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

Hideyuki MURAOKA, Nobuyuki NEGORO, Fumio TERASAKI, Takahiro NAKAKOJI, Shigeyuki KOJIMA, Masaaki HOSHIGA, Masakazu SUGINO, Takafumi HOSOKAWA, Tadashi ISHIHARA, Toshiaki HANAFUSA
2005 Internal medicine (Tokyo. 1992)  
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
more » ... e fatty infiltration and interstitial fibrosis. Radiofrequency catheter ablation at the biopsy site could eliminate VT, so it was strongly suggested that the reentry circuit was formed by focal fatty-fibrosis. Careful observation should be continued for a long period. (Internal Medicine 44: 129-135, 2005)
doi:10.2169/internalmedicine.44.129 pmid:15750273 fatcat:nmkyvqwss5hwrkozblrc4q5bqu