TY - JOUR
T1 - Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study
AU - Hermans, Mieke C. E.
AU - Faber, Catharina G.
AU - Bekkers, Sebastiaan C. A. M.
AU - de Die-Smulders, Christine E. M.
AU - Gerrits, Monique M.
AU - Merkies, Ingemar S. J.
AU - Snoep, Gabriel
AU - Pinto, Yigal M.
AU - Schalla, Simon
PY - 2012/7/24
Y1 - 2012/7/24
N2 - 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
AB - 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
KW - Myotonic dystrophy
KW - Cardiomyopathy
KW - Cardiac magnetic resonance imaging
KW - Endomyocardial fibrosis
U2 - 10.1186/1532-429X-14-48
DO - 10.1186/1532-429X-14-48
M3 - Article
SN - 1097-6647
VL - 14
SP - 46
JO - Journal of Cardiovascular Magnetic Resonance
JF - Journal of Cardiovascular Magnetic Resonance
ER -