Correspondence (letter to the editor): New Insights

We think that some aspects of the article should be rectified, and that some additions are required.

Vascular Ehlers-Danlos syndrome (EDS) is not the most common form of EDS since 80–90% of all cases of EDS are “classic” EDS. The categorization of EDS cited by the authors, into 11 forms has been obsolete for 10 years. The authors do not mention Loeys-Dietz syndrome in their review article, which has a rather more aggressive clinical course than Marfan syndrome. Children with this syndrome often have a split uvula and hypertelorism. In patients with such findings, echocardiography should be considered.

We also missed the modalities combining surgery and catheterization (“frozen elephant trunk”) from the spectrum of surgical therapeutic options, which are used to replace the ascending aorta, the aortic arch, and the descending aorta in one session and which usually save the patient from having to undergo a second operation.

Unfortunately the authors do not discuss the importance of the angiotensin II receptor blocker losartan, the most important advance in the long term management of patients with aortic disorders in years. Mouse models have shown that losartan can prevent the development of aortic aneurysms (1), and recently, the New England Journal of Medicine reported positive outcomes for losartan in patients with Marfan syndrome (2).

In the light of these data it seems increasingly questionable whether beta blocker monotherapy reflects the current state of knowledge. With regard to the wide spectrum of readers of the Deutsches Ärzteblatt, we wish to point out these new insights.