Myotonic heart disease

Hawley, Rollin J.; Milner, Mark R.; Gottdiener, John S.; Cohen, Andrew I.

doi:10.1212/wnl.41.2_part_1.259

Cited by 82 publications

(39 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…8 9 19 The mean value for our patients was 2.5 (4.5) ms/year, or 1.2% per year, similar to that of 1.5% found by Hawley and colleagues. 8 Patients with larger CTG expansions were more likely to have prolonged QRS complexes at baseline, and had higher rates of increase during follow up. A previous report found no correlation between conduction disease and mutation size.…”

Section: Study Populationmentioning

confidence: 92%

“…5 6 Sudden death is rare, and the relative importance of ventricular tachyarrhythmias and bradyarrhythmias remains unclear. [7][8][9][10][11] Prediction of cardiac events has proved diYcult, and several potential risk factors have been evaluated. There is an association between neurological severity and both electrocardiographic conduction system disease and cardiac events.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Endomysium in left ventricle

Macchiarelli¹

2001

Heart

View full text Add to dashboard Cite

Objective-To assess whether the size of the cytosine-thymine-guanine (CTG) expansion mutation in myotonic dystrophy predicts progression of conduction system disease and cardiac events. Design-Longitudinal study involving ECG and clinical follow up over (mean (SD)) 4.8 (1.8) and 6.2 (1.9) years, respectively, of patients stratified by CTG expansion size (E0 to E4). Patients-73 adult patients under annual review in a regional myotonic dystrophy clinic. Patients were grouped into E0/E1 (n = 25), E2 (n = 34), and E3/E4 (n = 14). Results-The proportion of patients with a QRS complex > 100 ms at baseline increased with the size of the CTG expansion (EO/E1, 4%; E2, 12%; E3/E4, 36%; p = 0.02). This trend was more pronounced at follow up (E0/E1, 4%; E2, 21%; E3/E4, 57%; p = 0.0004). The rate of widening of the QRS complex (ms/year) was similarly related to the size of the mutation (EO/E1, 0.4 (1.3); E2, 1.4 (2.5); E3/E4, 1.5 (1.6); p = 0.04). First degree atrioventricular block was present in 23% of patients at baseline and 34% at follow up, with no significant relation to expansion size. Seven patients suVered a cardiac event during follow up (sudden death in two, permanent pacemaker insertion in three, chronic atrial arrhythmia in two), of whom six were in CTG expansion group E2 or greater. Patients who experienced a cardiac event during follow up had more rapid rates of PR interval increase (9.9 (11.1) v 1.6 (2.9) ms/year; p = 0.008) and a trend to greater QRS complex widening (3.6 (4.5) v 0.9 (1.5) ms/year; p = 0.06) than those who did not. Conclusions-Larger CTG expansions are associated with a higher rate of conduction disease progression and a trend to increased risk of cardiac events in myotonic dystrophy. (Heart 2001;86:411-416)

show abstract

Section: Study Populationmentioning

confidence: 92%

mentioning

confidence: 99%

Endomysium in left ventricle

Macchiarelli¹

2001

Heart

View full text Add to dashboard Cite

show abstract

“…Cardiac involvement (CI) is a common finding in patients with myotonic dystrophy (MD), Becker’s muscular dystrophy (BMD) and mitochondrial myopathy (MMP), possibly leading to cardiac dysfunction and even death [1, 2, 3, 4, 5, 6, 7, 8, 9]. CI seems to be unrelated to the clinical severity or duration of the skeletal muscle disorder and can even precede the onset of neurologic symptoms [9, 10, 11, 12].…”

Section: Introductionmentioning

confidence: 99%

Progression of Cardiac Involvement in Patients with Myotonic Dystrophy, Becker’s Muscular Dystrophy and Mitochondrial Myopathy during a 2-Year Follow-Up

Stöllberger

Finsterer²,

Keller³

et al. 1998

Cardiology

View full text Add to dashboard Cite

The aim of this prospective noninvasive follow-up study was (1) to assess the progression of cardiac involvement (CI) in patients with myotonic dystrophy (MD), Becker’s muscular dystrophy (BMD) and mitochondrial myopathy (MMP), (2) to find out if CI and neurologic impairment are related and (3) to determine how often cardiac investigations should be performed. Clinical, electrocardiographic, echocardiographic and 24-hour ambulatory electrocardiographic examinations were performed at yearly intervals. CI was assessed qualitatively by the presence of pathologic examinations and quantitatively by the number of pathologic examinations. Qualitatively, CI was present at baseline in 36 of the 38 cases (18–68 years) and the number of cases did not change. Quantitatively, CI progressed after 1 (2) year in 50% (50%) of patients with MD (n = 16), 0% (43%) of patients with BMD (n = 7) and 27% (27%) of patients with MMP (n = 15). CI and neurologic impairment were not related (p = 0.54). Cardiac investigations should be repeated at yearly intervals irrespective of whether there is neurologic deterioration or not.

show abstract

“…Aucune variable clinique, telle que la progression de la faiblesse musculaire, ne peut aider à prédire la progression de l'atteinte du système de conduction cardiaque (Hawley et al 1991). Les anomalies cardiaques les plus fréquemment observées à l'ECG consistent en intervalles PR prolongés et en délais de conduction intraventriculaire (Nguyen et al 1988).…”

Section: La Myotonieunclassified

“…D'autres anomalies fatales peuvent survenir dans la maladie: apnée du sommeil et hypertension pulmonaire (Coccagna et al 1975), prolapsus de la valvule mitrale (Winters et al 1976), insuffisance ventriculaire gauche (Church 1967), traumatismes découlant de la faiblesse musculaire (Hawley et al 1991), infarctus du myocarde (Nguyen et al 1988), asphyxie par aspiration alimentaire (De Backer et al 1976) et embolie pulmonaire associée à un prolapsus de la valvule mitrale (Cooke et al 1978 …”

Section: Causes De Décèsunclassified

Étude descriptive de la mortalité dans la dystrophie myotonique au Saguenay-Lac-Saint-Jean /

Potvin¹

1994

View full text Add to dashboard Cite

Myotonic heart disease

Cited by 82 publications

References 0 publications

Endomysium in left ventricle

Endomysium in left ventricle

Progression of Cardiac Involvement in Patients with Myotonic Dystrophy, Becker’s Muscular Dystrophy and Mitochondrial Myopathy during a 2-Year Follow-Up

Étude descriptive de la mortalité dans la dystrophie myotonique au Saguenay-Lac-Saint-Jean /

Contact Info

Product

Resources

About