AORTIC ROOT DILATATION AND MITRAL-VALVE PROLAPSE IN MARFANS-SYNDROME - ECHOCARDIOGRAPHIC STUDY
AORTIC ROOT DILATATION AND MITRAL-VALVE PROLAPSE IN MARFANS-SYNDROME - ECHOCARDIOGRAPHIC STUDY
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DOI：
10.1161/01.cir.52.4.651
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发表时间：
1975-01-01
期刊：
影响因子：
37.8
通讯作者：
BEALS, RK
中科院分区：
文献类型：
--
作者：
BROWN, OR;DEMOTS, H;BEALS, RK
超声心动图和心音图的结果在35例马凡氏综合征和10例无马凡氏或其他临床明显的结缔组织疾病，但与二尖瓣脱垂的血管造影和超声心动图证据的报告和比较。超声心动图显示97%的马凡氏综合征患者主动脉根部扩张和/或二尖瓣脱垂。该组60%的患者（男性74%，女性33%）发现主动脉根部扩张，而91%的患者（男性87%，女性100%）发现二尖瓣脱垂。马凡氏综合征患者主动脉扩张和二尖瓣脱垂的发生率在相同性别的儿童和成人中基本相等。9名成人或1名儿童二尖瓣脱垂，但没有马凡氏综合征或其他临床明显的结缔组织疾病的证据有主动脉根部扩大。54%的马凡氏综合征患者的听诊和心音图显示异常。该组23%的患者（男性35%，女性0%）发现主动脉瓣返流，而46%的患者（男性39%，女性58%）发现二尖瓣返流和/或二尖瓣喀喇音。主动脉瓣返流在成年男性马凡氏综合征（7/14，50%）中的发生率远高于男性儿童（1/9，11%），而二尖瓣音异常的发生率在成年（33%的男性，60%的女性）和同一性别的马凡氏综合征儿童（43%的男性，57%的女性）中基本相同。二尖瓣脱垂的非马凡氏病患者（90%）的二尖瓣音异常比马凡氏病患者（46%）更常见。心脏异常似乎是马凡氏综合征的一致表现，超声是此类患者中这些异常的比听诊或心音描记术更敏感的指标。
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.