Walter Corinna, Leinweber Maria Elisabeth, Mlekusch Irene, Assadian Afshin, Hofmann Amun Georg
Department of Vascular and Endovascular Surgery, Klinik Ottakring, 1160 Vienna, Austria.
Diagnostics (Basel). 2024 Aug 12;14(16):1749. doi: 10.3390/diagnostics14161749.
Ehlers-Danlos syndrome (EDS), Marfan syndrome (MFS), and Loeys-Dietz syndrome (LDS) are connective tissue disorders frequently associated with vascular aneurysm formation, dissections, and subsequent major complications. Regular imaging surveillance is recommended for these conditions. However, no guidelines currently exist regarding imaging modality or surveillance intervals.
This retrospective single-center observational study analyzed clinical and imaging data of patients attending an outpatient clinic for vascular connective tissue disorders between August 2008 and January 2024. Imaging (1424 data points in total) and clinical data were extracted from electronic health records. Analysis primarily included a comparison of vessel diameter progression across imaging modalities, with an additional review of the clinical history of vascular events.
In total, 19 patients with vascular connective tissue disorders (vCTDs) underwent consultations at our outpatient clinic. Nine (47.4%) patients experienced vascular events, while two (10.5%) passed away during the study period. Multimodal imaging surveillance revealed a tendency towards arterial diameter increase. Consistent ultrasound monitoring provided more reliable diameter progression data for the same arterial segment than a combination of imaging modalities. Temporal analysis indicated a tendency for the continuous growth of the abdominal aorta, the common and internal carotid artery, and the common femoral and popliteal artery.
The study highlights the importance of standardized, modality-specific imaging protocols in monitoring patients with vCTDs. The variability in disease progression among these patients further complicates surveillance strategies, contemplating the need for individualized approaches. Further research and prospective multicenter studies are required to refine and improve monitoring protocols.
埃勒斯-当洛综合征（EDS）、马凡综合征（MFS）和洛伊茨-迪茨综合征（LDS）是结缔组织疾病，常与血管动脉瘤形成、夹层以及随后的重大并发症相关。建议对这些疾病进行定期影像学监测。然而，目前尚无关于影像学检查方式或监测间隔的指南。
这项回顾性单中心观察性研究分析了2008年8月至2024年1月期间在门诊就诊的血管结缔组织疾病患者的临床和影像学数据。从电子健康记录中提取影像学（共1424个数据点）和临床数据。分析主要包括比较不同影像学检查方式下血管直径的进展情况，并额外回顾血管事件的临床病史。
共有19例血管结缔组织疾病（vCTD）患者在我们的门诊接受了会诊。9例（47.4%）患者发生了血管事件，2例（10.5%）在研究期间死亡。多模态影像学监测显示动脉直径有增加趋势。与多种影像学检查方式联合使用相比，持续的超声监测为同一动脉节段提供了更可靠的直径进展数据。时间分析表明腹主动脉、颈总动脉和颈内动脉以及股总动脉和腘动脉有持续生长的趋势。
该研究强调了标准化的、特定检查方式的影像学方案在监测vCTD患者中的重要性。这些患者疾病进展的变异性使监测策略更加复杂，考虑到需要个体化方法。需要进一步的研究和前瞻性多中心研究来完善和改进监测方案。
