AORTIC DILATION & MARFAN SYNDROME

马凡氏综合征是一种先天性疾病，其中马凡氏蛋白生物合成异常。主动脉根部逐渐扩张和随后的破裂是这种疾病患者早期死亡的病理过程。β-肾上腺素能受体拮抗剂被证明延迟该过程，因此是标准的长期预防性治疗。然而，这些药物具有副作用，经常影响患者对其方案的依从性。在这项研究中，我们将前瞻性地评估长效维拉帕米对马凡氏综合征患者主动脉扩张延迟的疗效。不耐受或不愿意依从b-肾上腺素能受体拮抗剂的患者将随机接受长效维拉帕米与安慰剂治疗。将进行系列超声心动图评价。研究持续时间为2年，包括1年中期安全性监测。将测量所有患者的以下参数：心率、血压、主动脉直径、横截面积和主动脉比率、射血力、主动脉僵硬度、扩张性和脉搏波速度。将通过比较该组与b受体阻滞剂组和无治疗组来评价维拉帕米治疗的疗效。

Marfan syndrome is a congenital disease in which fibrillin biosynthesis is abnormal. Gradual aortic root dilation and subsequent rupture is the pathologic process responsible for early demise in patients with this disease. b-adrenergic receptor antagonists are proven to delay this process, and are therefore the standard long term prophylactic treatment. These medications however have side effects that frequently impacts on the patients' compliance with their regimen. In this study we will prospectively assess the efficacy of long acting verapamil for the delay of aortic dilation in patients with Marfan syndrome. Patients that are intolerant to or unwilling to comply with b-adrenergic receptor antagonists will be offered randomization to treatment with long acting verapamil versus placebo. Serial echocardiographic evaluation will be performed. The duration of the study is two years, with 1 year interim safety monitoring. The following parameters will be measured in all patients: Heart rate, blood pressure, aortic diameter, cross sectional area and aortic ratio, ejection force, aortic stiffness, distensibility and pulse wave velocity. The efficacy of treatment with verapamil will be evaluated by comparing this group with the b-blocker and no treatment groups.