Marfan's syndrome. Broad spectral surgical treatment cardiovascular manifestations. 1983

E S Crawford

Most patients with Marfan's syndrome have cardiovascular manifestations and complications of these abnormalities lead to death in 50% of patients by the age of 32. This report is concerned with the performance of 79 operations to control these problems in 41 patients during a 16-year period. There were 3 early deaths and 11 late deaths, with survival at 15 years in 62%. The cardiovascular manifestations assumed eight patterns of involvement, in brief, consisting of aneurysms of ascending aorta, mitral valve insufficiency, aortic dissection and dissecting aortic aneurysm, and degenerative distal fusiform aortic aneurysm. These lesions occurred in isolated form or in association with others, hence the larger number of patterns of involvement. Various methods were employed in treatment of aneurysms of the ascending aorta, which was associated with aortic valvular insufficiency in most. The most reliable method of controlling disease at this level was composite valve graft replacement of fusiform aneurysms and separate valve graft operation for dissections occurring in the previously uninvolved aorta. Isolated mitral valve insufficiency was relieved by standard mitral valve replacement and this operation could be safely combined with composite valve graft replacement of the ascending aorta. More distant aneurysms of the aorta, either degenerative or chronic dissections, were susceptible to reconstruction as employed in the nonMarfan patient. Treatment of multiple lesions was staged, treating the more symptomatic condition first. Regular follow-up examination is important in these patients to detect new lesions and to evaluate known lesions. An aggressive approach is suggested in their treatment because 63% of the 11 late deaths in this series were due to lesions that could be successfully treated by presently available methods.

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
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
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
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D012008 Recurrence The return of a sign, symptom, or disease after a remission. Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006350 Heart Valve Prosthesis A device that substitutes for a heart valve. It may be composed of biological material (BIOPROSTHESIS) and/or synthetic material. Prosthesis, Heart Valve,Cardiac Valve Prosthesis,Cardiac Valve Prostheses,Heart Valve Prostheses,Prostheses, Cardiac Valve,Prostheses, Heart Valve,Prosthesis, Cardiac Valve,Valve Prostheses, Cardiac,Valve Prostheses, Heart,Valve Prosthesis, Cardiac,Valve Prosthesis, Heart

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