Aortic valve replacement in a XXXX with ostegenesis imperfecta A case report.

@entity1 @entity2680 @entity174 @entity384 @entity3454 @entity2100 @entity337 @entity74 @entity6926
AIM: @entity6926 ( @entity6926 ) is an @entity74 in which fragile bones readily cause @entity174 . @entity384 , @entity337 and @entity3454 are uncommon cardiovascular manifestations of @entity6926 . Cardiac surgery in these @entity1 carries a high risk of complications due to increased tissue and @entity2100 . We describe an open heart surgery in a @entity1 with isolated @entity337 secondary to @entity6926 . MATERIAL OF STUDY: A 58-year-old @entity1 was referred to our hospital for surgical correction of @entity337 . She had a past history of recurrent @entity174 , and @entity6926 was diagnosed in the childhood. A standard median sternotomy was performed; the sternum was found to be thin and brittle. The native aortic valve was replaced with a size 23 mm stented aortic bioprosthesis. The sternum was closed with stainless steel wires. RESULTS: The postoperative course was uneventful, and the @entity1 was discharged home on the eighth postoperative day. We used thoracic band to avoid sternal diastasis. One year postoperatively, the echocardiogram showed a normal aortic bioprosthesis function without @entity2680 . The sternum was stable without dehiscence. DISCUSSION: The mortality rate in cardiac surgery @entity1 with heritable generalized connective tissue disorders, such as @entity6926 , is high. Although tissue friability had no impact on surgical outcome, it should be kept in mind when operating on @entity1 with @entity6926 . CONCLUSIONS: We highlight the importance of a meticulous surgical technique, together with a strategy for management of anticipated perioperative complications to ensure a successful outcome. KEY WORDS: Aortic valve, Endocardirtis, Mitral valve, Replacement.
