Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement

Abstract

Aims Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. Methods and results The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich’s ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne’s muscular dystrophy (BMD/DMD, n = 15), Duchenne’s carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. Conclusions 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

Prognostic Utility of Cardiovascular Magnetic Resonance–Based Phenotyping in Patients With Muscular Dystrophy

“… 17 , 23 , 24 Several studies have examined the role of myocardial strain in patients with MD. 25 , 26 , 27 , 28 , 29 Of particular relevance, a recent study also applied a 3D strain analysis technique to study conventional, geometry‐dependent measures in 111 patients with a broad range of neuromuscular diseases inclusive of, but not limited, to MD. 29 Similar to our study, they observed both 3D‐GCS and 3D‐GRS are associated with the presence of replacement fibrosis.…”

“… 25 , 26 , 27 , 28 , 29 Of particular relevance, a recent study also applied a 3D strain analysis technique to study conventional, geometry‐dependent measures in 111 patients with a broad range of neuromuscular diseases inclusive of, but not limited, to MD. 29 Similar to our study, they observed both 3D‐GCS and 3D‐GRS are associated with the presence of replacement fibrosis. Incremental to this publication, our study provided a greater focus on MD, assessed geometry‐independent 3D strain markers, and followed up patients for MACEs to determine the predictive value of these markers.…”

Comprehensive Cardiovascular Management of Myotonic Dystrophy Type 1 Patients: A Report from the Italian Neuro-Cardiology Network

“…Several observational MRI studies showed cardiac structural abnormalities among MD1 subjects, including reduced LV [47,66] or right ventricle [46] systolic function, LV hypertrophy [47] and LV non-compaction [46,67,68]; moreover, reduced values of myocardial strain, both in the longitudinal, circumferential and strain area components, have been described among MD1 patients with preserved LVEF, as per an early detection of LV contractility impairment [66,69]. (Interestingly, a non-negligible prevalence of LV LGE with a non-ischemic distribution pattern (i.e., in the mid-wall or subepicardial myocardial layers), mostly located in the inter-ventricular septum or the postero-lateral wall, has been detected in MD1 subjects, with a prevalence ranging from 12.5% [46,47] to 42% [70][71][72].…”

Imagenetics for Precision Medicine in Dilated Cardiomyopathy

“…Radial and circumferential strain can be also derived by speckle tracking echocardiography or 3-dimensional feature-tracking CMR, albeit with less-well validated cut-off values. 15 Similarly, diastolic dysfunction may be also detectable (eg, reductions in lateral/septal e′ velocities by tissue Doppler imaging) before the development of overt systolic dysfunction.…”