Utility of real-time three-dimensional echocardiography for Duchenne muscular dystrophy with echocardiographic limitations

Tsuburaya, Ryuji; Uchizumi, Hiroshi; Ueda, Mitsuharu; Demura, Yutaka; Mukaida, Souichi; Sudou, Shinji; Irahara, K; Sakai, Naoko; Shiraishi, Kazuhiro

doi:10.1016/j.nmd.2013.12.007

Cited by 5 publications

(4 citation statements)

References 23 publications

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“…Yu et al demonstrated that (E A ) derived from 3D-STE had an 85.7% sensitivity and a 71.0% specificity for differentiating DMD patients ( n = 56) from controls ( n = 31; ( 38 ); Table 1 ). While these metrics show improvement over 2D-STE-derived metrics, this method still suffers from limited echocardiographic windows, low spatial and temporal resolution, relatively small study populations, and inter-vendor differences ( 11 , 32 , 46 – 48 ).…”

Section: Transthoracic Echocardiographymentioning

confidence: 99%

Myocardial strain imaging in Duchenne muscular dystrophy

Earl

Soslow

Markham

et al. 2022

Front. Cardiovasc. Med.

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Cardiomyopathy (CM) is the leading cause of death for individuals with Duchenne muscular dystrophy (DMD). While DMD CM progresses rapidly and fatally for some in teenage years, others can live relatively symptom-free into their thirties or forties. Because CM progression is variable, there is a critical need for biomarkers to detect early onset and rapid progression. Despite recent advances in imaging and analysis, there are still no reliable methods to detect the onset or progression rate of DMD CM. Cardiac strain imaging is a promising technique that has proven valuable in DMD CM assessment, though much more work has been done in adult CM patients. In this review, we address the role of strain imaging in DMD, the mechanical and functional parameters used for clinical assessment, and discuss the gaps where emerging imaging techniques could help better characterize CM progression in DMD. Prominent among these emerging techniques are strain assessment from 3D imaging and development of deep learning algorithms for automated strain assessment. Improved techniques in tracking the progression of CM may help to bridge a crucial gap in optimizing clinical treatment for this devastating disease and pave the way for future research and innovation through the definition of robust imaging biomarkers and clinical trial endpoints.

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Section: Transthoracic Echocardiographymentioning

confidence: 99%

Myocardial strain imaging in Duchenne muscular dystrophy

Earl

Soslow

Markham

et al. 2022

Front. Cardiovasc. Med.

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“…Any patient reporting cardiac symptoms, and all patients suffering from conditions that are associated with cardiac dysfunction (the hereditary muscular dystrophies, myotonic dystrophy, mitochondrial disorders and congenital myopathies) [75,76], should be screened regularly with electrocardiograms for arrhythmias [77] and echocardiograms for ventricular dysfunction [76]. It is important to note that the value of echocardiogram decreases with progressive scoliosis and obesity, and so the availability of cardiac magnetic resonance imaging [78] or realtime three-dimensional echocardiography [79] is an important consideration. The availability of a dedicated cardiologist with expertise in NMD in the respiratory NMD MDT would be the gold standard, so that patients can be evaluated and reviewed at the same time.…”

Section: Cardiovascular Carementioning

confidence: 99%

The adult multidisciplinary respiratory neuromuscular clinic

Shah

Murphy

Kaltsakas

2020

Breathe

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Many neuromuscular disorders (NMD) are complicated by respiratory failure. These patients are best managed in a multidisciplinary outpatient clinic to provide timely access to the various disciplines they require. The key mainstay of treatment of respiratory failure in patients with NMD is noninvasive ventilation, supported by secretion clearance, speech and language therapy, optimisation of nutrition and the maintenance of mobility. Patients with specific conditions may also require cardiology, neurology, orthopaedics, urology and psychological services. The respiratory NMD multidisciplinary team should also provide access to palliative care, and caregiver health and wellbeing should also be reviewed at clinical reviews. The future of care for the respiratory NMD patient will increasingly involve home services and telehealth and the clinic should be equipped and resourced to deliver these. Although not all health systems will be able to provide all elements of the multidisciplinary team discussed here, this review provides the “ideal” recipe for the adult multidisciplinary team and the evidence base underpinning this from which a clinic can be developed.Educational aimsTo provide an overview of the care of an adult neuromuscular disorder patient presenting to the multidisciplinary respiratory clinic.To provide the evidence base for establishing the different elements of the multidisciplinary respiratory clinic.

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“…Clinically overt heart failure, however, was documented in only 2%, according to the same report. In a study evaluating diastolic heart function in MD1 using echocardiography, an increase of the left atrial diameter and mitral deceleration time suggesting diastolic abnormalities was identified (127). In cases with a suboptimal acoustic window, three-dimensional echocardiography can serve as an excellent alternative (128).…”

Section: Currently Used Techniques For the Evaluation Of Heart Involvmentioning

confidence: 99%