Myotonic dystrophy associated with QT prolongation and torsade de pointes

Clin Cardiol. 1999 Feb;22(2):136-8. doi: 10.1002/clc.4960220219.


A rare case of myotonic dystrophy (MD) with congestive heart failure, associated with QT prolongation and torsade de pointes (TdP) is reported. A 53-year-old woman was admitted to the hospital because of congestive heart failure. Electrocardiograph (ECG) showed first-degree atrioventricular block and QT prolongation. During hospitalization, TdP appeared but returned to sinus rhythm spontaneously. As the patient had quadriplegia, a myopathic face, cataracts, diabetes mellitus, and an increased number of cytosine-thymineguanine (CTG) repeats (760 repeats), she was diagnosed as having MD. Electrocardiographic analysis of her family also revealed abnormal QT(U) prolongation in her daughter and brother who both had MD, while ECG findings of other family members without MD were normal. Thus, the presence of QT(U) prolongation was associated with MD in this family.

Publication types

  • Case Reports

MeSH terms

  • Cardiotonic Agents / therapeutic use
  • Diuretics / therapeutic use
  • Dopamine / therapeutic use
  • Drug Therapy, Combination
  • Echocardiography
  • Electrocardiography*
  • Female
  • Follow-Up Studies
  • Heart Failure / drug therapy
  • Heart Failure / etiology*
  • Heart Failure / physiopathology
  • Humans
  • Middle Aged
  • Myotonic Dystrophy / complications*
  • Myotonic Dystrophy / diagnosis
  • Pedigree
  • Radiography, Thoracic
  • Torsades de Pointes / complications*
  • Torsades de Pointes / diagnosis
  • Torsades de Pointes / physiopathology


  • Cardiotonic Agents
  • Diuretics
  • Dopamine