*7.2. Ethical Aspects*

Actually, end-of-life management preferences in neuromuscular diseases, including DMD, are a challenging area. Ethical concerns remain open about which patient should be a candidate or excluded. Additionally, The Working Group acknowledged the value of a long-term patient/family/physician relationship before the urgent need for device placement [72]. A multidisciplinary approach with careful evaluation of frailty and co-morbidities is crucial to assess the proper selection of DMD patients. A shared decision process is necessary to obtain a collaborative contact with patient, parents, and caregivers, making this strategy successful [95,96].

## **8. Arrhythmias in DMD**

Arrhythmias occur frequently in cardiomyopathies. They may be also isolated manifestations, mostly in myotonic dystrophies and muscle channelopathies [97]. Potentially fatal arrhythmias are terminal events, and require the implantation of incatracardiac defibrillator (ICD). ECG may show right axis deviation, deep and narrow Q waves in inferolateral leads, conduction defects, sinus tachycardia, short PR intervals, and tall R wave in the right precordial leads, right bundle branch block and flat and inverted T waves [98]. In a large multicenter French study, left bundle branch block was present in 13% of patients; 2/3 of them disclosed exonic deletions. Left bundle branch block was significantly associated with cardiac events and mortality [99]. The incidence of supraventricular (6%) and ventricular arrhythmia (VT, 2%) was low in that study, in line with previous data that reported atrial flutter in 5%, sinus pause 5% [100], VT in 7% [101]. Others described that the QRS duration tended to increase progressively with age, irrespective of LV systolic function in patients with DMD [102].
