Joris H, Demoulin J C
Arch Mal Coeur Vaiss. 1981 Aug;74(8):951-60.
A previously healthy 46 year old man was found to have a murmur suggestive of mitral incompetence, when examined after an accident. Investigations revealed corrected transposition of the great arteries and severe regurgitation of the left atrioventricular valve. A complementary bibliographic study of 19 other cases, aged over 40 years old was undertaken to assess the natural history of the condition, apparently fatal before the fifth decade in cases with associated lesions, shunt or valvular disease, but of better prognosis in the rare cases of isolated corrected transposition. The anatomical study suggested a congenital malformation of the regurgitant valve (cleft and abnormal chordal pattern) but a traumatic origin could not be excluded. Examination of the conduction system showed the presence of degenerative lesions. The atrioventricular node was located in the classical "posterior position", which is unusual in this type of congenital malformation.
一名46岁既往健康的男性在一次事故后接受检查时，发现有提示二尖瓣关闭不全的杂音。检查发现存在大动脉转位矫正型以及左房室瓣严重反流。对另外19例年龄超过40岁的病例进行了补充文献研究，以评估该病的自然史。在伴有相关病变、分流或瓣膜疾病的病例中，该病在50岁之前显然是致命的，但在罕见的单纯大动脉转位矫正型病例中预后较好。解剖学研究提示反流瓣膜存在先天性畸形（瓣叶裂和异常腱索形态），但不能排除创伤性起源。传导系统检查显示存在退行性病变。房室结位于经典的“后位”，这在这种类型的先天性畸形中并不常见。
