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文章论述主动脉夹层(AD)、主动脉壁间血肿(出血)、主动脉穿透性溃疡的影像学诊断的进展,并讨论三者的关系,提出影像学检查在诊断和鉴别诊断中具有重要的作用。     

A larger aortic annulus causes aortic regurgitation and a smaller aortic annulus causes aortic stenosis in bicuspid aortic valve

A bicuspid aortic valve (BAV) often causes aortic stenosis (AS) or regurgitation (AR). In 54 patients with a BAV (48 +/- 16 years), transthoracic and transesophageal echo were performed to measure aortic annulus diameter (AAD), to evaluate the severity of aortic valve disease (AVD) and to calculate the area eccentricity index (AEI) of a BAV defined as a ratio of the larger aortic cusp area to a smaller aortic cusp area. By multiple linear regression analysis, the severity of AR correlated significantly with the AAD (r = 0.38) and AEI (r = 0.35) (P < 0.05) and that of AS correlated significantly with the AAD (r =-0.40) and AEI (r = 0.34) (P < 0.05). Thirty-six patients showed anteroposteriorly (A-P) located BAVs and 18 patients showed right-left (R-L) located BAVs. The AAD was larger in A-P type than in R-L type (15 +/- 3 vs 13 +/- 2 mm/BSA, P < 0.05) and there was no difference in the age and AEI between the two groups. AR was more severe in A-P type than in R-L type while AS was more severe in R-L type than in A-P type (P < 0.05). Twenty-nine patients showed raphes. The AEI was larger in raphe (+) type than in raphe (-) type (1.83 +/- 0.53 vs 1.51 +/- 0.47, P < 0.05) and there was no difference in the AAD and severity of AVD between the two groups. In conclusion, a BAV with larger aortic annulus or A-P located will tend to cause AR while a BAV with smaller aortic annulus or R-L located will tend to cause AS.     

Sheathless transcatheter aortic valve implantation via the direct aortic approach

Transcatheter aortic valve replacement (TAVR) has become an accepted alternate treatment modality to surgical aortic valve replacement in high surgical risk patients. There remains, however, a subset of patients in whom safe delivery of a TAVR device cannot be achieved by the femoral or subclavian routes. In such cases the direct aortic or transapical routes can be used though this still requires suitable anatomy. We present the first reported case of a direct aortic TAVR using a sheathless technique in order to provide sufficient distance to safely deliver the prosthesis. © 2013 Wiley Periodicals, Inc.     

主动脉瓣成形术治疗主动脉瓣反流的现状及进展

主动脉瓣成形术（aortic valve repair, AVr）保留了自体瓣膜结构的完整,具有良好的血流动力学指标,无需长期抗凝治疗,免除了人工瓣膜昂贵的经济负担,以及术后较低的瓣膜相关并发症,使得这项技术被广泛接受。AVr适合任何年龄的患者,尤其有利于青少年、孕妇及老年患者。本文将AVr治疗AI（aortic insufficiency, AI）的现状及进展做一综述。     

Asymptomatic chronic traumatic aortic valve perforation with severe aortic regurgitation

Here, we present a young asymptomatic male patient incidentally diagnosed to have aortic regurgitation (AR). The patient had a history of a blunt trauma to the thorax two years back but did never have any symptoms. Transthoracic echocardiography showed a moderately dilated left ventricle with normal systolic function and severe AR with normal nondilated aortic root and tri-leaflet aortic valve. To diagnose the etiology of the AR, a transesophageal echocardiogram (TEE) was done, which revealed a perforation in the nonadjacent leaflet (NAL) and confirmed severe AR with two AR jets being clearly visualized, one through the point of incomplete coaptation and other one through the perforated area in the NAL. The patient was treated with aortic valve replacement and was doing well on follow-up.     

