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Thoracoabdominal aortic aneurysm associated with Marfan's syndrome--report of a case. 1991

M Kubota, and Y Osuka, and Y Hojo, and M Miyake, and T Shiba, and J Oono, and S Kimura
Second Department of Surgery, Ehime University School of Medicine, Japan.

A case of Marfan's syndrome associated with thoracoabdominal aortic aneurysm and mitral regurgitation in a 29 year old male is reported herein. The aneurysm was replaced with a Y-shaped graft using Crawford's technique, while the major branches of the abdominal aorta were separately cannulated from inside the aneurysm and perfused via partial extracorporeal circulation using a left femoro-femoral bypass. We found this technique useful in the prevention of tissue ischemia during the operation. The patient's postoperative course was uneventful and he has encountered no problems in the year and half since his operation.

UI MeSH Term Description Entries
D008297 Male Males
D008382 Marfan Syndrome An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2. Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D008944 Mitral Valve Insufficiency Backflow of blood from the LEFT VENTRICLE into the LEFT ATRIUM due to imperfect closure of the MITRAL VALVE. This can lead to mitral valve regurgitation. Mitral Incompetence,Mitral Regurgitation,Mitral Valve Incompetence,Mitral Insufficiency,Mitral Valve Regurgitation,Incompetence, Mitral,Incompetence, Mitral Valve,Insufficiency, Mitral,Insufficiency, Mitral Valve,Regurgitation, Mitral,Regurgitation, Mitral Valve,Valve Incompetence, Mitral,Valve Insufficiency, Mitral,Valve Regurgitation, Mitral
D001807 Blood Vessel Prosthesis Device constructed of either synthetic or biological material that is used for the repair of injured or diseased blood vessels. Vascular Prosthesis,Blood Vessel Prostheses,Tissue-Engineered Vascular Graft,Graft, Tissue-Engineered Vascular,Grafts, Tissue-Engineered Vascular,Prostheses, Blood Vessel,Prostheses, Vascular,Prosthesis, Blood Vessel,Prosthesis, Vascular,Tissue Engineered Vascular Graft,Tissue-Engineered Vascular Grafts,Vascular Graft, Tissue-Engineered,Vascular Grafts, Tissue-Engineered,Vascular Prostheses,Vessel Prostheses, Blood,Vessel Prosthesis, Blood
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001012 Aorta, Abdominal The aorta from the DIAPHRAGM to the bifurcation into the right and left common iliac arteries. Abdominal Aorta,Abdominal Aortas,Aortas, Abdominal
D001013 Aorta, Thoracic The portion of the descending aorta proceeding from the arch of the aorta and extending to the DIAPHRAGM, eventually connecting to the ABDOMINAL AORTA. Aorta, Ascending,Aorta, Descending,Aortic Arch,Aortic Root,Arch of the Aorta,Descending Aorta,Sinotubular Junction,Ascending Aorta,Thoracic Aorta,Aortic Roots,Arch, Aortic,Ascending Aortas,Junction, Sinotubular,Root, Aortic,Sinotubular Junctions
D001014 Aortic Aneurysm An abnormal balloon- or sac-like dilatation in the wall of AORTA. Aneurysm, Aortic,Aneurysms, Aortic,Aortic Aneurysms
D014656 Vascular Surgical Procedures Operative procedures for the treatment of vascular disorders. Vascular Surgery,Procedure, Vascular Surgical,Procedures, Vascular Surgical,Surgical Procedure, Vascular,Surgical Procedures, Vascular,Vascular Surgical Procedure,Surgeries, Vascular,Surgery, Vascular,Vascular Surgeries

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