Wahbi Karim, Sebag Frederic A, Lellouche Nicolas, Lazarus Arnaud, Bécane Henri-Marc, Bassez Guillaume, Stojkovic Tanya, Fayssoil Abdallah, Laforêt Pascal, Béhin Anthony, Meune Christophe, Eymard Bruno, Duboc Denis
AP-HP, Pitié-Salpêtrière Hospital, Myology Institute, Paris, France; AP-HP, Cochin Hospital, Department of Cardiology, Paris Descartes University, Paris, France; Pierre and Marie Curie University, Paris, France.
AP-HP, Henri Mondor Hospital, Department of Cardiology, INSERM U 955, Paris Est Creteil University, Creteil, France.
Neuromuscul Disord. 2016 Mar;26(3):227-33. doi: 10.1016/j.nmd.2016.01.005. Epub 2016 Feb 10.
The prevalence and the incidence of atrial flutter in patients with myotonic dystrophy type 1 (DM1) and the most appropriate strategies for its management are unknown. We retrospectively included in the DM1 Heart Registry 929 adult patients with DM1 admitted to our Institutions between January 2000 and September 2013. We selected patients presenting with atrial flutter and analysed data relative to the occurrence of arterial thromboembolism, severe bradyarrhythmias and atrial flutter recurrences. Atrial flutter was present in 79 of the 929 patients included in our Registry, representing a 8.5% prevalence. Patients with atrial flutter were older, had a higher muscular disability rating scale score and had higher prevalence of other cardiac manifestations of DM1. Sixty patients presented with a first episode of atrial flutter, representing a 4.6% incidence. Severe bradyarrhythmias requiring permanent pacing were present in 4 patients (6.7%). Over a 53 ± 28 months mean follow-up duration, 2 patients (3.3%) had ischaemic stroke and 12 (20%) had atrial flutter recurrences. Patients who underwent radiofrequency ablation were more frequently free of atrial flutter recurrence than other patients (95 vs. 61%; HR = 0.17; P = 0.04). Atrial flutter is a common manifestation of DM1, potentially complicated by arterial thromboembolism or severe bradyarrhythmias. Radiofrequency catheter ablation is associated with a lower risk for recurrences.
1型强直性肌营养不良（DM1）患者心房扑动的患病率和发病率以及最恰当的管理策略尚不清楚。我们回顾性纳入了DM1心脏登记处的929例成年DM1患者，这些患者于2000年1月至2013年9月间入住我们的机构。我们选择了出现心房扑动的患者，并分析了与动脉血栓栓塞、严重缓慢性心律失常及心房扑动复发相关的数据。在我们登记的929例患者中，有79例存在心房扑动，患病率为8.5%。有心房扑动的患者年龄更大，肌肉残疾评定量表评分更高，且DM1其他心脏表现的患病率更高。60例患者首次出现心房扑动，发病率为4.6%。4例患者（6.7%）存在需要永久起搏的严重缓慢性心律失常。在平均53±28个月的随访期内，2例患者（3.3%）发生缺血性卒中，12例患者（20%）出现心房扑动复发。接受射频消融的患者比其他患者更常无心房扑动复发（95%对61%；HR=0.17；P=0.04）。心房扑动是DM1的常见表现，可能并发动脉血栓栓塞或严重缓慢性心律失常。射频导管消融与较低的复发风险相关。
