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The Bentall procedure is a type of cardiac surgery involving composite graft replacement of the aortic valve, aortic root, and ascending aorta, with re-implantation of the coronary arteries into the graft. This operation is used to treat combined disease of the aortic valve and ascending aorta, including lesions associated with Marfan syndrome.
Marfan syndrome is named after Antoine Marfan, [11] the French pediatrician who first described the condition in 1896 after noticing striking features in a five-year-old girl. [ 12 ] [ 77 ] The gene linked to the disease was first identified by Francesco Ramirez at the Mount Sinai Medical Center in New York City in 1991.
From 2004-2005, he served as president of the American Association for Thoracic Surgery. [4] He has published extensively in peer reviewed journals, is the author of many chapters in surgical textbooks, and is the editor or co-editor of 5 surgical textbooks. David has received numerous awards.
Congenital contractural arachnodactyly (CCA), also known as Beals–Hecht syndrome, is a rare autosomal dominant congenital connective tissue disorder. [1] As with Marfan syndrome, people with CCA typically have an arm span that is greater than their height and very long fingers and toes. [2]
[9] [15] Surgical repair of the dura may provide symptomatic relief for some patients. [5] It has been reported that acetazolamide can be used to treat dural ectasia in individuals with Marfan syndrome, however, the only supporting evidence for this assertion is a small study of 14 patients which was not peer-reviewed or submitted for publication.
This feature can occur on its own with no underlying health problems, or it can be associated with certain medical conditions, including Marfan syndrome, [1] Ehlers–Danlos syndromes, [2] Loeys–Dietz syndrome, and homocystinuria. [3]
[6] [7] [8] A spontaneous spinal cerebrospinal fluid leak may occur sometimes in those with predisposing heritable connective tissue disorders including Marfan syndrome and Ehlers–Danlos syndromes. [ 5 ] [ 9 ] A loss of CSF greater than its rate of production leads to a decreased volume inside the skull known as intracranial hypotension .
Marfan syndrome; Mitral valve repair; Aortic surgery including valve-sparing aortic root replacement; He is the editor, along with Stephen C. Yang, of Current Therapy in Thoracic and Cardiovascular Surgery. In January 2008, Cameron reported a very large surgical series, the 30-year history of surgical treatment in 372 cases of Marfan syndrome ...