[Heart involvement in progressive external XXXX ( @entity12295 ): electrophysiologic, hemodynamic and morphologic findings].

@entity1 @entity296 @entity970 @entity8655 @entity714 @entity3055 @entity713 @entity12295 @entity4610 @entity528 @entity616 @entity2383
The @entity12295 is combined with a progressive external @entity4610 ( @entity970 ), retinal @entity528 and @entity616 . In some @entity1 , progression of incomplete forms has been described and potentially fatal conduction disturbances may occur. The disease is considered as a systemic @entity2383 . As part of an ongoing prospective study 6 @entity1 (3 female, 3 male; age 32 +/- 9 years) with @entity970 and typical ultrastructural changes of a @entity3055 in their skeletal muscle were examined. The ECG disclosed atrio/intraventricular conduction defects in 5 @entity1 : 1 @entity1 had a @entity713 which was treated by a pacemaker. Another @entity1 had @entity714 with complete @entity714 . In 3 other @entity1 an incomplete @entity714 was registered. In 1 @entity1 , His-bundle electrography disclosed a block distal to His by atrial high rate pacing before and after i.v. injection of @entity8655 ; prophylactically a pacemaker was implanted. The mean HV-interval increased significantly under @entity8655 by 44% in @entity1 with sinus rhythm. Hemodynamic studies were normal in 5 @entity1 at rest; only 1 @entity1 was abnormal during exercise. Myocardial biopsy demonstrated @entity2383 such as variability in shape and size. In 4 @entity1 concentric cristae mitochondriales were seen. Our results suggest that @entity713 are common in @entity1 with @entity970 . By means of endomyocardial catheterbiopsy a @entity296 could be detected.
