Management

In general, cardiovascular disease is the major cause of morbidity and mortality in aortopathy syndromes, such as LDS. While IPO8syndrome closely resembles LDS, which typically presents with aortic dissections at relatively small aortic diameters, and IPO8patients often present with large aneurysms already early in life, arterial dissections/ruptures have not yet been reported. Given the fact that this condition has only recently been identified and very few patients above the age of 25 have been described, the current paucity of dissection reports in IPO8patients might inflict underestimation of the true dissection/rupture risk. In light of the striking resemblance with LDS, both from a clinical and pathomechanistic point of view, it is proposed to adhere to the same management recommendations (for both the arterial and non-vascular manifestations) as for LDS (Loeys-Dietz Syndrome - GeneReviews® - NCBI Bookshelf).