Circulation, Vol 52, 651-657, Copyright © 1975 by American Heart Association
OR Brown, H DeMots, FE Kloster, A Roberts, VD Menashe and RK Beals
Echocardiographic and phonocardiographic findings in 35 patients with
Marfan's Syndrome and ten patients without Marfan's or other clinically
apparent connective tissue disorders but with angiographic and
echocardiographic evidence of mitral prolapse are reported and compared.
Echocardiography revealed aortic root dilatation and/or mitral valve
prolapse in 97% of the patients with Marfan's Syndrome. Aortic root
dilatation was found in 60% of this group (74% of males, 33% of females)
while mitral valve prolapse was found in 91% (87% of males, 100% of
females). The incidence of aortic dilatation and mitral prolapse in
patients with Marfan's syndrome was essentially equal in children and
adults of the same sex. None of the nine adults or one child with mitral
prolapse but without evidence of Marfan's Syndrome or other clinically
apparent connective tissue disorder had aortic root enlargement.
Ausculatory examination and phnocardiography revealed abnormalities in 54%
of the patients with Marfan's Syndrome. Aortic regurgitation was found in
23% of this group (35% of males, 0% of females) while mitral regurgitation
and/or mitral clicks were found in 46% (39% of males, 58% of females).
Aortic regurgitation was much more frequent in adult males with Marfan's
Syndrome (7/14, 50%) than male children (1/9, 11%), while the incidence of
abnormal mitral sounds was essentially the same in adults (33% of males,
60% of females) and children (43% of males, 57% of females) of the same sex
with Marfan's Syndrome. Abnormal mitral sounds were more frequent in
patients without Marfan's who had mitral prolapse (90%) than in those with
Marfan's (46%). It appears that cardiac abnormalities are a consistent
manifestation of Marfan's Syndrome and that ultrasound is a more sensitive
indicator of these abnormalities in such patients than ausculation or
phonocardiography.
ARTICLES
Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study
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