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+/-12.3 months. Arrhythmias were recorded by the pacemaker in 17 patients (85%): 14 developed arrhythmic episodes that occurred either in absence or in presence of concomitant sleep apnoea. Conversely, among these 14 patients, the majority of sleep apnoea episodes were not associated with concomitant arrhythmias. In the other three patients who developed arrhythmias, simultaneous sleep apnoea was never observed. Episodes of sleep apnoea were observed in all patients, though only 85% presented with sleep apnoea syndrome. In conclusion, a high incidence of arrhythmias and sleep apnoea was observed. While arrhythmias are generally attributable to an organic substrate, they are sometimes precipitated by functional triggers, as in the case of sleep apnoea.

Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing: a multicenter diagnostic pacemaker study.

OBJECTIVES: We hypothesized that pacemaker (PM) implantation in patients with myotonic dystrophy (MD) with a prolonged HV interval, even asymptomatic, may protect them against sudden death related to atrioventricular (AV) block. We sought to prospectively document the true incidence of AV block episodes in this high-risk population and accurately trace, in the long term, by the PM, the occurrence of arrhythmias that may remain undetected during conventional follow-up. BACKGROUND: Myotonic dystrophy is associated with a high risk of sudden death, commonly attributed to AV block or ventricular arrhythmias, but cardiac pacing is only recommended as a secondary prevention. METHODS: Patients with MD with an HV interval > or =70 ms, even in the absence of related symptoms, prospectively received a cardiac PM, including an algorithm capable of diagnosing episodes of bradycardia and tachyarrhythmias. RESULTS: The population consisted of 49 patients (45.5 +/- 8.9 years old) followed for 53.5 +/- 27.2 months. Paroxysmal arrhythmias were recorded in 41 patients (83.7%), consisting of complete AV block (n = 21), sino-atrial block (n = 4), or atrial (n = 25) or ventricular (n = 13) tachyarrhythmias. No patient died of AV block during follow-up, but 10 deaths occurred, 4 of them sudden. An arrhythmic cause could be excluded by postmortem PM interrogation in two cases of typical sudden death. CONCLUSIONS: Arrhythmias are common in patients with MD with infrahisian conduction abnormalities. The prophylactic implantation of a pacing system when the HV interval is > or =70 ms seems appropriate. The PM protects the patient against the clinical consequences of paroxysmal profound bradycardia and facilitates the diagnosis and management of frequent paroxysmal tachyarrhythmias.

Right ventricular MR abnormalities in myotonic dystrophy and relationship with intracardiac electrophysiologic test findings: initial results.

PURPOSE: To prospectively determine whether a relationship exists between magnetic resonance (MR) imaging abnormalities of the right ventricle (RV) and intracardiac electrophysiologic (EP) test results in patients with myotonic dystrophy. MATERIALS AND METHODS: Conventional T1-weighted single-shot black-blood fast spin-echo and gradient-echo MR imaging of the heart was prospectively performed in 32 patients with myotonic dystrophy who required EP testing. Patients were divided into two groups according to EP test results: (a) inducible (n = 15), indicating inducible ventricular tachyarrhythmias, and (b) noninducible (n = 17). Morphologic and functional MR data were analyzed by two independent investigators. Nonparametric statistical methods and kappa statistics were used. RESULTS: No morphologic or functional abnormalities of the RV wall were observed in noninducible patients. Increased signal intensity of the RV wall, indicative of fatty replacement, was identified in 13 inducible patients. Myocardial thinning of the RV was observed in six inducible patients. An overlap of morphologically abnormal areas and areas of hypo- or dyskinesis were present in 11 inducible patients. RV outflow tract diameter was larger and RV ejection fraction was smaller in inducible patients than in noninducible patients, although differences were not significant. Interobserver agreement for MR findings was good (increased signal intensity: kappa = 0.87, P >.30 [pairwise Wilcoxon signed rank test]; myocardial thinning: kappa = 0.87, P >.30; hypo- or dyskinesis: kappa = 1.00, P >.99). There was a strong relationship between MR abnormalities and inducibility during EP testing (increased signal intensity, P <.001; myocardial thinning, P <.01; hypo- or dyskinesis, P <.01). CONCLUSION: The relationship between MR morphologic and functional RV abnormalities and EP testing suggests potential for the use of MR imaging as a noninvasive method to estimate the individual risk of arrhythmia in patients with myotonic dystrophy.