Fluid Dynamics of Coarctation of the Aorta and Effect of Bicuspid Aortic Valve

Up to 80% of patients with coarctation of the aorta (COA) have a bicuspid aortic valve (BAV). Patients with COA and BAV have elevated risks of aortic complications despite successful surgical repair. The development of such complications involves the interplay between the mechanical forces applied on the artery and the biological processes occurring at the cellular level. The focus of this study is on hemodynamic modifications induced in the aorta in the presence of a COA and a BAV. For this purpose, numerical investigations and magnetic resonance imaging measurements were conducted with different configurations: (1) normal: normal aorta and normal aortic valve; (2) isolated COA: aorta with COA (75% reduction by area) and normal aortic valve; (3) complex COA: aorta with the same severity of COA (75% reduction by area) and BAV. The results show that the coexistence of COA and BAV significantly alters blood flow in the aorta with a significant increase in the maximal velocity, secondary flow, pressure loss, time-averaged wall shear stress and oscillatory shear index downstream of the COA. These findings can contribute to a better understanding of why patients with complex COA have adverse outcome even following a successful surgery.     

成人降主动脉置换术中三种体外循环灌注技术的应用

目的总结分析成人降主动脉置换术中各种体外循环灌注的技术特点和管理方法。方法 2006年1月至2009年12月,共有60例患者行降主动脉置换手术。按体外循环灌注技术类型分为3组：左心转流组4例,股动静脉转流组37例,上下半身分别灌注组19例。三组预充均采用勃脉力A和胶体,常规加入白蛋白和激素,监测混合静脉氧饱和度和血细胞压积,积极应用超滤技术和自体血液回收技术。结果术中转流平稳,血流动力学稳定,监测指标均在正常范围,仅出现9例并发症（截瘫、偏瘫、谵妄、苏醒延迟、低氧血症）。结论成人降主动脉置换术中根据不同的手术方式,正确选择和熟练应用相应的灌注技术是决定手术成功的重要因素。     

改良Cabrol分流在主动脉夹层手术后的疗效

目的：探讨改良cabrol分流在升主动脉人工血管置换中的临床疗效。方法：收集2002年9月至2009年12月升主动脉人工血管置换术共72例,其中行改良Cabrol分流为39例（GroupⅠ）、瘤壁包裹人工血管不分流和完全剔除瘤壁不分流为31例为31例（GmupⅡ）。计算两组术后24h、48h的心包纵隔引流量并进行统计学分析,并对cabml分流的病人在术后1周和3月行心脏超声心动图检查了解是否有分流存在。结果：术后24h、48h心包纵隔引流量引流量GroupⅠ〈GroupⅡ,差异有统计学意义。cabrol分流的病人在术后1周行心脏超声心动图检查有2例存在分流信号,术后3月复查分流消失。结论：改良cabrol分流在升主动脉人工血管置换术中能显著降低术后出血量,对注射鱼精蛋白后吻合口仍有明显渗血的病例在术中应预防性采取分流术,无相关并发症发生,可取得满意的止血效果,远期效果尚待观察。     

Novel Loci for Non-Syndromic Coarctation of the Aorta in Sporadic and Familial Cases

BackroundCoarctation of the aorta (CoA) accounts for 5-8% of all congenital heart defects. CoA can be detected in up to 20% of patients with Ullrich-Turner syndrome (UTS), in which a part or all of one of the X chromosomes is absent. The etiology of non-syndromic CoA is poorly understood. In the present work, we test the hypothesis that rare copy number variation (CNV) especially on the gonosomes, contribute to the etiology of non-syndromic CoA.MethodsWe performed high-resolution genome-wide CNV analysis using the Affymetrix SNP 6.0 microarray platform for 70 individuals with sporadic CoA, 3 families with inherited CoA (n=13) and 605 controls. Our analysis comprised genome wide association, CNV burden and linkage. CNV was validated by multiplex ligation-dependent probe amplification.ResultsWe identified a significant abundance of large (>100 kb) CNVs on the X chromosome in males with CoA (p=0.005). 11 out of 51 (~ 22%) male cases had these large CNVs. Association analysis in the sporadic cohort revealed 14 novel loci for CoA. The locus on 21q22.3 in the sporadic CoA cohort overlapped with a gene locus identified in all familial cases of CoA (candidate gene TRPM2). We identified one CNV locus within a locus with high multipoint LOD score from a linkage analysis of the familial cases (SEPT9); another locus overlapped with a region implicated in Kabuki syndrome. In the familial cases, we identified a total of 7 CNV loci that were exclusively present in cases but not in unaffected family members.ConclusionOf all candidate loci identified, the TRPM2 locus was the most frequently implicated autosomal locus in sporadic and familial cases. However, the abundance of large CNVs on the X chromosome of affected males suggests that gonosomal aberrations are not only responsible for syndromic CoA but also involved in the development of sporadic and non-syndromic CoA and their male dominance.     

Paradoxical Hypertension and the Abdominal Pain Syndrome Following Resection of Coarctation of the Aorta

Sixteen children with coarctation of the aorta were studied in respect of the incidence of paradoxical hypertension and the abdominal pain syndrome after repair of the coarctation. These postoperative complications were investigated in relation to vanillylmandelic acid (VMA) excretion and extent of operative repair. The results, compared to those in a control group, indicated no evident causal relationship between these complications and catecholamine production. The severity of coarctation, the degree of correction and the operative increase in luminal surface area, however, appeared to be significant factors.     

