Cardiac Manifestations in Myotonic Dystrophy Type 1 Patients Followed Using a Standard Protocol in a Specialized Unit

Long‐Term Arrhythmia Follow‐Up of Patients with Myotonic Dystrophy

“…6,7,9,10 Reported predictors of sudden death in this population include atrial tachyarrhythmias, a PR interval greater than 240 milliseconds, aberrant QRS conduction and any degree of AV block. 10 Although patients with cardiacconduction abnormalities in the setting of MD are observed to have ventricular tachyarrhythmias, 1,6,7,9,[11][12][13][14][15][16][17][18][19][20][21][22][23][24] our knowledge of the incidence of ventricular tachyarhythmia events in this unique population is limited.…”

An overview of heart rhythm disorders and management in myotonic dystrophy type 1

“…Other mechanisms, such as increased automaticity and re-entry not involving the His bundle with positive results of VT ablation have been described in isolated case reports [69,[71][72][73]. Inducibility of VT has been exhibited in myotonic dystrophy and although the prognostic value of such a finding in predicting sudden death has not been established, induction of a monomorphic sustained VT on EPS may constitute a favourable substrate for VT ablation [41,74].…”

2020 Clinical practice guidelines for Bradyarrhythmias and conduction disorders

“…• Пациентам с нервно-мышечными заболеваниями, связанными с нарушениями проводимости, включая мышечную дистрофию (например, тип миотонической дистрофии), у которых есть признаки ПЖБ ΙΙ и ΙΙΙ степени или HV-интервал 70 мс или более, независимо от симптомов постоянная стимуляция рекомендуется [182][183][184][185][186][187][188][189][190][191][192].…”

“…• Пациентам с постоянной ФП и симптомной брадикардией рекомендуется постоянная стимуляция [180][181][182][183][184][185][186][187][188][189][190][191][192].…”