Prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I

Bhakta, Deepak; Lowe, Miriam R.; Groh, William J.

doi:10.1016/j.ahj.2003.08.008

Cited by 104 publications

(62 citation statements)

References 27 publications

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“…Estimates on the frequency of ECG abnormalities are based mainly on retrospective case series data, 2,3,7 with atrioventricular blocks, bundle branch blocks, atrial flutter or fibrillation, and ventricular and superventricular tachycardia occurring in descending order of frequency. In addition, Bhakta and colleagues 8 revealed that structural abnormalities may also play an important role. Using transthoracic echocardiography to evaluate 382 patients with DM I, left ventricular hypertrophy was identified in 19.8% of patients, left ventricular systolic dysfunction in 14% of cases, and heart failure (HF) in 1.8% of cases based on clinical history.…”

Section: Discussionmentioning

confidence: 99%

Cardiomyopathy and Multiple Pulmonary Emboli in a Patient with Myotonic Dystrophy

McDonnell

Alcantar

Wachsner

et al. 2008

Congestive Heart Failure

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Section: Discussionmentioning

confidence: 99%

Cardiomyopathy and Multiple Pulmonary Emboli in a Patient with Myotonic Dystrophy

McDonnell

Alcantar

Wachsner

et al. 2008

Congestive Heart Failure

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“…79,88,89,206,207 Rarely, dilated cardiomyopathy and HF can occur. 208 Thus, patients with DM and their care providers should be questioned as to the presence of syncope, palpitations, or breathlessness, 79,209 and electrocardiographic abnormalities, non-sinus rhythm, prolongation of the QRS interval (particularly with evidence of HV-interval prolongation), PR interval >240 ms, or higher degree of atrioventricular block should be regarded as a risk factor for sudden death. 79,210,211 Because physical exertion has been observed to be a proarrhythmic influence, serial exercise stress testing has been recommended for young DM1 patients.…”

Section: Cardiac Evaluation In Dmmentioning

confidence: 99%

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Feingold¹,

Mahle²,

Auerbach³

et al. 2017

Circulation

223

195

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For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes. N euromuscular diseases (NMDs) encompass a broad spectrum of diagnoses with overlapping but distinct phenotypes. Common to many NMDs is cardiac involvement. Although the past 3 decades have seen marked advances in our understanding of many NMDs, significant gaps in knowledge remain on how best to approach cardiac care in these patients. For example, survival in Duchenne muscular dystrophy (DMD) has been extended through the use of glucocorticoid use and respiratory support, yet cardiac complications remain a significant cause of morbidity and mortality.1-3 To achieve further gains in care, we will need to improve our understanding of the pathophysiologies driving cardiac involvement in NMDs and advance treatments aimed at preventing the progression of heart failure (HF) and sudden death in NMDs. The recently published findings of an expert working group on cardiac involvement in DMD, which outlined key gaps in knowledge and made specific recommendations aimed at improving diagnosis and management, are a step toward this goal. 4 In this statement, we include a comprehensive overview of the major categories of NMDs with cardiac involvement. For each, a brief background of the gene defect(s), common clinical manifestations (particularly cardiac findings), and current therapies is summarized. Gaps in knowledge are highlighted, and where possible, clinical treatment suggestions are made by the expert writing group appointed by the American Heart Association to review the available literature. Selection of the writing group was performed in accordance with the American Heart Association's conflict-of-interest management policy. Participants volunteered to write sections relevant to their expertise and experience. CLINICAL STATEMENTS AND GUIDELINESconducted a general search of the literature, restricted to human subjects research published between 1980 and 2016. Drafts of each section were written and sent to the chair of the writing group for incorporation into a single document, which was then edited. The edited document was discussed electron...

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“…de sospecha de disfunción miocárdica 11 . Las anomalías de conducción resultan de la hipertrofia de miocitos, fibrosis, infiltración grasa focal e infiltración linfocítica, lo que puede ocurrir en cualquier lugar del sistema de conducción, incluido el sistema HisPurkinje 13 .…”

Section: Tabla 1 Evaluaciones Clínicas Y Tests Para El Diagnóstico Sunclassified

Taquicardia ventricular como expresión de Distrofia Miotónica tipo 1

et al. 2017

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A 48-year-old woman was brought to the emergency room with ventricular tachycardia that was eventually terminated by cardioversion. Clinical and neurologic evaluation, including electro-myography were highly suggestive of type I Myotonic Dystrophy and genetical studies confirmed the diagnosis. A discussion about the diagnostic procedures and management of the disease, especially the associated ventricular tachycardia, is included.

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Prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I

Cited by 104 publications

References 27 publications

Cardiomyopathy and Multiple Pulmonary Emboli in a Patient with Myotonic Dystrophy

Cardiomyopathy and Multiple Pulmonary Emboli in a Patient with Myotonic Dystrophy

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Taquicardia ventricular como expresión de Distrofia Miotónica tipo 1

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