Elefteriades John A
Division of Cardiothoracic Surgery, Yale University Medical Center, 333 Cedar Street, Room 121 FMB, New Haven, Connecticut 06510, USA.
World J Surg. 2008 Mar;32(3):366-74. doi: 10.1007/s00268-007-9398-3.
The purpose of the present study was to use the clinical database at the Yale University Center for Thoracic Aortic Disease to shed light on the pathophysiology of thoracic aortic aneurysm (TAA), the clinical behavior of thoracic aortic aneurysm, and the optimal clinical management.
The Yale database contains information on 3,000 patients with thoracic aortic aneurysm, with 9,000 patient-years of follow-up and 9,000 imaging studies. Advanced statistical techniques were applied to this information.
Analysis provided the following observations: (1) TAA is a genetic disease with a predominantly autosomal dominant mode of inheritance, (2) matrix metalloproteinase (MMP) enzymes are activated in the pathogenesis of TAA, (3) wall tension in TAA approaches the tensile limits of aortic tissue at a diameter of 6 cm, (4) by the time a TAA reaches a clinical diameter of 6 cm, 34% of affected patients have suffered dissection or rupture, (5) extreme physical exertion or severe emotion often precipitate acute dissection, and (6) single nucleotide polymorphisms (SNPs) are being identified which predispose a patient to TAA.
The "playbook" of TAA is gradually being read, with the help of scientific investigations, positioning practitioners to combat this lethal disease more effectively than ever before.
本研究旨在利用耶鲁大学胸主动脉疾病中心的临床数据库，以阐明胸主动脉瘤（TAA）的病理生理学、胸主动脉瘤的临床行为以及最佳临床管理方法。
耶鲁数据库包含3000例胸主动脉瘤患者的信息，随访时间达9000患者年，并有9000份影像学研究资料。对这些信息应用了先进的统计技术。
分析得出以下观察结果：（1）TAA是一种主要以常染色体显性遗传方式遗传的疾病；（2）基质金属蛋白酶（MMP）在TAA发病机制中被激活；（3）TAA的壁张力在直径达6厘米时接近主动脉组织的拉伸极限；（4）当TAA临床直径达到6厘米时，34%的受影响患者已发生夹层或破裂；（5）剧烈体力活动或强烈情绪常促使急性夹层形成；（6）正在识别出使患者易患TAA的单核苷酸多态性（SNP）。
借助科学研究，TAA的“剧本”正逐渐被解读，使从业者能够比以往更有效地对抗这种致命疾病。
