[ XXXX : surgical treatment].

@entity1 @entity3585 @entity132 @entity943 @entity1184 @entity944 @entity1163 @entity591 @entity1141 @entity4346 @entity74 @entity158 @entity432 @entity1121 @entity1002
@entity1121 ( @entity1121 ) is an autosomal, @entity74 which leads slowly to increasing subvalvular, septal and left @entity4346 . Difficult molecular-genetic investigations localized genetic defects on different chromosomes. The disease is pathological-anatomically characterized by asymmetric subvalvular (aortic) @entity1184 and @entity3585 resulting in additional @entity1002 . Histologically the myocytes are hypertrophied, exhibit atypical branching (disarray), and there is a high amount of interstitial connective tissue. In our biopsy material (from myectomies) @entity1141 could be detected in more than 30% of @entity432 with partly extential media. These changes may indicate @entity132 resulting in @entity943 , @entity1163 , @entity944 , and @entity158 on the basis of @entity132 and scar development. Today the discussion of DDD-pacemaker therapy has resumed, but one must wait for definite results, especially in @entity1 in whom surgical treatment seems to be the best choice. The indication for surgical treatment, which usually is transaortic subvalvular myectomy (Morrow) and modifications, is very restrictive. Only @entity1 in clinical degree III (NYHA) after long-term medical treatment are candidates for surgery. In some mainly younger @entity1 the indication in lower clinical degrees was accepted because of a family history with @entity944 and personal experience of @entity1163 , life-threatening @entity591 , or after resuscitation. In the period 1963 to 1994 466 @entity1 were operated upon. The mean age was 44.9 years (range 3 months to 82 years). Total early mortality was 4.9% (n = 12).(ABSTRACT TRUNCATED AT 250 WORDS)
