
Myotonic dystrophy associated with qt prolongation and torsade de pointes
Author(s) -
Umeda Yuji,
Ikeda Uichi,
Yamamoto Jun,
Fukazawa Hiroshi,
Hayashi Yuji,
Fujikawa Hideyuki,
Shimada Kazuyuki
Publication year - 1999
Publication title -
clinical cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.263
H-Index - 72
eISSN - 1932-8737
pISSN - 0160-9289
DOI - 10.1002/clc.4960220219
Subject(s) - medicine , myotonic dystrophy , qt interval , long qt syndrome , torsades de pointes , cardiology , heart failure , heart block , electrocardiography , prolongation
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.