Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands.
Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, Amsterdam, the Netherlands.
J Am Coll Cardiol. 2015 Jan 27;65(3):246-54. doi: 10.1016/j.jacc.2014.10.050.
Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome.
The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome.
Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome.
Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome (mean age 36 ± 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery (hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter ≥27 mm (hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections (hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030).
Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections.
马凡综合征患者的降主动脉夹层是一个主要的临床问题。
本研究旨在确定与 B 型主动脉夹层相关的临床参数，并建立预测马凡综合征患者 B 型主动脉夹层的风险模型。
对诊断为马凡综合征的患者进行随访，中位随访时间为 6 年，以观察是否发生 B 型夹层或 B 型主动脉夹层、远端主动脉手术和死亡的复合终点。使用各种临床参数和基因分型建立模型，以预测马凡综合征患者发生 B 型夹层的风险。
1998 年至 2013 年间，600 例马凡综合征患者中发生 54 例 B 型主动脉夹层（平均年龄 36 ± 14 岁，52%为男性）。与 B 型主动脉夹层相关的独立变量包括预防性主动脉手术史（风险比：2.1；95%置信区间：1.2 至 3.8；p = 0.010）和降主动脉近端直径≥27 mm（风险比：2.2；95%置信区间：1.1 至 4.3；p = 0.020）。在风险模型中，低危、中危和高危患者 10 年发生 B 型主动脉夹层的风险分别为 6%、19%和 34%。血管紧张素 II 受体阻滞剂治疗与较少发生 B 型主动脉夹层相关（风险比：0.3；95%置信区间：0.1 至 0.9；p = 0.030）。
即使降主动脉仅轻微扩张，行预防性主动脉手术的马凡综合征患者发生 B 型主动脉夹层的风险也很大。血管紧张素 II 受体阻滞剂治疗可能对预防 B 型主动脉夹层有保护作用。
