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Polymyositic heart disease.

There is an apparent correlation between the severity and duration of skeletal muscle involvement, cardiac manifestations and the extent of conduction system disease in polymyositis. Cardiac involvement during the course of polymyositis has been recognized as one of the typical features of skeletal muscle myositis. We report a patient with polymyositis in whom bifascicular block, prolonged P-R interval and congestive heart failure appeared three years before any clinical or laboratory evidence of active skeletal muscle myositis. To the best of our knowledge, this is the first report of polymyositis where cardiac manifestations preceded those of skeletal muscle myositis.

Cardiomyopathies↗

Does cytosine-thymine-guanine (CTG) expansion size predict cardiac events and electrocardiographic progression in myotonic dystrophy?

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 suffered 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.

Adult↗

Multifocal disturbance of cardiac conduction system.

The group of 127 patients with pathological finding in cardiac conduction system revealed by electrophysiological invasive examination was evaluated with regard to localization of conduction disturbance. A single focus was observed in 70 patients, multifocal affection was found in 57 cases (45%). This finding is in agreement with literary data and thus the use of the term "disease of cardiac conduction system" is recommended in all cases where multifocal disturbance of conduction system is diagnosed.

Arrhythmias, Cardiac↗