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Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.

ABSTRACT: This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD).Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (epsilon(cc)) would decrease in DMD before global systolic functional abnormalities regardless of age or ventricular ejection fraction (EF).We evaluated cardiac magnetic resonance image (MRI) data from 70 DMD patients and 16 aged-matched control subjects. Standard imaging data included steady-state free precession short-axis cine stack images, cine myocardial tagged images, and myocardial delayed enhancement (MDE) (an indicator of myocardial fibrosis) sequences. Analysis was performed with QMASS (Medis Medical Imaging Systems, Leiden, the Netherlands) and HARP (Diagnosoft, Palo Alto, California) software. The DMD patient data were subdivided by age (<10 or >10 years), EF (>55% or <55%), and the presence or absence of MDE.The DMD patients with normal EF had reduced epsilon(cc) at an early age (<10 years) compared with control subjects (p < 0.01). The DMD patients age >10 years with normal EF had further decline in epsilon(cc) compared with younger DMD patients (p < 0.01). There was further decline in epsilon(cc) with age in patients with reduced EF (p < 0.01) without MDE. The oldest patients, with both reduced EF and positive MDE, exhibited the lowest epsilon(cc). None of the patients had ventricular hypertrophy.Myocardial strain abnormalities are prevalent in young DMD patients despite normal EF, and these strain values continue to decline with advancing age. Strain analysis in combination with standard MRI and MDE imaging provides a means to stratify DMD cardiomyopathy.

SUBMITTER: Hor KN

PROVIDER: S-EPMC2709409 | biostudies-literature | 2009 Apr

REPOSITORIES: biostudies-literature

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Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.

Hor Kan N KN Wansapura Janaka J Markham Larry W LW Mazur Wojciech W Cripe Linda H LH Fleck Robert R Benson D Woodrow DW Gottliebson William M WM

Journal of the American College of Cardiology 20090401 14

<h4>Objectives</h4>This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD).<h4>Background</h4>Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (epsilon(cc)) would decrease in DMD before global systolic functional abnormalities regardless of age or ventricular ejection fraction ...[more]

PMID: 19341862

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Project description:BackgroundCardiomyopathy (CMP) is the leading cause of death in Duchenne muscular dystrophy (DMD). Characterization of disease trajectory can be challenging, especially in the early stage of CMP where onset and clinical progression may vary. Traditional metrics from cardiovascular magnetic resonance (CMR) imaging such as LVEF (left ventricular ejection fraction) and LGE (late gadolinium enhancement) are often insufficient for assessing disease trajectory. We hypothesized that strain patterns from a novel 4D (3D+time) CMR regional strain analysis method can be used to predict the rate of DMD CMP progression.MethodsWe compiled 115 short-axis cine CMR image stacks for n=40 pediatric DMD patients (13.6±4.2 years) imaged yearly for 3 consecutive visits and computed regional strain metrics using custom-built feature tracking software. We measured regional strain parameters by determining the relative change in the localized 4D endocardial surface mesh using end diastole as the initial reference frame.ResultsWe first separated patients into two cohorts based on their initial CMR: LVEF≥55% (n=28, normal cohort) and LVEF<55% (n=12, abnormal cohort). Using LVEF decrease measured two years following the initial scan, we further subclassified these cohorts into slow (ΔLVEF%≤5) or fast (ΔLVEF%>5) progression groups for both the normal cohort (n=12, slow; n=15, fast) and the abnormal cohort (n=8, slow; n=4, fast). There was no statistical difference between the slow and fast progression groups in standard biomarkers such as LVEF, age, or LGE status. However, basal circumferential strain (Ecc) late diastolic strain rate and basal surface area strain (Ea) late diastolic strain rate magnitude were significantly decreased in fast progressors in both normal and abnormal cohorts (p<0.01, p=0.04 and p<0.01, p=0.02, respectively). Peak Ea and Ecc magnitudes were also decreased in fast progressors, though these only reached statistical significance in the normal cohort (p<0.01, p=0.24 and p<0.01, p=0.18, respectively).ConclusionRegional strain metrics from 4D CMR can be used to differentiate between slow or fast CMP progression in a longitudinal DMD cohort. These results demonstrate that 4D CMR strain is useful for early identification of CMP progression in patients with DMD.Clinical perspectiveCardiomyopathy is the number one cause of death in Duchenne muscular dystrophy, but the onset and progression of the disease are variable and heterogeneous. In this study, we used a novel 4D cardiovascular magnetic resonance regional strain analysis method to evaluate 40 pediatric Duchenne patients over three consecutive annual visits. From our analysis, we found that peak systolic strain and late diastolic strain rate were early indicators of cardiomyopathy progression. This method offers promise for early detection and monitoring, potentially improving patient outcomes through timely intervention and management.

Project description:BackgroundDuchenne muscular dystrophy (DMD) leads to progressive cardiomyopathy. Detection of myocardial fibrosis with late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) is critical for clinical management. Due to concerns of brain deposition of gadolinium, non-contrast methods for detecting and monitoring myocardial fibrosis would be beneficial.ObjectivesWe hypothesized that native T1 mapping and/or circumferential (εcc) and longitudinal (εls) strain can detect myocardial fibrosis.Methods156 CMRs with gadolinium were performed in 66 DMD boys and included: (1) left ventricular ejection fraction (LVEF), (2) LGE, (3) native T1 mapping and myocardial tagging (εcc-tag measured using harmonic phase analysis). LGE was graded as: (1) presence/absence by segment, slice, and globally; (2) global severity from 0 (no LGE) to 4 (severe); (3) percent LGE using full width half maximum (FWHM). εls and εcc measured using feature tracking. Regression models to predict LGE included native T1 and either εcc-tag or εls and εcc measured at each segment, slice, and globally.ResultsMean age and LVEF at first CMR were 14 years and 54%, respectively. Global εls and εcc strongly predicted presence or absence of LGE (OR 2.6 [1.1, 6.0], p = 0.029, and OR 2.3 [1.0, 5.1], p = 0.049, respectively) while global native T1 did not. Global εcc, εls, and native T1 predicted global severity score (OR 2.6 [1.4, 4.8], p = 0.002, OR 2.6 [1.4, 6.0], p = 0.002, and OR 1.8 [1.1, 3.1], p = 0.025, respectively). εls correlated with change in LGE by severity score (n = 33, 3.8 [1.0, 14.2], p = 0.048) and εcc-tag correlated with change in percent LGE by FWHM (n = 34, OR 0.2 [0.1, 0.9], p = 0.01).ConclusionsPre-contrast sequences predict presence and severity of LGE, with εls and εcc being more predictive in most models, but there was not an observable advantage over using LVEF as a predictor. Change in LGE was predicted by εls (global severity score) and εcc-tag (FWHM). While statistically significant, our results suggest these sequences are currently not a replacement for LGE and may only have utility in a very limited subset of DMD patients.

Dataset Information

Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.

Publications

Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.

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