儿童先天性主动脉瓣畸形32例病理组织学特征

Histopathological study of congenital aortic valve malformation in 32 children

目的探讨儿童先天性主动脉瓣畸形的病理组织学特征。方法对2003年1月至2005年12月因先天性主动脉瓣畸形、主动脉瓣功能障碍行外科手术切除的32例儿童主动脉瓣进行大体观察和组织学检查。结果 32例儿童先天性主动脉瓣畸形中,男27例,女5例,男:女=5．4:1,年龄6-18岁,平均年龄14．9岁。二叶型12例(37．5％),三叶型 19例(59．4％),四叶型1例(3．1％)。主动脉瓣狭窄(AS)5例(15．6％),主动脉瓣关闭不全(AI)25例(78．1％),AS-AI 2 例(6．2％),均不伴其他心脏瓣膜病变,20例伴其他先天性心脏病。病理组织学改变为瓣叶增厚、大小不等、不规则(卷曲或脱垂),部分伴钙化,光镜下示纤维组织增生,黏液样变性,胶原纤维溶解、断裂,弹性纤维破坏,胶原纤维玻璃样变性2 例、灶性钙化2例,无小血管增生及炎性细胞浸润。4例可见心内膜面局部破溃,内皮下毛细血管增生,纤维素性渗出,炎性细胞浸润,继发性钙质、脂质沉着和纤维化。结论儿童先天性主动脉瓣畸形以男性多见,二叶型、三叶型主动脉瓣常见,常伴有其他先天性心脏病,瓣膜功能障碍以AI为主。

Objective To investigate the histopathological characteristics of congenital aortic valve malformation in children. Methods All the native surgically excised aortic valves from 32 pediatric patients with symptomatic aortic valve dysfunction due to congenital aortic valve malformation admitted from January 2003 to December 2005 were studied macroscopically and microscopically. The patients＇ medical records were reviewed and the clinical information was extracted. The diagnosis was made by the clinical presentation, preoperative echocardiography, intraoperative examination, and postoperative histopathological study,excluding rheumatic or degenerative aortic valve disease, infective endocarditis and primary connective tissue disorders, eg, Marfan syndrome. Results Among 32 aortic valves,patient age was ranged from 6 to 18 years,with a mean value of 14. 9 years, and there were 27 men and 5 women, male：female = 5.4：1. There were 5 aortic stenosis （AS, 15.6% ）, 25 aortic insufficiency （AI,78. 1% ） and 2 AS-AI （6. 2% ） cases, without other valve diseases. Twenty had other congenital heart diseases： ventricular septal defect, 19; patent ductus arteriosus, 2; double chamber right ventricles, 1; aortic right coronary sinus aneurysm, 3. Histopathological examination indicated that the cusp became thickening with unequal size, irregular shape （coiling and prolapse edge） ,enhanced hardness, and partly calcification. Microscopical investigation showed the unsharp structure of valve tissue, fibrosis,myxomatous, reduce of collagen fiber, rupture of elastic fibers, infiltration of inflammatory cells with different degrees, secondary calcareous and lipid deposit, and secondary fibrosis. Conclusions Congenital aortic valve malformation in children involves male more than female,is mostly associated with other congenital heart diseases. Aortic insut^ciency is more common in children with congenital aortic valve malformation. Histopathologically,the leaflets of aortic valve are mainly myxomatous,thickening with unequal size, irregular shape （coiling and prolapse edge）, reduce of collagen fiber, rupture of elastic fibers, without small vessel proliferation and inflammatory cell infiltration, fibrosis and calcification are rarely seen.