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Eur J Cardiothorac Surg 2005;28:283-285
© 2005 Elsevier Science NL

Original articles

Editorial comment

Institut Mutualiste Montsouris, Paris, France

^* Address: Departement de Pathologie Cardiovasculaire, 42, boulevard Jourdan, 75014 Paris cedex, France. Tel.: +33 1 56616508; fax: +33 1 56616511. (Email: jean.bachet@imm.fr).

The first 20% of the full text of this article appears below.

The article concerning acute type A dissection in pregnant Marfan patients published in the present issue of the EJCTS reflects, through the report of four cases observed over a period of 12 years, the yet often unresolved difficulties entailed by this accident and raises important questions related to its obstetrical and surgical management.

The first striking information brought by this article is the confirmation that despite a large literature dealing with the anatomical, genetic, orthopaedic, cardiovascular, ophthalmologic, obstetrical aspects of Marfan's syndrome, this affection remains ignored as a general medical entity with social as well as individual consequences.

In the present report, despite the fact that Marfan's syndrome had been diagnosed before pregnancy in the four patients, none received any preconceptional counselling regarding the risks of pregnancy and disease transmission to the offspring. Neither did the patients undergo any echographic monitoring of the aortic root size and evolution during pregnancy.

It is indeed quite dismaying to observe that, one century after the description of the syndrome by Antonin Marfan [1], 70 years after the recognition of its autosomal dominant inheritance, 50 years after the description of its various clinical aspects, 20 years after the first classification of the diagnostic criteria in Berlin and finally, 15 years after the discovery of the responsible genes and consequent mutations, the syndrome is not taken care of as a whole even in highly medically and technically developed countries. The cardiologist and the cardiac surgeon take care of the cardiovascular system, the orthopaedist takes care of the skeleton, the ophthalmologist takes care of the eyes but, either by ignorance or inability, or by fear to interfere with another specialty, very few of those specialists actually take care of the . . . [Full Text of this Article]

This article has been cited by other articles:

				M. Sakaguchi Reply to Jahangiri and Clark Eur. J. Cardiothorac. Surg., February 1, 2006; 29(2): 264 - 265. [Full Text] [PDF]