Unmasked Brugada pattern by ajmaline challenge in patients with myotonic dystrophy type 1.

BACKGROUND: Myotonic dystrophy type 1 (DM1) generates missplicing of the SCN5A gene, encoding the cardiac sodium channel (Nav 1.5). Brugada syndrome, which partly results from Nav 1.5 dysfunction and causes increased VF occurrence, can be unmasked by ajmaline. We aimed to investigate the response to ajmaline challenge in DM1 patients and its potential impact on their sudden cardiac death risk stratification. METHODS: Among 36 adult DM1 patients referred to our institution, electrophysiological study and ajmaline challenge were performed in 12 patients fulfilling the following criteria: (1) PR interval >200 ms or QRS duration >100 ms; (2) absence of complete left bundle branch block; (3) absence of permanent ventricular pacing; (4) absence of implantable cardioverter-defibrillator (ICD); (5) preserved left-ventricular ejection fraction >50%; and (6) absence of severe muscular impairment. Of note, DM1 patients with ajmaline-induced Brugada pattern (BrP) were screened for SCN5A. RESULTS: In all the 12 patients studied, the HV interval was <70 ms. A BrP was unmasked in three patients but none carried an SCN5A mutation. Ajmaline-induced sustained ventricular tachycardia occurred in one patient with BrP, who finally received an ICD. The other patients did not present any cardiac event during the entire follow-up (15 ± 4 months). CONCLUSION: Our study is the first to describe a high prevalence of ajmaline-induced BrP in DM1 patients. The indications, the safety, and the implications of ajmaline challenge in this particular setting need to be determined by larger prospective studies.

Optimizing pacemaker longevity with pacing mode and settings programming: results from a pacemaker multicenter registry.

BACKGROUND: This study aimed to describe the influence on dual-chamber devices' expected longevity of devices' settings. METHODS: Data from patients implanted with dual chamber devices (Symphony™, SORIN CRM SAS, Clamart, France) from 2003 to 2006 were collected in registries. Programmer files were retrieved: device-estimated longevity, assessed through algorithm prediction, was analyzed according to device settings. RESULTS: One thousand sixty-eight recipients of dual chamber pacemaker in sinus rhythm (75.3±11.1 years, 54.5% male, ventricular block 30%, brady-tachy syndrome 21%, and sinus node dysfunction 49%) were followed up to 14.2±12.1 months (ranging from first quartile Q1: 2.9 months to fourth quartile Q4: 49.3 months) after implantation. DDD with automatic mode conversion and minimized ventricular pacing (SafeR) modes were programmed in 34.3%, 2.9%, and 62.8% of the patients, respectively. The mean total longevity estimated by the device was 134.1±31.5 months (11.2±2.6 years). Significant increase in longevity was observed in devices undergoing at least one reprogramming (134.4±31.4 months) versus device presenting no reprogramming (103.4±32.3 months, P=0.0005). The parameters associated with the major increase in mean longevity were the mode (mean longevity increase of +23.9 months in SafeR as compared to DDD mode, P<0.0001) and the atrial (A) and ventricular (V) amplitudes (mean longevity increase of +29.6 and +26.9 months for a decrease of less than 1V in A and V outputs respectively, P<0.0001). CONCLUSION: This study provides information on dual chamber pacemakers' longevity and highlights the impact of devices' reprogramming on expected longevities.

Obsessive compulsive disorder in a patient with twiddler's syndrome.

Twiddler's or twist syndrome is the twisting of pulse generators around themselves. It may result from mechanical manipulation that can induce the malfunction of the device. In this case, twiddler's syndrome resulted from compulsive checking of the device. The implantable cardioverter-defibrillator (ICD) triggered the development of an obsessive compulsive disorder (OCD). Two invasive procedures were required to replace the ICD. Psychiatric intervention prevented the recurrence of twiddler's syndrome in this patient for more than 2 years. We believe that preimplant psychiatric assessment should be the rule.

Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study.

BACKGROUND: Brugada syndrome is an arrhythmogenic disease characterized by an increased risk of sudden cardiac death (SCD) by ventricular fibrillation. At present, an implantable cardioverter-defibrillator (ICD) is the recommended therapy in high-risk patients. This multicenter study reports the outcome of a large series of patients implanted with an ICD for Brugada syndrome. METHODS AND RESULTS: All patients (n=220, 46+/-12 years, 183 male) with a type 1 Brugada ECG pattern implanted with an ICD in 14 centers between 1993 and 2005 were investigated. ICD indication was based on resuscitated SCD (18 patients, 8%), syncope (88 patients, 40%), or positive electrophysiological study in asymptomatic patients (99 patients, 45%). The remaining 15 patients received an ICD because of a family history of SCD or nonsustained ventricular arrhythmia. During a mean follow-up of 38+/-27 months, no patient died and 18 patients (8%) had appropriate device therapy (10+/-15 shocks/patient, 26+/-33 months after implantation). The complication rate was 28%, including inappropriate shocks, which occurred in 45 patients (20%, 4+/-3 shocks/patient, 21+/-20 months after implantation). The reasons for inappropriate therapy were lead failure (19 patients), T-wave oversensing (10 patients), sinus tachycardia (10 patients), and supraventricular tachycardia (9 patients). Among implantation parameters, high defibrillation threshold, high pacing threshold, and low R-wave amplitude occurred, respectively, in 12%, 27%, and 15% of cases. CONCLUSIONS: In this large Brugada syndrome population, a low incidence of arrhythmic events was found, with an annual event rate of 2.6% during a follow-up of >3 years, in addition to a significant risk of device-related complications (8.9%/year). Inappropriate shocks were 2.5 times more frequent than appropriate ones.