Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome
Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome
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DOI：
10.3390/jcm8111848
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发表时间：
2019-11-01
影响因子：
3.9
通讯作者：
van Kimmenade, Roland R. J.
中科院分区：
文献类型：
--
作者：
Brouwer, Christel;Bulut, Haldun;van Kimmenade, Roland R. J.
目的：马凡综合征（MFS）是一种结缔组织疾病，与严重的心血管疾病的发病率和死亡率。目前尚不清楚MFS的主动脉并发症是否与进行性肺动脉（PA）扩张相关。研究方法：我们在我们专业中心的MFS患者人群中通过常规磁共振成像测量PA直径，并随访直径和结局。结果如下：PA扩张定义为直径增加2 mm或以上，我们的总队列（n = 162）中有71例患者（44%）符合该标准;两次扫描之间的平均随访时间为8.6年（标准差（SD）+/- 2.7年）。此外，28例患者发生夹层，其中14例为A型夹层，10例为B型夹层，4例患者同时发生两种夹层。在发生夹层的患者中，64%（28例中有18例）的PA扩张，而在未发生夹层的患者组中，39%（134例中有53例）的PA扩张（p < 0.05）。B型夹层与降主动脉直径（OR 1.14; 95% CI 1.05-1.24 p < 0.01）和PA扩张（OR 1.69; 95% CI 1.03-2.77 p = 0.04）之间存在显著相关性。在多变量分析中，B型夹层的最终模型中，只有收缩压（OR 1.06; 95% CI 1.01-1.11 p = 0.02）和PA扩张具有统计学显著性（OR 1.85; 95% CI 1.10-3.12 p = 0.02），而降主动脉直径无统计学显著性。结论：我们报告了进行性PA扩张和B型夹层之间的相关性。我们的研究结果鼓励在MFS PA尺寸的新的兴趣。
Objective: Marfan syndrome (MFS) is a connective tissue disorder associated with severe cardiovascular morbidity and mortality. It is unknown if aorta complications in MFS are associated with progressive pulmonary artery (PA) dilatation. Methods: We measured the PA diameter on routine magnetic resonance imaging in a population of MFS patients seen in our specialised centre with follow up of diameters as well as the outcome. Results: PA dilatation was defined as an increase in diameter of 2 mm or more, and 71 patients (44%) of our total cohort (n = 162) met this criterion; mean follow up between two scans was 8.6 years (standard deviation (SD) +/- 2.7 years). Furthermore, 28 patients suffered from dissections, of which 14 had a type A dissection, 10 had a type B dissection, and 4 patients suffered from both. Of those who suffered from dissection, 64% (18 out of 28) had a dilatation of the PA, versus 39% (53 out of 134) in the patient group without a dissection (p < 0.05). There was a significant association between type B dissection and descending aorta diameter (OR 1.14; 95% CI 1.05-1.24 p < 0.01) and PA dilatation (OR 1.69; 95% CI 1.03-2.77 p = 0.04). In the multivariable analysis the final model for type B dissection, only systolic blood pressure (OR 1.06; 95% CI 1.01-1.11 p = 0.02) and PA dilatation were statistically significant (OR 1.85; 95% CI 1.10-3.12 p = 0.02) while descending aorta diameter was not. Conclusions: We report an association between progressive PA dilatation and type B dissection. Our findings encourage a renewed interest in PA dimensions in MFS.