By Robert S. Bonser, Domenico Pagano, Axel Haverich, Jorge Mascaro
In this publication the authors assessment the surgical administration of sufferers with aortic disorder. This usually debatable zone of administration finds many thoughts open to cardiovascular expert. This reference studies each one controversy and offers functional solutions to the cardiac healthcare professional and may support them demonstrate the spectrum of problems and their management.
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J Am Coll Cardiol 2013;95:563–9. 30. Boileau C, Guo DC, Hanna N, et al. TGFB2 mutations cause familial thoracic aortic aneurysms and dissections associated with mild systemic features of Marfan syndrome. Nat Genet. 2012;44(8):916–21. 31. Lindsay ME, Schepers D, Bolar NA, et al. Loss-offunction mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm. Nat Genet. 2012;44:922–7. 32. Milewicz DM, Michael K, Fisher N, Coselli JS, Markello T, Biddinger A. Fibrillin-1 (FBN1) mutations in patients with thoracic aortic aneurysms.
Before the development of open-heart surgical procedures for prophylactic replacement of the aortic root, Marfan patients usually died from aortic dissection or rupture of the proximal aorta at a mean age of 32 years [21, 22]. Currently, Marfan patients and most other individuals with heritable TAAD may enjoy a nearly normal lifeexpectancy because elective replacement of the proximal aorta (type A dissection) is performed before aortic dissection or rupture develop . Method We discuss the question when to perform aortic surgery in adults with Marfan syndrome and other connective tissue disorders to protect against type A dissection.
A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37:275–81. 25. Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788–98. 14 26. Gutman G, Baris HN, Hirsch R, et al. Loeys-Dietz syndrome in pregnancy: a case description and report of a novel mutation. Fetal Diagn Ther. 2009;26:35–7. 27. van de Laar IM, Oldenburg RA, Pals G, et al.
Controversies in Aortic Dissection and Aneurysmal Disease by Robert S. Bonser, Domenico Pagano, Axel Haverich, Jorge Mascaro