Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies
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 terti...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2020-08-01
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| Series: | ESC Heart Failure |
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| Online Access: | https://doi.org/10.1002/ehf2.12763 |
| _version_ | 1818916503210164224 |
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| author | Johannes Schmid Meinrad Beer Andrea Berghold Tatjana Stojakovic Hubert Scharnagl Benjamin Dieplinger Stefan Quasthoff Josepha S. Binder Peter P. Rainer |
| author_facet | Johannes Schmid Meinrad Beer Andrea Berghold Tatjana Stojakovic Hubert Scharnagl Benjamin Dieplinger Stefan Quasthoff Josepha S. Binder Peter P. Rainer |
| author_sort | Johannes Schmid |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-20T00:19:12Z |
| format | Article |
| id | doaj.art-70f299ccb84846d5a2793a6bf3036c00 |
| institution | Directory Open Access Journal |
| issn | 2055-5822 |
| language | English |
| last_indexed | 2024-12-20T00:19:12Z |
| publishDate | 2020-08-01 |
| publisher | Wiley |
| record_format | Article |
| series | ESC Heart Failure |
| spelling | doaj.art-70f299ccb84846d5a2793a6bf3036c002022-12-21T20:00:14ZengWileyESC Heart Failure2055-58222020-08-01741900190810.1002/ehf2.12763Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathiesJohannes Schmid0Meinrad Beer1Andrea Berghold2Tatjana Stojakovic3Hubert Scharnagl4Benjamin Dieplinger5Stefan Quasthoff6Josepha S. Binder7Peter P. Rainer8Division of Cardiology, Department of Internal Medicine Medical University of Graz Auenbruggerplatz 15 8036 Graz AustriaClinic for Diagnostic and Interventional Radiology University Hospital Ulm Albert‐Einstein‐Allee 23 89081 Ulm GermanyInstitute for Medical Informatics, Statistics and Documentation Medical University of Graz Auenbruggerplatz 2 8036 Graz AustriaClinical Institute of Medical and Chemical Laboratory Diagnostics University Hospital Graz Auenbruggerplatz 15 8036 Graz AustriaClinical Institute of Medical and Chemical Laboratory Diagnostics Medical University of Graz Auenbruggerplatz 15 8036 Graz AustriaDepartment of Laboratory Medicine Konventhospital Barmherzige Brueder Linz Seilerstaette 4 4010 Linz AustriaDepartment of Neurology Medical University of Graz Auenbruggerplatz 22 8036 Graz AustriaDivision of Cardiology, Department of Internal Medicine Medical University of Graz Auenbruggerplatz 15 8036 Graz AustriaDivision of Cardiology, Department of Internal Medicine Medical University of Graz Auenbruggerplatz 15 8036 Graz AustriaAbstract 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.https://doi.org/10.1002/ehf2.12763Myotonic dystrophySkeletal muscle diseaseCardiac involvementConduction defectStrainCardiac magnetic resonance |
| spellingShingle | Johannes Schmid Meinrad Beer Andrea Berghold Tatjana Stojakovic Hubert Scharnagl Benjamin Dieplinger Stefan Quasthoff Josepha S. Binder Peter P. Rainer Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies ESC Heart Failure Myotonic dystrophy Skeletal muscle disease Cardiac involvement Conduction defect Strain Cardiac magnetic resonance |
| title | Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies |
| title_full | Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies |
| title_fullStr | Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies |
| title_full_unstemmed | Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies |
| title_short | Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies |
| title_sort | cardiac involvement in a cross sectional cohort of myotonic dystrophies and other skeletal myopathies |
| topic | Myotonic dystrophy Skeletal muscle disease Cardiac involvement Conduction defect Strain Cardiac magnetic resonance |
| url | https://doi.org/10.1002/ehf2.12763 |
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