Faculty of Medicine, University of Oslo, Norway.
Genet Med. 2012 Nov;14(11):922-7. doi: 10.1038/gim.2012.82. Epub 2012 Jul 12.
The objectives of this study were to establish the prevalence of pulmonary artery dilatation in Marfan syndrome using modern radiological methods and to correlate the diameter of the vessel with aortic disease.
Magnetic resonance or computed tomography imaging of the pulmonary artery and aorta was performed in 87 patients with proven Marfan syndrome. Diameters of the root and trunk of the pulmonary artery and of the aortic root were measured perpendicular to the long axes of the vessels. Pulmonary artery diameters were measured on axial images, and aortic diameters were assessed on oblique sagittal images.
As compared with normal values in the literature, 47 of the 87 patients (54%) had widening of the trunk of the pulmonary artery (≥30 mm). Of these 47, 15% had no sign of disease of the ascending aorta. The mean (SD) ratio between the diameters of the root and trunk of the pulmonary artery was 1.18 (0.155). Multivariate analysis showed that surgery of the ascending aorta and high body surface area were associated with dilatation of the trunk of the pulmonary artery.
Pulmonary artery dilatation is present in a high proportion of patients with Marfan syndrome as assessed using cutoff values based on measurements in the normal population. Severe disease of the ascending aorta correlates significantly with pulmonary artery trunk dilatation in patients with Marfan syndrome.
本研究旨在采用现代影像学方法确定马凡综合征患者肺动脉扩张的患病率，并将血管直径与主动脉疾病相关联。
对 87 例确诊为马凡综合征的患者进行了肺动脉和主动脉的磁共振或计算机断层扫描成像。垂直于血管长轴测量肺动脉根部和主干以及主动脉根部的直径。在轴位图像上测量肺动脉直径，在斜矢状位图像上评估主动脉直径。
与文献中的正常值相比，87 例患者中有 47 例（54%）的肺动脉主干增宽（≥30mm）。在这 47 例患者中，15%没有升主动脉疾病的迹象。肺动脉根部和主干直径的平均（SD）比值为 1.18（0.155）。多变量分析表明，升主动脉手术和高体表面积与肺动脉主干扩张有关。
根据基于正常人群测量值的截断值评估，马凡综合征患者中存在相当比例的肺动脉扩张。马凡综合征患者中严重的升主动脉疾病与肺动脉主干扩张显著相关。
