Atrial Flutter or Fibrillation is the Most Frequent and Life‐Threatening Arrhythmia in Myotonic Dystrophy

Abstract

AF and AFL were frequent in MD and increased mortality. AFL could present as 1/1 AFL with a poor tolerance and a risk of misdiagnosis despite frequent conduction disturbances. This arrhythmia could explain wide QRS tachycardia occurring in MD and interpreted as VT.

Lack of correlation between the ventilatory response to CO2 and lung function impairment in myotonic dystrophy patients: Evidence for a dysregulation at central level

“…Lung function impairment (respiratory muscle and diaphragmatic weakness, ventilatory restriction, hypoxaemia and hypercapnia) [5] and cardiac dysfunctions [6,7] have been well described and their prognostic value has long been C (%) FVC demonstrated, but the major issues affecting quality of life in DM1 patients are the CNS and cognitive dysfunctions [8,9].…”

“…Besides the classical view as a neuromuscular disease (the majority of DM1 research focused on neuromuscular aspects), nowadays DM1 has to be also considered as a brain disorder regarding the central nervous system (CNS) and cognitive dysfunctions. Indeed, if lung function impairment [5] or cardiac conduction diseases [6,7] have been well described and have a clear prognostic value, the major issues affecting quality of life in DM1 patients are the CNS and cognitive dysfunctions [8,9]. The control of breathing has been shown to be affected, with the description of numerous clinical findings such as irregular breathing pattern [10], sleep breathing disorders [11] or excessive daytime sleepiness [12], supporting a dysregulation at the central level.…”

Syncope and the risk of sudden cardiac death: Evaluation, management, and prevention

“…Atrial arrhythmias are also common in DM1, can cause syncope, and are a marker of worse prognosis. In a study of 161 patients with DM1, 27 (17%) had either atrial fibrillation or flutter (AFL), with two presenting with syncope‐related AFL with 1:1 AV nodal conduction [67]. These atrial arrhythmias were associated with an increase in mortality (30% died with AF vs 10% without, p <0.01).…”

The Role of the Atrial Electromechanical Delay in Predicting Atrial Fibrillation in Myotonic Dystrophy Type 1 Patients

“…The cardiac involvement is noticed in about 80% of cases, and it often precedes the skeletal muscle one. Paroxysmal atrial arrhythmias (atrial fibrillation, atrial flutter, atrial tachycardia) frequently occur in DM1 patients; these tachyarrhytmias may be the first clinical manifestation of a muscular dystrophy syndrome in young patients and seem to increase mortality in this population …”