By Prof. Reed E. Pyeritz (auth.), Prof. Dr. R. Hetzer, P. Gehle, PD Dr. J. Ennker (eds.)
Marfan Syndrome is a hereditary illness of connective tissue which essentially impacts the eyes, skeleton, blood vessels, and numerous constructions of the center.
Since 1943, while the cardiovascular manifestations of Marfan Syndrome have been first defined, realizing of its nature, diagnostic instruments, and surgical procedure were remarkably more desirable. even though the categorical surgeries have reached a sophisticated ordinary and normal recognition, many questions stay unanswered and feature given upward push to ongoing controversy.
Cardiovascular elements of Marfan Syndrome provides the advances in knowing the reason and pathogenesis of Marfan Syndrome and its present particular surgical therapy.
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Additional info for Cardiovascular Aspects of Marfan Syndrome
Amer J Cardiol 39: 13 10. Schlosser V, Knapp I, Schaefer HE (1987) Vergleichende klinisch-morphologische Untersuchungen zur Bedeutung der zystischen Medianekrose in der kardiovaskularen Chirurgie. Vasa 16: 40 II. Spillner G, Mittermaier Ch, Schlosser V (1978) Die Bedeutung der zystischen Media-Nekrose von Aorta und groBen Arterien. -Doz. Dr. G. Fraedrich Abteilung Herz- und GefaBchirurgie Chirurgische UniversiHitsklinik Hugstetter StraBe 55 79106 Freiburg im Breisgau, FRG 37 Surgical therapy for Marfan Syndrome - Then and now H.
Nature 352: 330-334 8. Marfan AB un cas de deformation congenitale des quatre membres, plus prononcee aux extremeties, characterisee par l'allongement des os, avec un certain degre d'amincissiment. Bull Mem Soc MeD Hop. (Paris) 13: 220-226 23 9. Maslen CL, Corson GM, Maddox BK, Glanville RW & Sakai LY (1991) Partial sequence of a candidate gene for the Marfan syndrome. Nature 352: 334-337 10. Miall-Allen VM, Taylor JFN, Rees PG, Allwork SP, Howarth SG (1991) Comparative aortic root measurement in controls and iu children and adolescents with Marfan's syndrome.
In some patients heart failure is rapidly progressive and much more severe than anticipated from the degree of valve insufficiency only. In this context the question has been raised, therefore, whether the basic defect in the coding for microfibrillar components of the connective tissues also may affect the integrity of the myocardial collagen meshwork. This remains as yet a matter of further investigations. The major catastrophe that can occur is that of aortic wall rupture, with or without aortic dissection (Fig.