MMP-2 Isoforms in Aortic Tissue and Serum of Patients with Ascending Aortic Aneurysms and Aortic Root Aneurysms

ObjectiveThe need for biological markers of aortic wall stress and risk of rupture or dissection of ascending aortic aneurysms is obvious. To date, wall stress cannot be related to a certain biological marker. We analyzed aortic tissue and serum for the presence of different MMP-2 isoforms to find a connection between serum and tissue MMP-2 and to evaluate the potential of different MMP-2 isoforms as markers of high wall stress.MethodsSerum and aortic tissue from n = 24 patients and serum from n = 19 healthy controls was analyzed by ELISA and gelatin zymography. 24 patients had ascending aortic aneurysms, 10 of them also had aortic root aneurysms. Three patients had normally functioning valves, 12 had regurgitation alone, eight had regurgitation and stenosis and one had only stenosis. Patients had bicuspid and tricuspid aortic valves (9/15). Serum samples were taken preoperatively, and the aortic wall specimen collected during surgical aortic repair.ResultsPro-MMP-2 was identified in all serum and tissue samples. Pro-MMP-2 was detected in all tissue and serum samples from patients with ascending aortic/aortic root aneurysms, irrespective of valve morphology or other clinical parameters and in serum from healthy controls. We also identified active MMP-2 in all tissue samples from patients with ascending aortic/aortic root aneurysms. None of the analyzed serum samples revealed signals relatable to active MMP-2. No correlation between aortic tissue total MMP-2 or tissue pro-MMP-2 or tissue active MMP-2 and serum MMP-2 was found and tissue MMP-2/pro-MMP-2/active MMP-2 did not correlate with aortic diameter. This evidence shows that pro-MMP-2 is the predominant MMP-2 species in serum of patients and healthy individuals and in aneurysmatic aortic tissue, irrespective of aortic valve configuration. Active MMP-2 species are either not released into systemic circulation or not detectable in serum. There is no reliable connection between aortic tissue—and serum MMP-2 isoforms, nor any indication that pro-MMP-2 functions as a common marker of high aortic wall stress.     

Percutaneous Aortic Valve Replacement: Emerging Tractability for Sufficient Intracardiac Resection of the Aortic Valve

OBJECTIVE:: The feasibility of endovascular resection of highly calcified aortic valves has already been demonstrated by our group. Different endovascular and intracardiac tractability methods were applied. In this study, these technologies were analyzed comparing the tractability, the resection time, and the lesions in the surrounding tissue. METHODS:: All aortic valve resections (seven human hearts and 21 porcine hearts) were performed using a Thulium:YAG laser (continuous wave, wavelength of 2.01 μm, 20 watts power rating). In the first resection system, the laser fiber was controlled by a free in-lying flexible endoscope (? 2.5 mm, length of 600 mm). The distal part of the endoscope (40 mm) was moved in one plane by proximal manual control (three degrees of freedom). The resection system was separated into defined rooms assigning one room for one tool. The fiber was controlled by the above-mentioned endoscope (*) (three degrees of freedom). The third resection system was a mechanical microactuator carrying the laser fiber (three degrees of freedom). The fourth resection system contains a rotatable inlay with defined rooms and a newly designed nitinol (NiTi) microactuator that controlled the laser fiber (four degrees of freedom). The resection time per leaflet was measured in minutes. Gross anatomy and histology in the surrounding tissue were evaluated. RESULTS:: The resection time in approaches 1, 2, 3, and 4 was 5.5 ± 2.3 minutes, 7.4 ± 2.7 minutes, ± 6.6 minutes, and2.3 ± 1.2 minutes, respectively. The gross anatomy and histology of collateral damages revealed only superficial lesions of the surrounding tissue. The amount of lesions and the resection time were lower in the fourth approach with four degrees of freedom. CONCLUSIONS:: This analysis demonstrated that a precise tractability with four degrees of freedom is necessary for a faster and safer endovascular resection of the aortic valve. The analysis will help to optimize the ongoing development of the endovascular and intracardiac resection technology.     

64层MSCT血管成像对婴幼儿主动脉缩窄及离断的评价

目的:评价64层螺旋CT血管成像在婴幼儿主动脉缩窄及离断诊断中的应用价值及其准确性.方法:对临床和超声怀疑主动脉缩窄的21例患儿行胸片、心脏大血管彩超及CT主动脉成像,其中5例行DSA检查,16例进行了手术治疗.CT血管重建技术包括VR、MIP、MPR及CPR等,对主动脉缩窄程度、长度、侧枝血管显示情况进行评价,并比较原始数据、VR、MPR、MIP及CPR几种后处理方式对主动脉缩窄诊断的准确性和敏感性.结果:21例患者中,19例为主动脉缩窄,2例为主动脉离断.病变部位于左锁骨下动脉远端、动脉导管开口附近20例(95.2%),正对左锁骨下动脉1例(4.8%).病变部位位于动脉导管近段2例,动脉导管附着处3例,动脉导管远段16例.多层螺旋CTA对主动脉狭窄诊断的总体准确率和敏感性均为100%,对主动脉缩窄的显示以靶血管VR最好,其次是CPR和MPR;对侧枝血管的显示以整体VR和CPR最好,其次是MIP和MPR;而整体VR和原始轴位图像对主动脉缩窄显示较差.结论:64层MSCT血管成像各种后处理技术相结合能为主动脉缩窄及离断患者提供了更多有价值的诊断信息,有助于临床术前评估和制定恰当的治疗方案.