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BackgroundAtrial flutter-related tachycardiomyopathy (AFL-TCM) is a rare and treatable cause of heart failure. Little is known about its epidemiology and long-term prognosis. Our aims are to determine the prevalence, predictors and outcomes of AFL-TCM. Methods and resultsA total of 1269 patients were referred for radiofrequency ablation of AFL between January 1996 and September 2014; 184 had reduced left ventricular ejection fraction (LVEF <40%). At 6 months after AFL ablation, 103 patients (8.1% of the population, 56% of patients with baseline LVEF <40%) had marked LVEF improvement: these were considered to have AFL-TCM. Patients with persisting reduced LVEF were considered to have systolic dysfunction unrelated to AFL. Patients were followed for a median (percentile 25-75 ) of 1.15 (0.4-2.8) years. Patients with AFL-TCM were younger, had lower prevalence of ischaemic cardiomyopathy and used less antiarrhythmic drugs than patients with systolic dysfunction unrelated to AFL. In multivariable analysis, ischemic cardiomyopathy [odds ratio (OR) = 0.32, 95% confidence interval (CI) 0.15-0.68) P = 0.003] and prescription of antiarrhythmic drug before ablation [OR = 0.41, 95% CI 0.20-0.84, P = 0.02] were significantly associated with a lower probability of LVEF improvement during follow-up. Patients with AFL-TCM had similar survival to patients without systolic dysfunction at baseline [hazard ratio (HR) = 0.96 95% CI 0.34-2.65, P = 0.929], whereas patients with systolic dysfunction unrelated to AFL had higher mortality rates compared with patients without systolic dysfunction at baseline [HR = 2.88, 95% CI 1.45-5.72, P = 0.002].

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AF occurrence after AFL ablation is frequent (>20%), especially in patients with a history of AF, in female patients, and in patients treated with class I antiarrythmics/amiodarone prior to AFL. Since most patients who experience AF after AFL ablation have a CHA2DS2-VASc ≥1, the decision to stop anticoagulants after ablation should be considered on an individual basis.

Various causes could explain syncope in 70% of patients with coronary disease and DCM, but differences were noted: VT was frequent in coronary disease with a bad prognosis, and ischemia could explain syncope; in DCM, different causes such as atrial tachycardia could be responsible for syncope, but the prognosis only depended on LVEF.

Transesophageal EPS was required to determine the prognosis of asymptomatic WPW in children. The maximal rate conducted in AP was higher in children younger than 16 years old than in teenagers; other data did not differ. AVRT was rare; 71% of children had no inducible arrhythmia and were authorized to resume physical activities.

Myotonic dystrophy type 1 (DM1) is a multisystemic disorder characterized by muscle weakness and multiple organ impairment, especially the eyes, lung, and heart. We conducted the current study to analyze the prevalence and intercorrelation among these disorders and their respective relationships with muscular disability. We assessed medical history, anthropometric data, lung volumes, arterial and venous blood samples, surface 12-lead electrocardiogram, echocardiography, ophthalmologic examination, and muscular impairment rating scale (MIRS) in 106 patients (48 male and 58 female) with DM1, aged 43.7 ± 12.8 years. Obesity, hypertriglyceridemia, and diabetes were found in respectively 25.6%, 47.6%, and 17.1% of patients. Disabling cataract was found in 43.4%, and was independently predicted by age and MIRS. Restrictive lung disease was noted in 34%, and was predicted by MIRS, CTG repeat expansion, and body mass index. Conduction disorders were found in 30.2% of patients and were predicted by left ventricular ejection fraction, MIRS, and CTG repeat expansion.We found significant relationships between cataract, restrictive lung disease, and conduction disorders: patients with cataract and those with conduction disorders exhibited more severe restrictive lung disease than the other patients. Conversely, the relative risk of restrictive lung disease was 2.42 (1% confidence interval [CI], 1.06-5.51) in patients with cataract and 2.54 (1% CI, 1.26-5.07) in patients with conduction disorders. Multivariate analysis revealed that MIRS was the only independent predictor for conduction disorders and restrictive lung disease. MIRS ≥3 and MIRS ≥4 were the best simple cutoff values to predict, respectively, lung and cardiac involvements.To conclude, muscular disability, ophthalmologic, and cardiac and pulmonary involvement are strongly correlated. Particular attention should be given to these entities in patients with distal or proximal muscular weakness.

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