法洛四联症病儿右心室结构和功能与基质金属蛋白酶的关系.docVIP

法洛四联症病儿右心室结构和功能与基质金属蛋白酶的关系 【关键词】 法洛四联症;心肌;胶原;基质金属蛋白酶类 　　［摘要］目的 探讨法洛四联症（TOF）病儿右心室结构和功能与心肌胶原代谢和基质金属蛋白酶（MMPs）的关系。方法 应用改良BERGMAN法测定23例TOF和18例室间隔缺损（VSD）病儿右心室和右心耳心肌胶原的含量，同时应用RT-PCR技术测定右心室和右心耳心肌组织MMPs mRNA的表达。应用放免法测定血清Ⅲ型胶原氨基末端肽（P-Ⅲ-NP）和Ⅰ型胶原羧基末端肽（P-ⅠCP）的浓度，应用SONOS-5500彩色多普勒超声仪测定TOF病儿右心室内径（RVD）、右心室前壁厚度（RVAW）、右心室舒张早期与舒张晚期峰值血流充盈速度的比值（E/A）。结果 TOF病儿右心室心肌胶原含量、血清P-Ⅲ-NP和P-ⅠCP浓度明显高于VSD组和健康组儿童，右心室心肌组织MMP-1、MMP-2和MMP-9 mRNA的表达较VSD组明显下调。TOF病儿右心室心肌胶原含量、血清P-Ⅲ-NP和P-ⅠCP浓度与E/A呈负相关，与RVD和RVAW呈正相关;TOF病儿右心室心肌组织MMP-1、MMP-2和MMP-9 mRNA的表达与E/A、RVD和RVAW呈正相关。结论 TOF病儿右心室结构和功能与心肌胶原代谢和基质金属蛋白酶的表达密切相关。 　　［关键词］ 法洛四联症;心肌;胶原;基质金属蛋白酶类 　　［ABSTRACT］ObjectiveTo explore the relationship between the structure and function of right ventricle and matrix metal-loproteinases （MMPs） in children with tetralogy of FALLOT （TOF）. MethodsThe contents of myocardial collagen of right ventricle and right auricle were measured in 18 children with ventricular septal defect （VSD） and 23 with TOF using modified Bergmans method; and the myocardial MMPs mRNA expression was determined by RT-PCR. The concentrations of P-Ⅲ-NP and P-ⅠCP were detected by radioimmunoassay. The internal diameter of right ventricle （RV）， the thickness of the anterior wall of RV， and the ratio of early and late diastolic blood flow filling velocity of RV （E/A） were measured by SONOS-5500 echocardio-graphy. ResultsThe contents of myocardial collagen of RV and the serum concentration of P-Ⅲ-NP and P-ⅠCP in TOF group were higher than those of VSD group and healthy children group. The myocardial mRNA expression of MMP-1， MMP-2， and MMP-9 of RV in TOF group were lower than those of VSD. The content of cardial collagen of RV and serum P-Ⅲ-NP and P-I CP in TOF were negatively correlated with E/A， but positively correlated with IDRV and thickness of anterior wall of RV. The myo-cardial mRNA expression of MMP-1，MMP-2， and MMP-9 of RV in TOF group were positively related with E/A ， RVD and thick-ness of anterior wall of RV. ConclusionThe structure and funct