Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Schmid, J; Beer, M; Berghold, A; Stojakovic, T; Scharnagl, H; Dieplinger, B; Quasthoff, S; Binder, JS; Rainer, PP.
Cardiac involvement in a cross-sectional cohort of myotonic dystrophies and other skeletal myopathies.
ESC Heart Fail. 2020; 7(4):1900-1908
Doi: 10.1002/ehf2.12763
[OPEN ACCESS]
Web of Science
PubMed
FullText
FullText_MUG
- Führende Autor*innen der Med Uni Graz
- Rainer Peter
- Schmid Johannes
- Co-Autor*innen der Med Uni Graz
- Beer Meinrad
- Berghold Andrea
- Binder Josepha Stephanie
- Quasthoff Stefan
- Scharnagl Hubert
- Stojakovic Tatjana
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- AIMS: Cardiac involvement in myopathies that primarily affect the skeletal muscle is variable and may be subtle, necessitating sensitive diagnostic approaches. Here, we describe the prevalence of cardiac abnormalities in a cohort of patients with skeletal muscle disease presenting at a tertiary care neuromuscular centre. METHODS AND RESULTS: We systematically investigated patients with skeletal myopathies and comprehensively analysed their cardiac phenotype including 24 h electrocardiogram, echocardiography with strain analyses, contrast-enhanced cardiac magnetic resonance imaging, and, if at increased risk of coronary artery disease, computed tomography coronary angiography. We prospectively screened 91 patients with diverse skeletal myopathies and enrolled 73 patients. The most pronounced cardiac involvement was present in patients with dystrophic myopathies (cardiac abnormalities in 59% of patients). We analysed myotonic dystrophies (n = 29) in more detail and found prolonged QRS (99.4 ± 15.6 vs. 91.5 ± 10.3 ms; P = 0.027) and QTc times (441.1 ± 28.1 vs. 413.0 ± 23.3 ms; P < 0.001) and increased left atrial size (27.28 ± 3.9 vs. 25.0 ± 3.2 mm/m2 ; P = 0.021) when compared with healthy controls. Left ventricular systolic function was reduced (ejection fraction < 55%) in 31% of myotonic dystrophies, while only 4% had an ejection fraction < 50%. Apical peak systolic longitudinal strain was slightly reduced (P = 0.023). CONCLUSIONS: Screening for cardiac involvement in the skeletal muscle disease seems prudent particularly in patients with dystrophic myopathies. In the subset of myotonic dystrophy patients, QRS and QTc times as well as myocardial strain may be useful parameters. Their potential for predicting cardiac adverse events needs further evaluation.
- Find related publications in this database (using NLM MeSH Indexing)
- Cross-Sectional Studies - administration & dosage
- Echocardiography - administration & dosage
- Electrocardiography - administration & dosage
- Heart Diseases - complications, diagnosis, epidemiology
- Humans - administration & dosage
- Myotonic Dystrophy - complications, diagnosis, epidemiology
- Find related publications in this database (Keywords)
- Myotonic dystrophy
- Skeletal muscle disease
- Cardiac involvement
- Conduction defect
- Strain
- Cardiac magnetic resonance