Quadricuspid aortic valves

Quadricuspid aortic valves (QAV) are a rare but well recognized cause of significant aortic regurgitation. The first case was found reported in 1862. Since then there have been 110 reported cases of QAV and we report 4 more. Previously, these were diagnosed at the time of surgery or postmortem examination. With advances in echocardiography, including harmonic imaging, and also the advent of transesophageal echocardiography, more cases are being diagnosed prior to surgery. We describe four more cases, three diagnosed preoperatively and one at the time of surgery, and then review the previously reported cases. Of the 114 cases reported, 46 had the aortic valve replaced, most commonly in the 5th and 6th decade of life. Hurwitz and Roberts classified quadricuspid valves according to the size of the leaflets. It has previously been believed that QAVs with four equal sized leaflets were less likely to develop significant aortic regurgitation; however, on review of the available cases, this would not appear to be the case. The preoperative diagnosis of QAVs is important as they can be associated with abnormally placed coronary ostium. Of the 114 cases reported, there are 10 reports of abnormally placed ostia. There has been at least one reported case of death occurring because of obstruction of an abnormally placed right coronary ostium by a prosthetic aortic valve.     

二瓣化畸形加速主动脉瓣钙化的机制研究进展

主动脉二瓣化畸形是最常见的先天性主动脉瓣发育异常,发病率约0.5%-2%,易并发主动脉瓣钙化、主动脉夹层、主动脉瘤等疾病。几乎所有的该类患者在成年期后都会出现主动脉瓣钙化,且发病年龄比三瓣化主动脉瓣者提前20年。为何二瓣化畸形容易出现主动脉瓣钙化加速,其机制仍存在许多争议。目前研究认为目前研究认为异常的遗传学表达、血流动力学、炎症反应及内皮功能障碍之间的相互作用可能是重要原因,本综述将从以上几个方面对其进行讨论。     

同种异体带瓣主动脉行主动脉根部置换的临床效果

目的 :探讨应用低温保存的同种异体带瓣主动脉行主动脉根部重建手术的临床效果。方法 :85例主动脉瓣膜病变患者行同种主动脉根部置换手术 ,术后随访观察临床结果。结果 :随访 39± 13（1.5～ 91）个月。早期手术死亡率 1% （1/ 85 ） ,远期手术死亡率 6 % （5 / 85 ） ;与术前比较心功能明显改善 （P<0 .0 5 ） ;术后随访心内膜炎发生率占2 % （2 / 85 ） ;生存者中瓣膜无或有轻度返流 84 % （6 6 / 79） ,中度返流 16 % （13/ 79）。结论 :同种异体带瓣主动脉根部重建手术效果可靠 ,且并发症少。     

Leaflet entrapment causing acute severe aortic insufficiency during balloon aortic valvuloplasty

Cusp entrapment is a previously unreported mechanism of aortic insufficiency after balloon aortic valvuloplasty. In a patient who developed severe acute aortic insufficiency and expired after balloon aortic valvuloplasty, pathologic evaluation revealed a large, solitary, fractured, calcific nodule in the noncoronary cusp in which the malaligned, irregular fracture surfaces of the nodule had entrapped the leaflet in an open position. The ease with which the entrapped leaflet was reduced by manipulation on postmortem examination suggests that further manipulation of the cusp might restore valve competence in some cases when aortic insufficiency occurs after balloon valvuloplasty in calcific aortic stenosis.     

仿“Z”字主动脉窦部成形技术在急性Stanford A型主动脉夹层中的应用

目的：评估仿“Z”字主动脉窦部成形技术在急性Stanford A型主动脉夹层中的临床疗效;方法：回顾性分析自2014年9月至2018年12月在武汉亚洲心脏病医院大血管中心共收治急性Stanford A型主动脉夹层412例,其中入组125例,包含主动脉瓣重度关闭不全患者60例。男性80例,女性45例;年龄 30-77岁,平均年龄（51.9±9.37）岁。该组患者根部处理均应用仿“Z”字主动脉窦部成形技术,远端半主动脉弓置换15例,全主动脉弓置换110例。全主动脉弓置换患者均置入“象鼻”支架。结果：全组体外循环时间（170±41.2）min;主动脉阻断时间（130.1±30.6）min;深低温停循环时间（25.1±5.9）min。术后随访1.5月—4.3年,术后所有患者主动脉瓣返流程度均为0-1级。 术后经食道超声检查即刻主动窦部残余夹层3例,分别随访1.5年、2.5年和3.0年,未形成动脉瘤,主动脉窦部直径分别为4.3cm、4.2cm和4.5cm。余患者随访期间未发现因主动脉瓣返流及窦部情况再次手术。结论;仿“Z”字主动脉窦部成形技术在急性Stanford A型主动脉夹层中的临床疗效较好,手术操作简单、易掌握、安全有效、易开展。尤其在合并主动脉瓣反流的患者中,避免了换瓣手术,缩短了手术时间,提高了患者的生活质量。     

