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Cardiovascular disease in children with marfan syndrome
Robida, Andrej
To study the cardiovascular changes in children with Marfan syndrome the hospital records of 27 children with this syndrome admitted to our institution between 1975 and 1984 were reviewed. ...Echocardiographic examinationwas done in all of them. Heart catheterization and angiocardiography were performed in 7 children. 15 children were without cardiovascular involvement (mean age 7.9 years). Twelve children with cardiovascular abnormalities (mean age 12.3 years) were significantly older than children without cardiovascular disease (P<0.02). The most frequrnt cardiovascular abnormality (9 children) was mitral valve diseas (mitral regurgitation, mitral valve prolapse with and without regurgitation). In one patient artificial mitral valve was inserted. Aortic root aneurysm was found in 7 children, in some of them in combination with mitral valve disease. Fusiform aneurysm of thoracic aorta together with aortic root aneurysm was present in one child. In 2 patients tetralogy of Fallot was diagnosed. This review shows that the cardiovascular system is more frequently affected in older children and that about half of the children suffer from cardiovascular disease. Mitral valve involvement predominates. Surgery for the correction of the cardiovascular abnormalities in children with Marfan syndrome is seldom required.
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