The Natural Course of Cardiac Conduction Disturbances in Myotonic Dystrophy

Fragola, Pietro V.; Autore, Camillo; Magni, Giuseppina; Antonini, Giovanni; Picelli, Antonella; Cannata, D

doi:10.1159/000174865

Cited by 28 publications

(14 citation statements)

References 11 publications

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“…These patients exhibited many of the classic electrocardiographic features of myotonic dystrophy,5 6but there were no significant correlations between the measured ECG intervals and diastolic filling times. Neither were there any significant associations between the presence of diastolic and conduction abnormalities.…”

Section: Discussionmentioning

confidence: 78%

“…This is mainly manifest in disturbances of the conduction system, with first degree heart block, variable degrees of bundle branch block, and complete heart block being described 45 Such conduction abnormalities tend to be progressive, carry a risk of sudden death, and may require permanent pacemaker insertion6 7 during adulthood. Atrial7 8 and ventricular tachyarrhythmias9 10 are also reported.…”

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confidence: 99%

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Left ventricular diastolic function in congenital myotonic dystrophy

Bu’Lock

Sood²,

Giovanni³

et al. 1999

Archives of Disease in Childhood

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Objective-Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. Design-Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. Results-Fractionalshortening was slightly reduced (by 28-29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74 v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68 v 81 beats/ min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. Conclusions-Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study. (Arch Dis Child 1999;80:267-270)

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Section: Discussionmentioning

confidence: 78%

mentioning

confidence: 99%

Left ventricular diastolic function in congenital myotonic dystrophy

Bu’Lock

Sood²,

Giovanni³

et al. 1999

Archives of Disease in Childhood

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“…Cardiac arrhythmia is due to selective degeneration (fibrosis, infiltration and atrophy) of the cardiac conduction system [20]. Heart involvement was found to be progressive during the course of the disease [8,22]. The cardiac complications have been correlated inconsistently with the length of the CTG triplet repeat [1,11,16,18].…”

Section: Introductionmentioning

confidence: 99%

Cardiac involvement and CTG expansion in myotonic dystrophy

Merlevede¹,

Vermander

Theys

et al. 2002

Journal of Neurology

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Although cardiac complications are well known in myotonic dystrophy (DM), patients rarely manifest symptoms of cardiac disease, and if so they most often show conduction abnormalities or arrhythmia. In this study, specific cardiac findings were reviewed in 79 patients with DM. No correlation was found between the cardiac assessments and the CTG expansion. Thus, for a single patient the cardiac involvement in the disease can not be predicted from the findings of the genetic investigation. On the other hand, a clear positive relationship of the PR interval with the QRS duration was revealed, as well as a positive correlation between the age of the DM patient and the QRS duration, which increases with 0,54 ms/year. Systolic dysfunction, evaluated by transthoracic echocardiography, seems to be quite uncommon. In 32 % of the patients with a normal ECG, the 24 h Holter monitoring showed arrhythmias and conduction abnormalities. Based on these findings we recommend a follow up of DM patients not only based on the ECG, but also through 24 h Holter monitoring.

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“…Although fol low-up has not been conducted for a sufficiently long peri od to provide data regarding the likelihood of subsequent overt myocardial impairment, none of our patients mani fested symptoms of cardiac failure during observation. Also, it should be pointed out that during the natural course of heart involvement in myotonic dystrophy, pri mary myocardial impairment does not represent a clinical problem in the vast majority of these patients [4][5][6]. It is also possible that abnormal left ventricular relaxation does not indicate a true diastolic dysfunction that will become clinically evident with time, but rather represent an intrinsic feature of the primary underlying myocardial disease process in myotonic dystrophy (myocardial myo tonia).…”

Section: Discussionmentioning

confidence: 99%

Doppler Echocardiographic Assessment of Left Ventricular Diastolic Function in Myotonic Dystrophy

Fragola

Calò²,

Luzi

et al. 1997

Cardiology

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We utilized Doppler echocardiography to characterize left ventricular diastolic function in 42 patients with myotonic dystrophy (mean age 37 ± 12 years, 64% male) who had no symptoms of heart failure and had normal left ventricular systolic function. Data were compared with those in 41 normal control subjects of similar age and gender. Heart rate, systemic blood pressure, and cardiac dimensions (wall thickness, left atrial and left ventricular cavity dimensions) were similar and not significantly different in patients and controls. As a group, patients showed significantly increased deceleration time and decreased rate of decline of flow velocity in early diastole (p < 0.0001 and p < 0.01, respectively) when compared to controls. Individual patient analysis showed that 10 (24%) of the 42 patients with myotonic dystrophy had 2 or more abnormal Doppler indexes of diastolic function consistent with a pattern of impaired left ventricular relaxation. The most common abnormalities were increased deceleration time ( > 224 ms; 9 patients), prolonged isovolumic relaxation time ( > 103 ms; 8 patients) and reduced rate of decline of flow velocity in early diastole ( < 2.1 m/s²; 5 patients). In addition, peak early diastolic flow velocity was reduced ( < 43 cm/s) in 3 patients and early to atrial peak flow velocity ratio was reduced ( < 1) in 2 patients. Comparison of subgroups of patients with and without abnormal Doppler indexes showed no significant differences with regard to age, gender, heart rate, systemic blood pressure, severity of neuromuscular disease, and cardiac dimensions. After study, patients were clinically followed up for a mean period of 20 ± 7 months (range 12-35). During observation no patients died and none experienced symptoms of heart failure. This Doppler echocardiographic analysis demonstrates that diastolic abnormalities may be present in patients with myotonic dystrophy, even in the absence of symptoms of cardiac failure or left ventricular systolic dysfunction. These diastolic abnormalities suggest an intrinsic myocardial abnormality in patients with myotonic dystrophy; however, whether they represent a preclinical phase of myocardial involvement or an intrinsic feature of the primary myocardial disease process in myotonic dystrophy remains to be elucidated.

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The Natural Course of Cardiac Conduction Disturbances in Myotonic Dystrophy

Cited by 28 publications

References 11 publications

Left ventricular diastolic function in congenital myotonic dystrophy

Left ventricular diastolic function in congenital myotonic dystrophy

Cardiac involvement and CTG expansion in myotonic dystrophy

Doppler Echocardiographic Assessment of Left Ventricular Diastolic Function in Myotonic Dystrophy

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