进行性肺动脉扩张与马凡综合征患者的B型主动脉夹层相关。

Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome.

作者信息

Brouwer Christel, Bulut Haldun, van Gemert Willemijn, Staal Alexander Hj, Cortenbach Kim, Snoeren Miranda, Nijveldt Robin, Duijnhouwer Anthonie, Loeys Bart L, van Royen Niels, Timmermans Janneke, van Kimmenade Roland Rj

机构信息

Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands.

Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands.

出版信息

J Clin Med. 2019 Nov 2;8(11):1848. doi: 10.3390/jcm8111848.

DOI:10.3390/jcm8111848

PMID:31684091

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6912475/

Abstract

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 ( = 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 ( < 0.05). There was a significant association between type B dissection and descending aorta diameter (OR 1.14; 95% CI 1.05-1.24 < 0.01) and PA dilatation (OR 1.69; 95% CI 1.03-2.77 = 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 = 0.02) and PA dilatation were statistically significant (OR 1.85; 95% CI 1.10-3.12 = 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.

摘要

目的

马凡综合征（MFS）是一种与严重心血管疾病发病率和死亡率相关的结缔组织疾病。MFS患者的主动脉并发症是否与进行性肺动脉（PA）扩张相关尚不清楚。

方法

我们在我们的专业中心对一组MFS患者进行常规磁共振成像测量PA直径，并对直径以及结果进行随访。

结果

PA扩张定义为直径增加2mm或更多，我们的总队列（n = 162）中有71名患者（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名患者发生夹层，其中14例为A型夹层，10例为B型夹层，4例同时患有两种夹层。在发生夹层的患者中，64%（28例中的18例）有PA扩张，而无夹层的患者组中为39%（134例中的53例）（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）之间存在显著关联。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扩张具有统计学意义（OR 1.85；95%CI 1.10 - 3.12，P = 0.02），而降主动脉直径则无统计学意义。