Cardiac manifestations of myotonic dystrophy type 1

Petri, Helle; Vissing, John; Witting, Nanna; Bundgaard, Henning; Køber, Lars

doi:10.1016/j.ijcard.2011.08.037

Cited by 135 publications

(116 citation statements)

References 43 publications

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“…W przeglądzie 18 badań (1828 pacjentów), który przeprowadzili Petri i wsp. [667], blok AV I stopnia stwierdzono u prawie 30% pacjentów, czas trwania zespołu QRS > 120 ms -u 20% pacjentów, częste PVC -u 15% pacjentów, a NSVT -u 4% chorych. Dysfunkcję skurczową LV stwierdzono u 7,2%, a AF lub trzepotanie przedsionków -u 5% pacjentów.…”

Section: Choroby Nerwowo-mięśnioweunclassified

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Priori

Blomström‐Lundqvist²,

Mazzanti³

et al. 2015

Kardiol Pol

233

337

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Section: Choroby Nerwowo-mięśnioweunclassified

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Priori

Blomström‐Lundqvist²,

Mazzanti³

et al. 2015

Kardiol Pol

233

337

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“…88 Dilated cardiomyopathy has been reported, but progressive atrioventricular or intraventricular conduction defects and tachyarrhythmias (ventricular and supraventricular) are the most life-threatening forms of cardiac complications. [89][90][91] In older patients, age-related cardiovascular diseases such as valvulopathy and coronary artery disease may also be observed. 92 Respiratory complications and cardiac arrhythmias are the most frequent primary causes of death in DM1.…”

Section: Myotonic Dystrophymentioning

confidence: 99%

“…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.…”

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

211

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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“…Multiple organs are affected and, among these, the heart. While cardiac involvement is well documented in DM1 [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] and predictors of AV conduction disturbances, cardiac arrhythmias and sudden death are being defined in DM1 [4,[16][17][18][19][20][21] there are still limited data in DM2 [22][23][24][25][26]. In DM2, cardiac abnormalities have been reported to be similar to those described in DM1 [22][23][24][25] but less frequent.…”

Section: Introductionmentioning

confidence: 99%

“…In DM2, cardiac abnormalities have been reported to be similar to those described in DM1 [22][23][24][25] but less frequent. This has led some authors [21,25] to suggest that DM2 may be a more favorable and milder disease compared to DM1. A more recent study including 25 patients with DM2 [27] demonstrated that, despite the older age and greater prevalence of cardiovascular disease (coronary heart disease and heart failure) among these patients, they had a similar rate of severe ECG abnormalities as patients with DM1.…”

Section: Introductionmentioning

confidence: 99%

The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): Long-term outcomes

Sansone

Brigonzi

Schöser

et al. 2013

International Journal of Cardiology

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Cardiac manifestations of myotonic dystrophy type 1

Cited by 135 publications

References 43 publications

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

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

The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): Long-term outcomes

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