Abstract：
OBJECTIVE: We aimed to cardiologically evaluate the consequences of glycosaminoglycan (GAG) accumulation in the large vessels of patients with mucopolysaccharidosis (MPS).
METHODS: The left ventricular wall thickness, left ventricular mass (LVmass) were evaluated and aortic annulus diameter (AA), aortic sinus valsalva diameter (SV), sinotubular junction diameter (STJ), systolic aortic diameter (ADs), diastolic aortic diameter (ADd) body indices were obtained by dividing by the surface area. Aortic distensibility and stiffness index were obtained using aortic strain. Ejection fraction, mitral E and A velocities, mitral early diastolic tissue velocity (e\'), E/A ratio, and E/e\' ratio were evaluated.
RESULTS: The LVED-i, LVmass-i, AA-i, SV-i, STJ-i, ADs-i, and ADd-i values were significantly higher in the MPS group. While the E and e\' velocities and E/A ratio were significantly low in the MPS group, the A velocity and E/e\' ratio were significantly high. While the stiffness index, SBP, and PP values were significantly higher in the MPS group, the aortic strain and distensibility were significantly lower. There was a correlation between the stiffness index and the aortic strain, distensibility, SBP, PP, and ventricular function. Cardiac function, aortic diameter, and aortic elasticity characteristics were similar between patients with MPS who received ERT and those who did not.
CONCLUSIONS: In the MPS group, aortic elasticity properties were impaired, and aortic stiffness increased. ERT has positive effects on cardiac function, aortic diameter, and aortic stiffness in MPS patients. An increased LVmass-i and impaired ventricular geometric structure in patients with MPS may be associated with increased aortic stiffness.
METHODS: The left ventricular wall thickness, left ventricular mass (LVmass) were evaluated and aortic annulus diameter (AA), aortic sinus valsalva diameter (SV), sinotubular junction diameter (STJ), systolic aortic diameter (ADs), diastolic aortic diameter (ADd) body indices were obtained by dividing by the surface area. Aortic distensibility and stiffness index were obtained using aortic strain. Ejection fraction, mitral E and A velocities, mitral early diastolic tissue velocity (e\'), E/A ratio, and E/e\' ratio were evaluated.
RESULTS: The LVED-i, LVmass-i, AA-i, SV-i, STJ-i, ADs-i, and ADd-i values were significantly higher in the MPS group. While the E and e\' velocities and E/A ratio were significantly low in the MPS group, the A velocity and E/e\' ratio were significantly high. While the stiffness index, SBP, and PP values were significantly higher in the MPS group, the aortic strain and distensibility were significantly lower. There was a correlation between the stiffness index and the aortic strain, distensibility, SBP, PP, and ventricular function. Cardiac function, aortic diameter, and aortic elasticity characteristics were similar between patients with MPS who received ERT and those who did not.
CONCLUSIONS: In the MPS group, aortic elasticity properties were impaired, and aortic stiffness increased. ERT has positive effects on cardiac function, aortic diameter, and aortic stiffness in MPS patients. An increased LVmass-i and impaired ventricular geometric structure in patients with MPS may be associated with increased aortic stiffness.
摘要：
目的：我们旨在从心脏病学上评估粘多糖贮积症（MPS）患者大血管中糖胺聚糖（GAG）积累的后果。
方法：左心室壁厚度，评估左心室质量（LVmass）和主动脉瓣环直径（AA），主动脉窦valsalva直径(SV),窦管连接直径（STJ），收缩期主动脉直径（AD），通过除以表面积获得舒张性主动脉直径（ADd）身体指数。使用主动脉应变获得主动脉扩张性和刚度指数。射血分数,二尖瓣E和A速度，二尖瓣早期舒张组织速度(e'),E/A比，和E/E比值进行了评估。
结果：LVED-i，LVmass-i,AA-i,SV-i,STJ-i,广告-i,MPS组的ADd-i值显著高于对照组。虽然MPS组的E和E'速度和E/A比显著较低，A速度和E/E比值显著较高。而刚度指数，SBP,MPS组的PP值明显更高，主动脉应变和扩张性显著降低.刚度指数和主动脉应变之间存在相关性，扩张性,SBP,PP,和心室功能。心功能,主动脉直径,接受ERT和未接受ERT的MPS患者的主动脉弹性特征相似.
结论：在MPS组中，主动脉弹性特性受损，主动脉僵硬度增加。ERT对心功能有积极作用，主动脉直径,MPS患者的主动脉僵硬度。MPS患者的LVmass-i增加和心室几何结构受损可能与主动脉僵硬度增加有关。
方法：左心室壁厚度，评估左心室质量（LVmass）和主动脉瓣环直径（AA），主动脉窦valsalva直径(SV),窦管连接直径（STJ），收缩期主动脉直径（AD），通过除以表面积获得舒张性主动脉直径（ADd）身体指数。使用主动脉应变获得主动脉扩张性和刚度指数。射血分数,二尖瓣E和A速度，二尖瓣早期舒张组织速度(e'),E/A比，和E/E比值进行了评估。
结果：LVED-i，LVmass-i,AA-i,SV-i,STJ-i,广告-i,MPS组的ADd-i值显著高于对照组。虽然MPS组的E和E'速度和E/A比显著较低，A速度和E/E比值显著较高。而刚度指数，SBP,MPS组的PP值明显更高，主动脉应变和扩张性显著降低.刚度指数和主动脉应变之间存在相关性，扩张性,SBP,PP,和心室功能。心功能,主动脉直径,接受ERT和未接受ERT的MPS患者的主动脉弹性特征相似.
结论：在MPS组中，主动脉弹性特性受损，主动脉僵硬度增加。ERT对心功能有积极作用，主动脉直径,MPS患者的主动脉僵硬度。MPS患者的LVmass-i增加和心室几何结构受损可能与主动脉僵硬度增加有关。
