[Cardiac involvement in Steinert myotonic dystrophy]. / Les anomalies cardiaques au cours de la dystrophie myotonique de Steinert.

INTRODUCTION: Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystemic disease with frequent cardiac involvement that may cause sudden death. This study was performed to determine the various cardiac manifestations in DM1, their frequency and the relevance of cardiac electrophysiological study in this disease. METHODS: Ten patients with DM1, five men and five women, mean age 44.3+/-7.8 years underwent neurological and cardiac assessments. RESULTS: The most frequent electrocardiographic findings were conduction abnormalities, essentially by intraventricular conduction defects (eight out of ten cases) such as bundle branch or fascicular blocks. Echocardiography showed alterations in systolic left ventricular function in two cases. Invasive electrophysiology testing showed sub-hisien block in three patients, requiring cardiac pacemaker implantation. These three patients had normal duration of PR interval and normal width of QRS complex. CONCLUSIONS: We recommend that all patients with DM1 should undergo cardiac investigation to detect subclinical cardiac involvement.

[Percutaneous implantation under local anesthesia of an implantable automatic defibrillator: report of the first Tunisian case]. / Implantation percutanée sous anesthesie locale d'un defibrillateur automatique implantable: a propos du premier cas Tunisien.

We report the case of a 58 years old male affected by a severe relapsing ventricular tachycardia despite a well administered anti arrhythmic therapy. Etiology is an arrhythmogene right ventricular dysplasia. Considering the severity of this arrhythmia we proceed to percutaneous implantation of an automatically implantable defibrillator under local anesthesia. Evolution was favorable with a follow-up of 3 months.

Ventricular tachycardia catheter ablation in arrhythmogenic right ventricular dysplasia: a 16-year experience.

Arrhythmogenic right ventricular dysplasia (ARVD) is a structural heart disease affecting young adults that leads to cardiac rhythm disorders including supraventricular and mostly ventricular arrhythmias. Sudden death may be the first presentation of the disease. Ablation techniques have been used for the treatment of ventricular tachycardia in cases resistant to drug therapy. Radiofrequency is appropriate as a first approach for ventricular tachycardia ablation in ARVD; however, its effectiveness is less than 40% at the first session. Fulguration is effective for ventricular tachy-cardia ablation and should be used in the same session after ineffective radiofrequency ablation. However, fulguration requires expertise, general anesthesia, and more than one session in half of all patients. Radiofrequency and fulguration plus other common forms of treatment including pacemakers and automatic implantable cardioverter defibrillators provides a clinical success rate of 81% to 93% in a series of 50 consecutive patients studied during 16 years. Earlier poor reputation of fulguration was the result of poorly understood technical problems concerning the physics and biophysics of the procedure under control with presently available methods. This in-depth study of a large population over a long time period demonstrates that fulguration should be rehabilitated.