Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study

Mieke C. E. Hermans*, Catharina G. Faber, Sebastiaan C. A. M. Bekkers, Christine E. M. de Die-Smulders, Monique M. Gerrits, Ingemar S. J. Merkies, Gabriel Snoep, Yigal M. Pinto, Simon Schalla

*Corresponding author for this work

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Abstract

Background: Myotonic dystrophy type 1 (MD1) is a neuromuscular disorder with potential involvement of the heart and increased risk of sudden death. Considering the importance of cardiomyopathy as a predictor of prognosis, we aimed to systematically evaluate and describe structural and functional cardiac alterations in patients with MD1. Methods: Eighty MD1 patients underwent physical examination, electrocardiography (ECG), echocardiography and cardiovascular magnetic resonance (CMR). Blood samples were taken for determination of NT-proBNP plasma levels and CTG repeat length. Results: Functional and structural abnormalities were detected in 35 patients (44%). Left ventricular systolic dysfunction was found in 20 cases, left ventricular dilatation in 7 patients, and left ventricular hypertrophy in 6 patients. Myocardial fibrosis was seen in 10 patients (12.5%). In general, patients had low left ventricular mass indexes. Right ventricular involvement was uncommon and only seen together with left ventricular abnormalities. Functional or structural cardiac involvement was associated with age (p = 0.04), male gender (p
Original languageEnglish
Pages (from-to)46
JournalJournal of Cardiovascular Magnetic Resonance
Volume14
DOIs
Publication statusPublished - 24 Jul 2012

Keywords

  • Myotonic dystrophy
  • Cardiomyopathy
  • Cardiac magnetic resonance imaging
  • Endomyocardial fibrosis

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