Cardiovascular Magnetic Resonance in Limb Girdle Muscular Dystrophy
Author Information
Author(s): Xiomara Q. Rosales, Sean J. Moser, Tam Tran, Beth McCarthy, Nicholas Dunn, Philip Habib, Orlando P. Simonetti, Jerry R. Mendell, Subha V. Raman
Primary Institution: The Ohio State University
Hypothesis
This study aims to define cardiac abnormalities in patients with limb girdle muscular dystrophy types 2B and 2I using cardiovascular magnetic resonance.
Conclusion
Patients with LGMD2I and LGMD2B generally show mild cardiac abnormalities, but some may have severe dilated cardiomyopathy.
Supporting Evidence
- The study found that 47% of LGMD patients with normal systolic function had focal myocardial fibrosis.
- One patient with LGMD2I had severe dilated cardiomyopathy, while others generally had normal heart function.
- Diastolic dysfunction was observed in 5 of the 7 LGE-positive patients.
Takeaway
This study looked at how heart health is affected in kids with certain muscle diseases, finding that while most have mild issues, some can have serious heart problems.
Methodology
Patients with genetically-proven LGMD types 2I and 2B underwent cardiac magnetic resonance imaging to assess heart function and structure.
Limitations
The study is limited by the small sample size and the observational nature of the findings.
Participant Demographics
The study included 16 patients (7 with LGMD2I and 9 with LGMD2B) and 8 control subjects, with a mix of genders and ages.
Digital Object Identifier (DOI)
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