Natural history of cardiac involvement in myotonic dystrophy: correlation with CTG repeats.
The authors prospectively studied the natural course of cardiac involvement and its relationship to cytosine-thymine-guanine (CTG) expansion in 50 patients with myotonic dystrophy who were submitted to periodic cardiovascular EKG and EKG-Holter monitoring during a median follow-up of 56 months. Nineteen patients (38%) developed major EKG changes. CTG length was not correlated with the frequency of EKG abnormalities, but was inversely correlated with the age at onset of EKG abnormalities (p < 0.0001). CTG length influences the timing of cardiac complications in myotonic dystrophy.
Antonini
G
G
(Department of Neurological Sciences, University of Rome, La Sapienza, Italy. antoninimusc@uniroma1.it
Giubilei
F
F
Mammarella
A
A
Amicucci
P
P
Fiorelli
M
M
Gragnani
F
F
Morino
S
S
Ceschin
P V
PV
Fragola
P V
PV
Gennarelli
M
M
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000235:genetics* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000235:genetics*; D011446:Prospective Studies; D018911:Trinucleotide Repeats / Q000235:genetics*