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房间隔缺损右心功能的评估及先天性心脏病伴肺动脉高压发病机制的探讨

The Evaluation of Right Ventricular Function in Patient with Atrial Septal Defect and to Research the Pathogenesis of Secondary Pulmonary Hypertension in Patient with Congenital Heart Disease

【作者】 丁建东

【导师】 陆凤翔; 许迪;

【作者基本信息】 南京医科大学 , 内科学, 2003, 博士

【摘要】 研究背景 房间隔缺损(Atrial septal defect,ASD)是最常见的先天性心脏病(Congenital heart disease,CHD)之一,其发病率约占整个先心病的10%~30%,以女性多见。ASD患者长期左向右分流可导致右心容量负荷过重、肺动脉高压、充血性心力衰竭及房性心律失常。右心功能的定量评估对于患者病情估价、治疗决策、疗效和预后判断均具有十分重要的价值。然而,右心功能的评价较困难,心室造影获得心功能虽准确,但由于是创伤性的,在临床应用上受到限制。CT和磁共振亦为无创性评价右心容量和射血分数的方法,但其价格昂贵、采集时间长、并需要复杂的采集技术、器械不易移动,使其在临床应用上也受到限制。超声心动图是一种无创性检查,是评价心功能的重要手段,但由于右心室是一种不规则的新月型结构,具有一个相对独立的流出道,肌小梁粗大,对其难以进行标准的几何学假设,故用传统的二维超声心动图(Two-dimensional echocardiography,2DE)难以进行精确的右心功能评价。随着不基于几何构形假设的超声心动图检测方法如三维超声心动图(Three-dimensional echocardiography,3DE)、心肌运动指数(Myocardial Performance index,MPI)、声学定量(Acoustic quantification,AQ)等的应用,从而可较准确的测定右心功能。 大量临床资料证明,ASD患者的并发症随年龄增大而逐渐加重与 南京医科大学博士学位论文恶化,既往早期手术治疗是其预防严重并友症发生的唯一的治疗方法。目前临床广泛采用的经皮穿刺ASD封堵术能有效地纠正。。脏的解剖畸形,减少左向右分流,必将对心脏的右心功能产生有利的影响。 另 卜,肺动脉高压(Pulmon叼 hypertension,PH)是左向右分流型CHD如ASD常见的并发症之一,PH的存在与否及其严重程度与先心病治疗决策、手术疗效及临床预后密切相关,但先心病合并肺动脉高压的发病机理仍不清楚,尚待进一步研究和探讨。 目 的 门)应用离体新鲜猪心模型,评价超声三维重建定量测定右。。室腔容量的准确性。 (2)应用3DE评价ASD患者右。G容量和功能及经皮穿刺ASD封堵术后右。C客量和功能的变化。 门)应用多普勒超声检测右心室MPI评价ASD患者右心功能及经皮穿刺ASD封堵术后右心功能的变化。 k)探讨CHD继发PH患者血浆内皮素门T上)、心钠素(AN’P)浓度的变化及其临床意义。 方 法 门)离体新鲜猪。C 15只,采用三维超声测量其右。。室腔容量,并与实测值比较。 门)ASD患者 58例,男 24例,女34例,年龄4-67岁,平均28.91 17.12岁,与之性别、年龄相匹配的正常对照组3Z例(男18例,女14例,年龄4-竹岁,平均24.75 t 12.co岁),应用3DE, 5g 南京医科大学博士学位论文 测量右。U Y舒张末期容量(ight ventricular end-dlastollc volume, RVEDV)、收缩末期客量(ight ventricular end-systollc volume, RVESV)、并计算右窒射血分数(ugM ve血i叨far gectlon fraction, RVEF)。其中 23例患者成功地施行了经皮穿刺 ASD封堵术,男N 例, 女 12例,年龄 6-57岁,平均 25.70 13.31 岁,均为继发孔型 ASD, ASD直径(球囊测量值)为 9-36(25*7 i 7.50)mm,均置入 Amplatzer 封堵器。所选封堵器大小为 11-40mm。分别于术前、术后 3d、l月、 3月检查测量RVESD,RVESV、并计算RVEF。观察经皮穿刺ASD封 堵术后ASD患者右。G容量及右心功能的变化。 门)ASD组共68例,男30例,女38例,年龄4-67岁,平 均ZS.79 t 17.SZ岁,与之性别、年龄相匹配的正常对照组sl例(男 41例,女40例,年龄3-65岁,平均29.02 L 14.22岁)8用多普勒 超声,C动图检查时,记录三尖瓣舒张期和右心室流出道收缩期脉冲多 普勒血流频谱,测量右心室WI,MPI 为心室等容舒张期时间 (Isovolumic relaxation time,I肚)及等容收缩期时间(Isovolumic co血附ion time,ICT)r和与。u室射血时间(Ejection time,ET)的比 值。其中 23例患者成功地施行了经皮穿刺 ASD封堵术,男 11例, 女12例,年龄6-57岁,平均25.70 L 13.引 岁,均为继发孔型ASD, ASD直径(球囊测量值伪 9-36(25*7 i 7.50)mm,均置入 inplatzer 封堵器。所选封堵器大小为 11—40mm。分别于术前、术后 3d、l月、 3月检查测量测量右心室Nrpl。观察经皮穿刺ASD封堵术后ASD患 者右。G星 MPI的变化。

