Re-entry Circuit in Ventricular Tachycardia Due to Focal Fatty-fibrosis in a Patient with Myotonic Dystrophy

A 69-year-old man with a recurrent ventricular tachycardia (VT) was admitted. The patient was diagnosed as myotonic dystrophy type 1 (DM1) and DNA analysis revealed 1,800 CTG-repeat expansion in the myotonic dystrophy protein kinase (DMPK) gene. Ultrasonic cardiogram (UCG), left ventriculogram (LVG)...

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Published inInternal Medicine Vol. 44; no. 2; pp. 129 - 135
Main Authors MURAOKA, Hideyuki, NEGORO, Nobuyuki, TERASAKI, Fumio, NAKAKOJI, Takahiro, KOJIMA, Shigeyuki, HOSHIGA, Masaaki, SUGINO, Masakazu, HOSOKAWA, Takafumi, ISHIHARA, Tadashi, HANAFUSA, Toshiaki
Format Journal Article
LanguageEnglish
Published Japan The Japanese Society of Internal Medicine 01.02.2005
Subjects
Adipose Tissue - pathology
Aged
Biopsy
Cardiomyopathies - complications
Cardiomyopathies - diagnosis
Catheter Ablation
Diagnosis, Differential
DNA - analysis
Electrocardiography
endomyocardial biopsy
fatty-fibrosis
Fibrosis
Heart Rate - physiology
Heart Ventricles - diagnostic imaging
Heart Ventricles - pathology
Humans
Magnetic Resonance Imaging
Male
myotonic dystrophy
Myotonic Dystrophy - complications
Myotonic Dystrophy - diagnosis
Myotonic Dystrophy - enzymology
Myotonin-Protein Kinase
Protein-Serine-Threonine Kinases - genetics
Radionuclide Ventriculography
Tachycardia, Ventricular - etiology
Tachycardia, Ventricular - physiopathology
Tachycardia, Ventricular - surgery
Ultrasonography
ventricular tachycardia
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Summary:A 69-year-old man with a recurrent ventricular tachycardia (VT) was admitted. The patient was diagnosed as myotonic dystrophy type 1 (DM1) and DNA analysis revealed 1,800 CTG-repeat expansion in the myotonic dystrophy protein kinase (DMPK) gene. Ultrasonic cardiogram (UCG), left ventriculogram (LVG) and magnetic resonance imaging (MRI) did not show any abnormal sign including fatty infiltration. But, endomyocardial biopsy obtained from ventricular outflow tract revealed severe fatty infiltration and interstitial fibrosis. Radiofrequency catheter ablation at the biopsy site could eliminate VT, so it was strongly suggested that the re-entry circuit was formed by focal fatty-fibrosis. Careful observation should be continued for a long period.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
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ObjectType-Report-1
ObjectType-Article-3
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.44.129