Cardiac involvement over 10 years in myotonic and Becker muscular dystrophy and mitochondrial disorder

Finsterer, Josef; Stöllberger, Claudia; Blazek, Gerhard; Kunafer, Michael; Prager, Elfriede

doi:10.1016/j.ijcard.2006.07.121

Cited by 18 publications

(22 citation statements)

References 48 publications

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“…In a study of six patients (14), ECG abnormalities included IRBBB and intraventricular conduction delay. In a 10-year study of five patients (33), sinus tachycardia, ST elevation, IRBBB and, in one patient, tall R and S waves were found (33). In some patients, CI may manifest as a complete AV block early in life (34) or late in life (35).…”

Section: Blood Chemical Investigationsmentioning

confidence: 96%

Cardiac involvement in Becker muscular dystrophy

Finsterer¹,

Stöllberger²

2008

Canadian Journal of Cardiology

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Section: Blood Chemical Investigationsmentioning

confidence: 96%

Cardiac involvement in Becker muscular dystrophy

Finsterer¹,

Stöllberger²

2008

Canadian Journal of Cardiology

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“…Previous studies of cardiac involvement in MD patients have mostly focused on cardiomyopathy11-13, 22, 23), and the studies that do focus on conduction abnormalities have been restricted to Kerns-Sayre syndrome, which is associated with long QT syndrome24, 25). In this study, we could observe a variety of ECG abnormalities in our patients.…”

Section: Discussionmentioning

confidence: 60%

“…It follows that cardiovascular involvement could also be an important prognostic factor in MD11, 12). A previous study reported that the prevalence of cardiovascular involvement ranked second behind visceral involvement in patients with MD13). Also, Finsterer14) followed 113 MRC defect patients over a ten-year period and found that 40% of the patients developed heart disease including cardiomyopathy and heart failure.…”

Section: Introductionmentioning

confidence: 99%

Electrocardiography as an early cardiac screening test in children with mitochondrial disease

et al. 2010

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PurposeTo evaluate myocardial conductivity to understand cardiac involvement in patients with mitochondrial disease.MethodsWe performed retrospective study on fifty-seven nonspecific mitochondrial encephalopathy patients with no clinical cardiac manifestations. The patients were diagnosed with mitochondrial respiratory chain complex defects through biochemical enzyme assays of muscle tissue. We performed standard 12-lead electrocardiography (ECG) on all patients.ResultsECG abnormalities were observed in 30 patients (52.6%). Prolongation of the QTc interval (>440 ms) was seen in 19 patients (33.3%), widening of the corrected QRS interval in 15 (26.3%), and bundle branch block in four (7.0%). Atrioventricular block, premature atrial contraction and premature ventricular contraction were seen in two patients each (3.5%) and Wolff-Parkinson-White syndrome in one patient (1.8%).ConclusionGiven this finding, we recommend active screening with ECG in patients with mitochondrial disease even in patients without obvious cardiac manifestation.

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“…While cardiac involvement (CI) in MD is usually characterized by arrhythmias (conduction abnormalities and tachyarrhythmias), myocardial damage with symptoms of heart failure is rare because CI is mainly due to selective degeneration of the infra-nodal conduction system 3-5). On histologic evaluation, CI in MD involves interstitial myocardial fibrosis and fatty infiltration 6).…”

Section: Discussionmentioning

confidence: 99%