【Abstract】 Atrial septal defect (ASD) is one of most common congenital heart disease (CHD). It accounts for 10%~30% of all CHD and is common in women. Long-term left to right shunt in ASD leads to the development of right heart volume overload, secondary pulmonary hypertension, congestive heart failure and atrial arrhythmias. The quantitative evaluation of right ventricular (RV) function has a very important significance in evaluating the patient’s conditions, determinating therapeutic methods and assessing the curative effect and prognosis of the patients. However, the quantitative evaluation of RV function is very difficult, for many years, angiography has been the clinical standard for determining RV volume, but due to its invasiveness, its use has been restriction. Recently, compute tomography (CT) and magnetic resonance imaging (MRI) has been validated as an accurate technique for determining RV volume. However, the high cost, long examination time, complex image acquisition technique and immobility of CT and MRI instrumentation limit the extensive use of these techniques in clinical practice. Compared with these techniques,echocardiography has the advantages of lower cost, better portability, high temporal resolution, and allows accurate estimation of heart dimensions. Similar to angrography, two-dimensional echocardiography relies on geometric assumptions for determining ventricular volume. However, the right ventricule has a crescentric shape, with a separate infundibulum and prominent trabeculations, it is difficult to describe the right ventricule by a simple geometric figure and therefore, difficult to assess its function in a conventional two-dimensional echocardiography. Recently, progress has made in accurate assessing RV function with application of echocardiography (three-dimensional echocardiography, myocardial performance index, acoustic quantification) independent of geometric assumptions.It has been proved by numerous clinical studies that the elder the patient is, the worse of the complications in patients with ASD is more common. As before operation was its sigulare therapeutic method, at present, transcatheter closure of ASD has many reports. It corrects anatomic anomaly of heart, decreases left to right shunt and will give rise to affect the RV function.Pulmonary hypertension (PH) is one of the most common complications in patients with CHD, and associates closely with therapeutic method, surgical effectiveness, clinical prognosis. But the mechanism PH due to CHD is not clear. Studies about it needs to gostep further. Objectives(1) To assess the accuracy of three-dimensional echocardiography in the quantitative measurement of RV volume by using excised fresh pig heart model.(2) To evaluate RV volume and function and the its change in patients with ASD after transcatheter closure of ASD by three-dimensional echocardiography(3) To evaluate RV function and the its change in patients with ASD after transcatheter closure of ASD by using Doppler echocardiography to measure RVMPI(4) To research the change and clinical significance of plasma endothelin-1 (ET-1) and atrial natriuretic peptide (AMP) concentrations in patients with congenital heart disease (CHD).Methods(1) In 15 excised fresh pig heart model, three-dimensional echocardiography was used to reconstruct the right ventricular cavity, and compare it with actual volume.(2) Three-dimensional echocardiography was performed in 58 patients with ASD (24 men, 34 women; mean [ ± SD] age 28.91 ± 17.12, range 4 to 67) and 32 sex-matched, age-matched healthy people (control group) (18 men, 14 women; mean [ ± SD] age 24.75 ± 12.00, range 4 to 45)to calculate RVEDV(Right ventricular end-diastolic volume), RVESV(Right ventricular end-systolic volume) and RVEF (Right ventricular ejection fraction). Among patients with ASD, 23 patients( 11 men, 12 women; mean [±SD] age 25.70± 13.31, range 6 to 57) were diagnosed to secundum ASD (the stretched diameters of ASD were from 9

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