Detection of Progressive Cardiac Dysfunction by Serial Evaluation of Circumferential Strain in Patients With Duchenne Muscular Dystrophy

Hagenbuch, Sean; Gottliebson, William; Wansapura, Janaka; Mazur, Wojciech; Fleck, Robert; Benson, D. Woodrow; Hor, Kan N.

doi:10.1016/j.amjcard.2009.12.070

Cited by 64 publications

(56 citation statements)

References 11 publications

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“…Similar findings showed that abnormal myocardial strain precedes both the age-dependent decrease in ejection fraction and the development of myocardial fibrosis in DMD (134). Finally, strain analysis is more sensitive to detect serial impairment in LV function compared with LV ejection fraction (135); therefore, it is of great value to assess treatment efficacy. These indexes, although promising, are not widely accepted and do not represent a robust tool for detection of cardiac involvement in MD.…”

Section: Why Cmr In Md?supporting

confidence: 57%

Muscular dystrophies and the heart: The emerging role of cardiovascular magnetic resonance imaging

Mavrogeni¹,

Markousis‐Mavrogenis²,

Papavasiliou³

et al. 2015

Curr Res Cardiol

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Section: Why Cmr In Md?supporting

confidence: 57%

Muscular dystrophies and the heart: The emerging role of cardiovascular magnetic resonance imaging

Mavrogeni¹,

Markousis‐Mavrogenis²,

Papavasiliou³

et al. 2015

Curr Res Cardiol

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“…Given the recognized limitations of TTE in this population, CMR with contrast enhancement has been used routinely in the surveillance of global and regional cardiac function, and evaluation of myocardial fibrosis in DMD patients [12,13]. Using CMR, we demonstrated that myocardial circumferential strain (e cc ) is a sensitive indicator of early systolic dysfunction, apparent well before changes can be detected by traditional functional parameters such as shortening fraction (SF) or EF [12].…”

Section: Introductionmentioning

confidence: 96%

Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain

Mazur

Hor

Germann

et al. 2011

Int J Cardiovasc Imaging

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The cardiac disease ubiquitously associated in Duchenne Muscular Dystrophy (DMD) has traditionally been considered a progressive dilated cardiomyopathy (DCM). However, left ventricular (LV) dilatation as measured with cardiac MRI has not been a consistent finding in this population, even as circumferential strain (ε(cc)) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of ε(cc) decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, ε(cc) and myocardial delayed enhancement (MDE) were determined in DMD patients and normal control subjects. The LV Remodeling Index (LVRI) was calculated as the ratio of LV Mass to Volume (LVM/EDV). Statistical comparisons between all LV parameters and genotype were also performed. Median LVRI in DMD (n = 127) and control subjects (n = 41) were different (0.75 vs. 0.65, P = 0.0150) but within normal range. Furthermore, the median LVRI in DMD boys with reduced LV systolic function was significantly reduced compared to those with normal LV systolic function (0.64 vs. 0.75, P = 0.0974). However, the presence of MDE was associated with a lower median LVRI (0.57 vs. 0.76, P = 0.0471). Regression analysis showed no significant correlation between ε(cc) and LVRI (r = -0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with ε(cc.) Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.

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“…However, despite the clinical challenges, CMR imaging has been shown to be sensitive for early findings of cardiac disease prior to systolic dysfunction including reduced myocardial circumferential strain and areas of fibrosis identified with the use of late gadolinium enhancement. [26][27][28] CMR has also been used to characterize the pattern of remodelling and changes in LV geometry that occur with cardiac disease in DMD as a unique process not fully explained by the traditional thinking of progressive dilated cardiomyopathy. 29 CMR is not currently included in clinical guidelines for DMD, but it is a promising area of research to further understand the development and progression of cardiac disease in this population.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

et al. 2014

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As life expectancy of patients with Duchenne muscular dystrophy (DMD) has increased to the 5th decade, in part due to improved ventilatory support, cardiomyopathy is projected to increase as a cause of death. International guidelines recommend an annual assessment of cardiac function and initiation of appropriate pharmacological treatment. We conducted an audit of the cardiac management in patients with DMD requiring ventilatory support and reported a case series of the collated cardiac investigations. Patients with DMD requiring ventilatory support were included in the study. The date of the last electrocardiogram (ECG), echocardiogram (ECHO), cardiology review and pharmacological management were retrieved from the medical records. If an annual cardiac assessment had not been performed this was requested and the latest ECGs and ECHO reports were collated. A total of 30 patients with DMD (29 males, mean (SD) age of 30 (7) years) met the inclusion criteria. Although there was ECG and ECHO documentation in 24 and 21 individuals, respectively, it was only recent in 10 and 6 individuals. In all, 60% of patients had been assessed by a cardiologist, but only 10% within the last year. Over half of the patients failed to attend their new appointments. From the available results, 18 of the 19 patients had an abnormal ECG, 11 of the 16 patients had left ventricular (LV) impairment and 55% of patients had a change in prescription following cardiac investigations. There is a need for a coordinated cardiorespiratory approach towards adult patients with DMD. Over a third of patients had normal LV function suggesting that cardiomyopathy is not inevitable in this group.

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Detection of Progressive Cardiac Dysfunction by Serial Evaluation of Circumferential Strain in Patients With Duchenne Muscular Dystrophy

Cited by 64 publications

References 11 publications

Muscular dystrophies and the heart: The emerging role of cardiovascular magnetic resonance imaging

Muscular dystrophies and the heart: The emerging role of cardiovascular magnetic resonance imaging

Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

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