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Segmental Strain and Strain-Rate Imaging to Assess Cardiac Function in Patients With Limb-Girdle Muscular Dystrophy
Safak Caglayan1, Hatice Akay Caglayan2,3, Synnøve M Jensen4
1University Hospital of North Norway, Tromsø, Norway.
Insights
Limb-girdle muscular dystrophy R9 (LGMDR9) patients show impaired cardiac function, with reduced ejection fraction and strain, particularly in mid and apical heart segments. Advanced imaging can detect early myocardial dysfunction in LGMDR9.
Area of Science:
- Cardiology
- Neuromuscular Disorders
- Medical Imaging
Background:
- Cardiomyopathy is common in limb-girdle muscular dystrophy R9 (LGMDR9), but its cardiac impact is not fully understood.
- Assessing cardiac function in LGMDR9 is crucial for patient management and understanding disease progression.
Purpose of the Study:
- To evaluate global and segmental myocardial function in patients with LGMDR9 using advanced echocardiography.
- To characterize the extent and pattern of cardiac dysfunction in LGMDR9.
Main Methods:
- Compared 42 LGMDR9 patients with 97 controls using conventional and speckle tracking echocardiography.
- Analyzed left ventricular ejection fraction (LVEF), global longitudinal strain (GLS), and regional strain/strain-rate.
- Adjusted statistical analyses for age and sex.
Main Results:
- LGMDR9 patients had significantly reduced LVEF and GLS compared to controls.
- Both systolic and diastolic dysfunction were observed, with increased end-systolic volumes and altered filling velocities.
- Mid and apical myocardial segments showed reduced strain, while basal segments were less affected. Decreased GLS was noted even in patients with normal LVEF.
Conclusions:
- Patients with LGMDR9 exhibit significant systolic and diastolic myocardial dysfunction, predominantly affecting mid and apical segments.
- Strain and strain-rate imaging are sensitive tools for early detection of cardiac dysfunction in LGMDR9.
- These imaging techniques can aid in clinical management and future interventional studies for LGMDR9.
Purpose:
Cardiomyopathy is a prevalent feature of limb-girdle muscular dystrophy R9 (LGMDR9), but its characterization remains incomplete. This study aims to assess cardiac function in patients with LGMDR9 using global and segmental myocardial function imaging.
Methods:
A total of 42 participants (aged 23-71 years) from the Norwegian LGMDR9 cohort were compared with 97 controls (aged 40-69 years) from the Tromsø Study. Conventional and speckle tracking echocardiography were performed to assess cardiac function. Statistical analyses were adjusted for age and sex.
Results:
Patients with LGMDR9 had significantly reduced left ventricular ejection fraction (LVEF: 52.2% vs. 57.0%, p < 0.001) and decreased absolute global longitudinal strain (GLS: 17.6% vs. 20.5%, p < 0.001). Systolic dysfunction in patients was further observed by increased end-systolic left ventricular volume and internal diameter. Diastolic dysfunction was evident with reduced early to late ventricular filling velocities and further verified by decreased longitudinal strain-rate during early diastole (SR-E) and increased longitudinal strain-rate during atrial contraction (SR-A). Importantly, segmental analysis revealed region-specific differences in strain and strain-rate; mid and apical segments had decreased absolute strain, while basal segments remained largely unaffected. Notably, patients with normal LVEF also displayed decreased GLS (18.7% vs. 20.6%, p < 0.001) and regional and segmental alterations in strain and strain-rate.
Conclusion:
We demonstrate that patients with LGMDR9 exhibit both systolic and diastolic dysfunction, primarily affecting the mid and apical myocardial segments. Strain and strain-rate imaging may serve as sensitive tools for early detection of myocardial dysfunction, and support clinical management and interventional studies in LGMDR9.
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