Sep 30 2013

Fingerprint

Myotonic Dystrophy
Muscle Strength
Ambulatory Electrocardiography
Italy
Germany
Echocardiography
Myocardium
Electrocardiography

Keywords

  • Cardiac arrhythmias
  • Myotonic dystrophy type 1
  • Myotonic dystrophy type 2
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2) : Long-term outcomes. / Sansone, V. A.; Brigonzi, E.; Schoser, B.; Villani, S.; Gaeta, M.; De Ambroggi, G.; Bandera, F.; De Ambroggi, L.; Meola, G.

In: International Journal of Cardiology, Vol. 168, No. 2, 30.09.2013, p. 1147-1153.

Research output: Contribution to journalArticle

Sansone, V. A. ; Brigonzi, E. ; Schoser, B. ; Villani, S. ; Gaeta, M. ; De Ambroggi, G. ; Bandera, F. ; De Ambroggi, L. ; Meola, G. / The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2) : Long-term outcomes. In: International Journal of Cardiology. 2013 ; Vol. 168, No. 2. pp. 1147-1153.
@article{faefd7e4e969466393fae59dc0f4f3ec,
title = "The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): Long-term outcomes",
abstract = "Background: Frequency and severity of cardiac involvement in DM2 are still controversial. The aims of our study were to determine the frequency and progression of cardiac and muscle involvement in a relatively large cohort of patients with DM2 throughout Italy and Germany and to provide long-term outcomes in this disorder. Methods: 104 DM2 and 117 DM1 patients underwent baseline and follow-up assessments of, ECG, 24 h Holter monitoring, 2D echocardiography and electrophysiological study (EPS) when appropriate, and manual muscle strength testing (mean follow-up: 7.4±4.1 for DM2 and 5.7±4 years for DM1). Results: Overall, 10{\%} of DM2 patients vs 31{\%} of DM1 patients had PR≥200 ms and 17{\%} of DM2 patients vs 48{\%} of DM1 patients had QRSD≥100 ms. Six patients with DM2 vs 28 patients with DM1 required PM/ICD implantations. DM2 patients were stronger than DM1 patients at baseline, but muscle strength worsened significantly over time (p",
keywords = "Cardiac arrhythmias, Myotonic dystrophy type 1, Myotonic dystrophy type 2, Sudden cardiac death",
author = "Sansone, {V. A.} and E. Brigonzi and B. Schoser and S. Villani and M. Gaeta and {De Ambroggi}, G. and F. Bandera and {De Ambroggi}, L. and G. Meola",
year = "2013",
month = "9",
day = "30",
doi = "10.1016/j.ijcard.2012.11.076",
language = "English",
volume = "168",
pages = "1147--1153",
journal = "International Journal of Cardiology",
issn = "0167-5273",
publisher = "Elsevier Ireland Ltd",
number = "2",

}

TY - JOUR

T1 - The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2)

T2 - Long-term outcomes

AU - Sansone, V. A.

AU - Brigonzi, E.

AU - Schoser, B.

AU - Villani, S.

AU - Gaeta, M.

AU - De Ambroggi, G.

AU - Bandera, F.

AU - De Ambroggi, L.

AU - Meola, G.

PY - 2013/9/30

Y1 - 2013/9/30

N2 - Background: Frequency and severity of cardiac involvement in DM2 are still controversial. The aims of our study were to determine the frequency and progression of cardiac and muscle involvement in a relatively large cohort of patients with DM2 throughout Italy and Germany and to provide long-term outcomes in this disorder. Methods: 104 DM2 and 117 DM1 patients underwent baseline and follow-up assessments of, ECG, 24 h Holter monitoring, 2D echocardiography and electrophysiological study (EPS) when appropriate, and manual muscle strength testing (mean follow-up: 7.4±4.1 for DM2 and 5.7±4 years for DM1). Results: Overall, 10% of DM2 patients vs 31% of DM1 patients had PR≥200 ms and 17% of DM2 patients vs 48% of DM1 patients had QRSD≥100 ms. Six patients with DM2 vs 28 patients with DM1 required PM/ICD implantations. DM2 patients were stronger than DM1 patients at baseline, but muscle strength worsened significantly over time (p

AB - Background: Frequency and severity of cardiac involvement in DM2 are still controversial. The aims of our study were to determine the frequency and progression of cardiac and muscle involvement in a relatively large cohort of patients with DM2 throughout Italy and Germany and to provide long-term outcomes in this disorder. Methods: 104 DM2 and 117 DM1 patients underwent baseline and follow-up assessments of, ECG, 24 h Holter monitoring, 2D echocardiography and electrophysiological study (EPS) when appropriate, and manual muscle strength testing (mean follow-up: 7.4±4.1 for DM2 and 5.7±4 years for DM1). Results: Overall, 10% of DM2 patients vs 31% of DM1 patients had PR≥200 ms and 17% of DM2 patients vs 48% of DM1 patients had QRSD≥100 ms. Six patients with DM2 vs 28 patients with DM1 required PM/ICD implantations. DM2 patients were stronger than DM1 patients at baseline, but muscle strength worsened significantly over time (p

KW - Cardiac arrhythmias

KW - Myotonic dystrophy type 1

KW - Myotonic dystrophy type 2

KW - Sudden cardiac death

UR - http://www.scopus.com/inward/record.url?scp=84885315415&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84885315415&partnerID=8YFLogxK

U2 - 10.1016/j.ijcard.2012.11.076

DO - 10.1016/j.ijcard.2012.11.076

M3 - Article

C2 - 23266299

AN - SCOPUS:84885315415

VL - 168

SP - 1147

EP - 1153

JO - International Journal of Cardiology

JF - International Journal of Cardiology

SN - 0167-5273

IS - 2

ER -