Left Ventricular Magnetic Resonance Imaging Strain Predicts the Onset of Duchenne Muscular Dystrophy–Associated Cardiomyopathy

Abstract

Background: Early detection of left ventricular (LV) dysfunction before the onset of overt Duchenne muscular dystrophy–associated cardiomyopathy (DMDAC) may direct clinical management to slow onset of dysfunction. We aimed to assess whether LV strain will predict those who develop DMDAC. Methods: We performed a single center retrospective case control study of patients with Duchenne muscular dystrophy who underwent serial cardiac magnetic resonance between 2006 and 2019. Patients with Duchenne muscular dystrophy with an LV ejection fraction ≥55% on ≥1 cardiac magnetic resonance were identified and grouped into age-matched +DMDAC and –DMDAC. Within 3 years, +DMDAC had a subsequent cardiac magnetic resonance with a decline in LV ejection fraction ≥10% and absolute LV ejection fraction ≤50%. −DMDAC maintained an LV ejection fraction ≥55% on serial cardiac magnetic resonances. Two-dimensional and 3-dimensional global radial strain, global circumferential strain (GCS), and global longitudinal strain were measured using tissue tracking software and their ability to predict DMDAC onset was assessed. Multivariable analysis adjusted for late gadolinium enhancement. Results: Thirty +DMDAC and 30 age-matched −DMDAC patients were included with a total of 164 studies analyzed. Before DMDAC onset, 2-dimensional global radial strain and GCS were significantly worse in +DMDAC compared with −DMDAC (25.1±6.0 versus 29.0±6.3, P =0.011; −15.4%±2.4 versus −17.3%±2.6, P =0.003). Three-dimensional GCS and global radial strain had similar findings. Among strain measures, 3-dimensional GCS had the highest area under the curve to predict DMDAC in our cohort. These findings persisted after adjusting for the presence of late gadolinium enhancement. Conclusions: Reduced global radial strain and GCS may predict those at risk for developing DMDAC before onset of LV dysfunction and its clinical utility warrants further exploration.

Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging

“…Though the values for these volumetric and mass parameters were lower than the unaffected population, they were still within the normal range except for LVEDV. Our results agree with previous cross sectional studies [27,28,32,48] which have found composite circumferential strain on tagged and CINE images (using feature tracking) strain to be significantly worse in comparison to healthy controls and to be affected earlier in the disease process prior to structural remodeling [28,49]. These findings emphasize the importance of measuring strain in this patient population at an early stage of the disease.…”

“…Based on these limitations, there is a need to develop cardiac assessment tools that are non-invasive, safe, and sensitive to detect cardiac dysfunction at an early stage [2,3,19,25,26]. Various non-invasive techniques such as circumferential strain using tagged and feature tracking techniques, T1 mapping, and change in volumetric functions have been used to quantify disease progression in DMD [26][27][28][29].…”

“…Peak strain can be defined as a measure of biomechanical distortion in tissue length with respect to resting length in the heart wall as it contracts [30,31]. Studies in DMD have reported circumferential peak strain (ℇcc%) at the mid-ventricular level is a sensitive marker to examine cardiac involvement and the effect of therapeutic interventions on cardiac muscle [3,27,28,[32][33][34]. A major advantage of ℇcc% is its ability to detect changes at both global and regional levels of myocardium without use of any contrast [3,28,32].…”

The association between cardiac involvement and long‐term clinical outcomes in patients with Duchenne muscular dystrophy

“…Cardiovascular manifestations of DMD vary and include dilated cardiomyopathy, arrhythmias, and congestive heart failure 4,5 . Because cardiovascular events frequently occur during DMD progression, non‐invasive imaging techniques and laboratory tests can help identify patients at risk of clinical deterioration and assess treatment response 6–10 . However, early prediction of disease progression for DMD is challenging despite emphasizing the increased mortality rate of DMD patients due to cardiomyopathy.…”

“… 4 , 5 Because cardiovascular events frequently occur during DMD progression, non‐invasive imaging techniques and laboratory tests can help identify patients at risk of clinical deterioration and assess treatment response. 6 , 7 , 8 , 9 , 10 However, early prediction of disease progression for DMD is challenging despite emphasizing the increased mortality rate of DMD patients due to cardiomyopathy. However, despite emphasizing the increased mortality rate of DMD patients due to cardiomyopathy, early prediction of disease progression for DMD is challenging due to the rarity of DMD 11 and the difficulty of gathering data to investigate long‐term effects regarding left ventricular systolic functions.…”