Zhuraev R K, Zerbino D D
Angiol Sosud Khir. 2014;20(4):14-20.
The authors examined the state of patients suffering from Marfan syndrome (MS) who endured operation for ascending aorta aneurysm with replacement of the ascending aorta and aortic valve (Bentall operation), studying alterations of the skeleton, face, heart and eyes, as well as pathomorphological restructurings in the aortic wall. The study was carried out 7.0 ± 4.2 years after the operation. We examined a total of 39 patients with MS - 27 (69.2%) men and 12 (30.8%) women aged from 22 to 70 years old (average age - 42.1 ± 13.4 years). All patients were operated on for dissecting aneurysm of the ascending aorta accompanied by a considerable degree of aortic valve insufficiency or aortic ostium stenosis. The mean diameter of the aorta at the level of the sinuses of Valsalva amounted to 7.0 ± 1.3 cm (minimal - 5.0 cm, maximal - 12.0 cm), the Z-score prior to operation was 12.7 ± 6.5. The time form making the diagnosis of MS to surgical intervention for aortic aneurysm amounted to 9.6 ± 5.9 years. The condition after operative treatment in all patients was satisfactory, with the haemodynamic indices stable: systolic AP - 133.5 ± 19.1 mm Hg, diastolic AP 85.1 ± 12.9 mm Hg, heart rate 74.8 ± 7.2 bpm. The average systemic score for the symptoms and tests of MS patients amounted to 8.2 ± 3.3 points. Pathohistological alterations of the aorta in patients with Marfan syndrome consisted in pronounced restructuring of the wall with deep irreversible alternative changes. The pathological process extended in the middle aortic layer all alone the length, but not only in the portions of rupture and dissection. The main pathomorphological signs in MS were as follows: focal accumulations of mucoid substances, dystrophic alterations of smooth-muscle cells, ribbon-like anuclear zones, formation of cystlike cavities, alterations of elastic fibres - fragmentation, hyperelastosis, multiplication, thinning and straightening, zones of elastolysis.
作者对患有马凡综合征（MS）且接受升主动脉瘤手术并置换升主动脉和主动脉瓣（Bentall手术）的患者状态进行了检查，研究骨骼、面部、心脏和眼睛的改变，以及主动脉壁的病理形态学重构。该研究在手术后7.0±4.2年进行。我们共检查了39例MS患者，其中男性27例（69.2%），女性12例（30.8%），年龄在22至70岁之间（平均年龄42.1±13.4岁）。所有患者均因升主动脉夹层动脉瘤伴相当程度的主动脉瓣关闭不全或主动脉瓣口狭窄而接受手术。在主动脉瓣窦水平的主动脉平均直径为7.0±1.3厘米（最小5.0厘米，最大12.0厘米），术前Z评分12.7±6.5。从诊断MS到因主动脉瘤进行手术干预的时间为9.6±5.9年。所有患者术后治疗情况良好，血流动力学指标稳定：收缩压133.5±19.1毫米汞柱，舒张压85.1±12.9毫米汞柱，心率74.8±7.2次/分钟。MS患者症状和检查的平均全身评分为8.2±3.3分。马凡综合征患者主动脉的病理组织学改变表现为壁的明显重构，伴有深度不可逆的交替变化。病理过程仅沿长度在主动脉中层扩展，而非仅在破裂和夹层部分。MS的主要病理形态学征象如下：黏液样物质的局灶性积聚、平滑肌细胞的营养不良性改变、带状无核区、囊样腔隙形成、弹性纤维改变——断裂、弹性组织增生、增多、变薄和变直、弹性组织溶解区。
