Critical appraisal of the revised Ghent criteria for diagnosis of XXXX .

@entity1 @entity2597 @entity4122 @entity384 @entity1614 @entity3454 @entity854 @entity20377 @entity1714
@entity1614 ( @entity1614 ) is a @entity2597 with major features in @entity854 . Recently, diagnostic criteria were revised where more weight was given to the @entity384 . We applied the revised Marfan nosology in an established adult Marfan population to define practical repercussions of novel criteria for clinical practice and individual @entity1 . Out of 180 @entity1614 @entity1 , in 91% (n = 164) the diagnosis of @entity1614 remained. Out of 16 @entity1 with rejected diagnosis, four @entity1 were diagnosed as MASS ( @entity1714 , @entity3454 , borderline non-progressive @entity384 , skeletal findings and striae) phenotype, three as @entity20377 and in nine @entity1 no alternative diagnosis was established. In 13 @entity1 , the diagnosis was rejected because the Z-score of the aortic root was <2, although the aortic diameter was larger than 40 mm in six of them. In three other @entity1 , the diagnosis of @entity1614 was rejected because dural @entity4122 was given less weight in the revised nosology. Following the revised Marfan nosology, the diagnosis of @entity1614 was rejected in 9% of @entity1 , mostly because of the absence of @entity384 defined as Z-score >= 2. Currently used Z-scores seem to underestimate @entity384 , especially in @entity1 with large body surface area (BSA). We recommend re-evaluation of criteria for aortic root involvement in adult @entity1 with a suspected diagnosis of @entity1614 .
