Elective replacement of the aortic root in Marfan's syndrome. 1993

T Treasure

We offer elective aortic replacement ot those we regard as being at high risk. The aortic root dimension and its rate of increase are the best predictors we have. We regard 5.5 cm as the probable upper limit and we are inclined to advocate surgery at an earlier stage in high risk families and in women planning pregnancy. These operations are planned for a calculated gain in life expectancy and it would be reasonable for there to be a degree of centralisation of referral and development of surgical expertise. We use beta-blockade for life, both before and after surgery, in all patients with Marfan's syndrome with aortic root enlargement. The data on which these recommendations are based are incomplete and we can only hope that with an increasing number of carefully documented cases we will refine them and improve upon them in the future. The concept of a "forme fruste" or a "Marfanoid aorta" in the absence of the syndrome is highly questionable so the comments made apply only to patients with Marfan's syndrome--not to other forms of annuloaortic ectasia or other less well characterised forms of aortic root disease.

UI MeSH Term Description Entries
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
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011249 Pregnancy Complications, Cardiovascular The co-occurrence of pregnancy and a cardiovascular disease. The disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS. Cardiovascular Pregnancy Complications,Complications, Cardiovascular Pregnancy,Pregnancy, Cardiovascular Complications,Cardiovascular Pregnancy Complication,Complication, Cardiovascular Pregnancy,Pregnancies, Cardiovascular Complications,Pregnancy Complication, Cardiovascular
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000784 Aortic Dissection A tear in the inner layer of the AORTA leading to interstitial HEMORRHAGE, and splitting (dissecting) of the aortic TUNICA MEDIA layer. It typically begins with a tear in the TUNICA INTIMA layer. Aneurysm, Dissecting,Aortic Dissecting Aneurysm,Dissecting Aneurysm,Dissecting Aneurysm Aorta,Aneurysm Aorta, Dissecting,Aneurysm, Aortic Dissecting,Aorta, Dissecting Aneurysm,Aortic Dissecting Aneurysms,Aortic Dissections,Dissecting Aneurysm Aortas,Dissecting Aneurysm, Aortic,Dissecting Aneurysms,Dissection, Aortic
D001011 Aorta The main trunk of the systemic arteries. Aortas
D001014 Aortic Aneurysm An abnormal balloon- or sac-like dilatation in the wall of AORTA. Aneurysm, Aortic,Aneurysms, Aortic,Aortic Aneurysms
D001018 Aortic Diseases Pathological processes involving any part of the AORTA. Aortic Disease,Disease, Aortic,Diseases, Aortic
D012307 Risk Factors An aspect of personal behavior or lifestyle, environmental exposure, inborn or inherited characteristic, which, based on epidemiological evidence, is known to be associated with a health-related condition considered important to prevent. Health Correlates,Risk Factor Scores,Risk Scores,Social Risk Factors,Population at Risk,Populations at Risk,Correlates, Health,Factor, Risk,Factor, Social Risk,Factors, Social Risk,Risk Factor,Risk Factor Score,Risk Factor, Social,Risk Factors, Social,Risk Score,Score, Risk,Score, Risk Factor,Social Risk Factor

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