Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies.

Background: An accurate estimation of the risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with LMNA mutations is crucial to select candidates for implantable cardioverter-defibrillator implantation.

Methods: We included 839 adult patients with LMNA mutations, including 660 from a French nationwide registry in the development sample, and 179 from other countries, referred to 5 tertiary centers for cardiomyopathies, in the validation sample. LTVTA was defined as (1) sudden cardiac death or (2) implantable cardioverter defibrillator-treated or hemodynamically unstable VTA.

Development and Validation of a New Scoring System to Predict Survival in Patients With Myotonic Dystrophy Type 1.

Importance: Life expectancy is greatly shortened in patients presenting with myotonic dystrophy type 1 (DM1), the most common neuromuscular disease. A reliable prediction of survival in patients with DM1 is critically important to plan personalized health supervision.

Objective: To develop and validate a prognostic score to predict 10-year survival in patients with DM1.

Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1.

Aims: To describe the incidence and identify predictors of sudden death (SD), major conduction defects and sustained ventricular tachyarrhythmias (VTA) in myotonic dystrophy type 1 (DM1).

Methods And Results: We retrospectively enrolled 1388 adults with DM1 referred to six French medical centres between January 2000 and October 2013. We confirmed their vital status, classified all deaths, and determined the incidence of major conduction defects requiring permanent pacing and sustained VTA.

Responders to cardiac resynchronization therapy with narrow or intermediate QRS complexes identified by simple echocardiographic indices of dyssynchrony: the DESIRE study.

Background: Cardiac resynchronization therapy (CRT) is recommended for patients with NYHA class III-IV refractory heart failure (HF), ejection fraction <35% and a QRS >120 ms. We attempted to identify responders to CRT from echocardiographic (echo) indices of mechanical dyssynchrony in patients with QRS<150 ms.

Methods And Results: The study enrolled 51 men and 9 women (mean age: 64.

Relationship between cardiac arrhythmias and sleep apnoea in permanently paced patients with type I myotonic dystrophy.

The long-term relationship between cardiac arrhythmias and sleep apnoea in myotonic dystrophy (DM1) is unknown. Pacemakers enabling the long-term monitoring of electrocardiographic and ventilation parameters were implanted in 20 patients with DM1 (mean age = 42+/-11.6 years), followed 40+/-12.

Remote, wireless, ambulatory monitoring of implantable pacemakers, cardioverter defibrillators, and cardiac resynchronization therapy systems: analysis of a worldwide database.

Study Objective: To describe the daily routine application of a new telemonitoring system in a large population of cardiac device recipients.

Methods: Data transmitted daily and automatically by a remote, wireless Home Monitoring system (HM) were analyzed. The average time gained in the detection of events using HM versus standard practice and the impact of HM on physician workload were examined.