Heart and Vascular Center, Semmelweis University, Városmajor u. 68, Budapest, H-1122, Hungary.
Hungarian Marfan Foundation, Városmajor u. 68, Budapest, H-1122, Hungary.
Orphanet J Rare Dis. 2020 Apr 15;15(1):91. doi: 10.1186/s13023-020-01369-w.
Clinical evidence suggests that the currently recommended approach to estimate the risk of aortic dissection in Marfan syndrome (MFS) is not reliable enough. Therefore, we investigated the possible role of visceral arterial tortuosity in the risk stratification.
Splenic and renal arteries of 37 MFS patients and 74 age and gender matched control subjects were segmented using CT angiography imaging. To measure tortuosity, distance metric (DM), sum of angles metric (SOAM), inflection count metric (ICM), and the ratio of ICM and SOAM (ICM/SOAM) were calculated. DM of the splenic, right and left renal artery was significantly higher in MFS patients than in controls (2.44 [1.92-2.80] vs. 1.75 [1.57-2.18] p < 0.001; 1.16 [1.10-1.28] vs. 1.11 [1.07-1.15] p = 0.011; 1.40 [1.29-1.70] vs. 1.13 [1.09-1.23] p < 0.001, respectively). A similar tendency for ICM and an opposite tendency for SOAM were observed. ICM/SOAM was significantly higher in the MFS group compared to controls in case of all three arteries (73.35 [62.26-93.63] vs. 50.91 [43.19-65.62] p < 0.001; 26.52 [20.69-30.24] vs. 19.95 [16.47-22.95] p < 0.001; 22.81 [18.64-30.96] vs. 18.38 [15.29-21.46] p < 0.001, respectively). MFS patients who underwent aortic root replacement had increased right and left renal DM and ICM/SOAM compared to MFS patients without surgery.
To our knowledge this is the first demonstration of increased arterial tortuosity in MFS on visceral arteries. Visceral arterial tortuosity, dominated by curves of lower frequency but higher amplitude according to the observed opposite tendency between the DM and SOAM metrics, could be a possible new predictor of serious manifestations of MFS.
临床证据表明，目前推荐的用于估计马凡综合征（MFS）主动脉夹层风险的方法并不足够可靠。因此，我们研究了内脏动脉迂曲在危险分层中的可能作用。
使用 CT 血管造影成像对 37 名 MFS 患者和 74 名年龄和性别匹配的对照者的脾动脉和肾动脉进行分割。为了测量迂曲度，计算了距离度量（DM）、角度总和度量（SOAM）、拐点计数度量（ICM）和 ICM 与 SOAM 的比值（ICM/SOAM）。与对照组相比，MFS 患者的脾动脉、右肾动脉和左肾动脉的 DM 明显更高（2.44 [1.92-2.80] 比 1.75 [1.57-2.18]，p < 0.001；1.16 [1.10-1.28] 比 1.11 [1.07-1.15]，p = 0.011；1.40 [1.29-1.70] 比 1.13 [1.09-1.23]，p < 0.001）。观察到 ICM 有类似的趋势，而 SOAM 则相反。在脾动脉、右肾动脉和左肾动脉中，MFS 组的 ICM/SOAM 均明显高于对照组（73.35 [62.26-93.63] 比 50.91 [43.19-65.62]，p < 0.001；26.52 [20.69-30.24] 比 19.95 [16.47-22.95]，p < 0.001；22.81 [18.64-30.96] 比 18.38 [15.29-21.46]，p < 0.001）。与未行主动脉根部置换术的 MFS 患者相比，行主动脉根部置换术的 MFS 患者的右肾和左肾 DM 及 ICM/SOAM 均增加。
据我们所知，这是首次在 MFS 内脏动脉中观察到动脉迂曲增加。内脏动脉迂曲，由较低频率但较高幅度的曲线主导，根据 DM 和 SOAM 度量之间观察到的相反趋势，可能是 MFS 严重表现的一个新的潜在预测指标。