Biomarkers in acute aortic dissection and other aortic syndromes

Acute aortic syndromes have an incidence of >30 per million per annum and a high mortality without definitive treatment. Survival may relate to the speed of diagnosis. Although pain is the most common symptom, there is a large fraction of patients in whom the diagnosis may be mistaken or overlooked. Currently, a high index of clinical suspicion is the chief prompt that diverts a patient into a definitive algorithm of imaging investigations. Although there is no point-of-care biochemical test that can be reliably used to positively identify dissection, biomarkers are available that could accelerate the diagnostic pathway and thereby expedite treatment.     

Prolapse of the "floppy" aortic valve as a cause of aortic regurgitation. A clinico-morphologic study

A clinico-pathologic study was performed in 25 patients undergoing aortic valve replacement because of regurgitation, caused by myxoid degeneration of the valve leaflets. Associated cardiac anomalies were floppy mitral valve (2 cases), floppy mitral valve and idiopathic hypertrophic subaortic stenosis (1), left atrial myxoma (1), and aortic coarctation at the isthmus (1). Three patients died (2 immediately and 1 on the 30th postoperative day). Pathological studies of the explanted valves showed deformities characterized by redundant thin leaflets which appeared soft and gelatinous. On histologic examination the fibrous layer of the leaflets was seen to be infiltrated by myxomatous tissue. Echocardiography showed the aortic root to be dilated in 13 patients and normal in the others. In those with normal aortic root, the histological examination of aortic wall disclosed minimal cystic medial necrosis in two cases. In contrast, more severe forms of cystic medial necrosis were evident in all patients having a dilated aortic root. Aortic valve replacement was performed in all cases. It was accompanied by a Bentall procedure (1 case), repair of ascending aorta dissection (2), replacement of the ascending aorta (1), mitral valve replacement (2), mitral valve replacement and apico-ascending aorta conduit (1) and excision of a left atrial myxoma (1). Our experience suggests that prolapse of the aortic valve due to floppy leaflets is a common degenerative disease which is generally associated with noninflammatory aortic root degeneration. This, together with aortic root dilatation, contributes to valve insufficiency. Nevertheless, the disease, when isolated (with normal aortic root), is liable in itself to produce aortic regurgitation. The need for early diagnosis is stressed, so as to be able to perform valve replacement.     

主动脉瓣成形术于A型主动脉夹层治疗中的应用△

目的总结采用主动脉瓣成形术治疗主动脉夹层撕裂引起的主动脉瓣反流（aorticregurgitation,AR）的经验及术后随访分析。方法分析2007年3月至2011年9月广东省人民医院收住的100例因主动脉夹层引起的AR,采用主动脉瓣成形术处理纠正AR的患者的资料,并对术后患者进行门诊随访,了解术后病情变化。所有患者病因均排除马凡氏综合征。结果住院死亡5例。随访（110＋39）周,术后1周、3个月及9个月患者AR面积、左心室舒张末期内径（1eftventficularenddiastolicdimension,LVEDd）、左心室收缩末期内径（1eftventricularend-systolicdimension,LYESd]、左心室射血分数（1eftventricularejectionfraction,LVEF）均较术前有明显改善,差异有统计学意义（P〈O．05）。术后1周、3个月及9个月后患者AR面积、LVEDd、LVESd及LVEF两两比较,差异无统计学意义（P〉0．05）。1例患者术后2年因AR加重而再次返院行主动脉瓣置换术,余患者门诊随访效果良好。结论主动脉瓣成形术是治疗主动脉夹层撕裂引起的AR有效的手术方法,且效果稳定。