Mechanisms underlying aortic dilatation in congenital aortic valve malformation
Mechanisms underlying aortic dilatation in congenital aortic valve malformation
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DOI：
10.1161/01.cir.99.16.2138
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发表时间：
1999-04-27
期刊：
影响因子：
37.8
通讯作者：
Lang, IM
中科院分区：
文献类型：
--
作者：
Bonderman, D;Gharehbaghi-Schnell, E;Lang, IM
背景-先天性主动脉瓣畸形患者主动脉疾病的高发病率提示这两种情况之间存在因果关系。主动脉扩张/动脉瘤/夹层的组织学观察结果是Erdheim囊状中层坏死（CMN），平滑肌细胞（SMC）的非炎性损失，弹性纤维的断裂和粘液样变性。从32例患者的胸心手术中收集升主动脉壁标本，并通过组织化学染色和末端脱氧核苷酸转移酶介导的脱氧尿苷三磷酸缺口末端标记进行检查。根据超声心动图结果，确定了4组患者：二叶瓣、带（bi/dil）或不带（bi/0）主动脉瓣扩张的瓣膜载体和带（tri/dil）或不带（tri/0）主动脉瓣扩张的三叶瓣载体。与tri/0（mAI，0.9+/-1.2; P=0.0079和P=0.037）相比，在bi/dil（平均凋亡指数[mAI]，8.1 +/-6.0）和tri/dil（mAI，8.1 +/-8.3）的中层中观察到大量局灶性细胞凋亡。与tri/0（mAI，0.9+/-1.2）相比，bi/0（mAI，9.1+/-5.7）中的中膜SMC凋亡率增加（P=0.0025）。Bi/dil组（平均年龄40.6 ± 15.7岁）明显小于tri/dil组（平均年龄56.4 ± 12.8岁）（P= 0. 0123. Conclusion-premature medial layer SMC apoptosis may be part of a genetic program underlying aortic disease in patients with aortic valve malformations.
Background-The high incidence of aortic disease in subjects with congenital aortic valve malformations suggests a causative relationship between these 2 conditions. The histological observation in aortic dilatation/aneurysm/dissection is Erdheim cystic medial necrosis (CMN), a noninflammatory loss of smooth muscle cells (SMCs), fragmentation of elastic fibers, and mucoid degeneration.Methods aad Results-To examine whether apoptosis is 1 of the mechanisms underlying CMN and aortic medial layer SMC loss, ascending aortic wall specimens from 32 patients were collected at cardiothoracic surgery and examined by histochemical staining and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling. From echocardiography results, 4 groups of patients were identified: bicuspid,valve carriers with (bi/dil) or without (bi/0) aortic:dilatation and tricuspid valve carriers with (tri/dil) or without (tri/0) aortic dilatation,Massive focal apoptosis was observed in the medial layers of bi/dil (mean apoptotic index [mAI], 8.1+/-6.0) and tri/dil (mAI, 8.1 +/- 8.3) compared with tri/0 (mAI, 0.9+/-1.2; P=0.0079 and P=0.037). In bi/0 (mAI, 9.1+/-5.7) compared with tri/0 (mAI, 0.9+/-1.2), rates of medial SMC apoptosis were-increased (P=0.0025). Bi/dil (mean age, 40.6+/-15.7 years) were significantly younger than tri/dil (mean age, 56.4+/-12.8 years) undergoing the same operation (P=0.0123).Conclusions-Premature medial layer SMC apoptosis could be part of a genetic program underlying aortic disease in patients with aortic valve malformations.