Association between bicuspid aortic valve morphotype and regional dilatation of the aortic root and trunk

Thoracic aortic disease, including thoracic aortic aneurysm (TAA), is frequently seen in patients with bicuspid aortic valve (BAV). We hypothesized that BAV morphotype would be associated with aortic aneurysm phenotypes but that other patient variables would significantly modify this relationship. 829 patients between 18 and 90 years with BAV and available raw imaging of the aortic valve and the ascending aorta to its mid-portion prior to aortic valve and aortic surgery were examined. The sinuses of Valsalva and proximal ascending aorta were measured from 2-dimensional co-planar echocardiographic images. We observed strong associations between patient habitus and raw and normalized dimensions of the aortic root and ascending aorta. Patients with R–L morphotype presented at an older age with larger aortic root but similar ascending aortic dimensions. After accounting for patient morphometric characteristics and severity of aortic valve disease, patients with R–L valve morphotype were marginally more likely to have an aortic root aneurysm (86% vs. 78%; P?=?0.043), defined as aortic root dimension Z score ≥3. We observed only small differences in aortic dimensions between BAV morphotypes, that are eclipsed by variation in patient habitus. We interpret these findings to mean that BAV patients will not likely benefit from therapies based on aortic valve morphotype. Rather, we propose that all BAV patients should undergo longitudinal follow-up, independent of valve morphotype. Guidelines for aortic surgery based upon dimensions alone may be improved by considering patient characteristics such as age, body size and other characteristics.     

Aortic valve replacement: technique and outcome with artificial heart valves and allografts

Prosthetic aortic valve replacement has become a highly effective surgical treatment for aortic valve stenosis and aortic valve incompetence. After a properly timed aortic valve replacement, age- and gender-related actuarial survival can be similar to those of a normal healthy population, if serious late valve-related complications do not occur. The perioperative risk of isolated aortic valve replacement approaches 1% to 2% mainly influenced by valve- and patient-related factors such as age, left ventricular function, New York Heart Association class, preoperative hemodynamic and time of operation. Despite a number of various mechanical, bioprosthetic and biological heart valve prostheses, aortic valve replacement remains a palliative surgical treatment. Life-long anticoagulation is required by patients with mechanical heart valve prostheses, and, degeneration and subsequent reoperation is inherent in all bioprosthetic devices. The availability of cryopreserved allograft is limited and the Ross-operation is a technically demanding procedure with a somewhat higher perioperative mortality. Perioperative morbidity after isolated aortic valve replacement is low. A fatal neurological event, the most dreaded complication after aortic valve replacement, is found in about 0.5% of patients. Post-operatively, left ventricular hypertrophy decreases and cardiac dysfunction, reflected by the left-ventricular ejection rate is reversed in most patients, associated with an excellent clinical outcome and an age- and sex-specific normal long-term survival. To offer the most appropriate valve substitute to the individual patient, the debate focus on the question of whether patients benefit from durable mechanical heart valves or a primary anticoagulation-free bioprosthesis. However, there is no difference found between the prostheses types with regard to serious valve related complications such as major thromboembolism and hemorrhage, fatal reoperation and valve-related mortality. The search for an ideal permanent substitute for the aortic valve continues. To date, proper indication, optimal timing of the operation and careful analysis of the late results may help to improve the long-term survival of patients with after isolated aortic valve replacement.     

Transcatheter Aortic Valve Implantation Three-Dimensional Echo Monitoring and Guidance

Transcatheter aortic valve implantation (TAVI) was first performed in 2002. With the Edwards-Sapien valve, more than 20,000 to 25,000 TAVI procedures have now been performed worldwide. In this paper, we share our experience using live three-dimensional (3D) transesophageal echocardiography (TEE) to guide and monitor TAVI. At our medical center, we have performed more than 150 transfemoral TAVI procedures. Three-dimensional TEE is useful for precise measurement of the aortic annulus in multiple planes, confirming aortic valve stenosis severity and morphology, monitoring of the position of guidewires and catheters, identifying the ventricular and aortic edges of the valve stent, and optimizing bioprosthetic valve positioning. Post-deployment 3D TEE is also used to assess the severity of residual aortic regurgitation as well as to identify potential complications of the procedure including valve malpositioning.     

Quadricuspid aortic valve abnormality associated with aortic stenosis and aortic insufficiency.

Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. Transthoracic 2-dimensional echocardiography can delineate aortic valve and diagnose most quadricuspid aortic valves. This article is a report of a quadricuspid aortic valve associated with aortic stenosis and aortic insufficiency diagnosed by transthoracic echocardiography.     

先天二叶式主动脉瓣畸形临床及超声心动图特征分析

目的:探讨先天二叶式主动脉瓣畸形(BAV)患者主动脉瓣功能异常和大血管病变的分布特征和危险因素。方法:对复旦大学附属中山医院2012年1月至2015年12月确诊为BAV患者的超声心动图数据进行横断面分析。结果:共纳入BAV患者2781例,占超声心动图检查总人数的1.1%,其中70.2%为男性,60.4%伴中度以上主动脉瓣功能异常,54.2%合并升主动脉和19.7%患者主动脉窦部扩张。单纯主动脉瓣狭窄和混合性双病变的检出率随年龄增长递增(P<0.0001);单纯主动脉瓣反流的检出率随年龄增长递减(P<0.0001)。男性、左右冠瓣融合型、单纯主动脉瓣反流为患者发生主动脉窦部扩张的独立危险因素;年龄、单纯主动脉瓣狭窄和主动脉瓣混合性双病变为患者发生升主动脉扩张的独立危险因素。结论:BAV发病率高且临床表现多样,以主动脉瓣功能异常和升主动脉扩张最常见,其表型差异对治疗有指导意义。     

The role of multimodality imaging in the selection of patients for aortic valve repair

Aortic valve sparing surgery for aortic regurgitation and/or aortopathy serves as an alternative to aortic valve and root replacement. One of the advantages of aortic valve sparing surgery over conventional replacement is that there is no need for life-long anticoagulation, which is particularly attractive in young patients who may receive a mechanical prosthesis otherwise. However, successful aortic valve repair requires high expertise. At present, reparability is determined intraoperatively by direct surgical inspection. Preoperative imaging techniques might improve the patient selection for aortic valve repair. The mechanism of aortic regurgitation, aortic valve morphology and calcification and aortic root dimensions are all of importance when aortic valve repair is considered. The present review focuses on the role of imaging techniques in determining aortic valve reparability.     

First-in-man implantation of the retrievable and repositionable VenusA-Plus valve

BACKGROUND: No retrievable and repositionable second generation transcatheter aortic valve is available in China. Here, we report the first-in-man implantation of the retrievable and repositionable VenusA-Plus valve.METHODS: A 76-year-old patient with symptomatic severe aortic stenosis and high surgical risk (STS 13.8%) was recommended for transcatheter aortic valve replacement (TAVR) by heart valve team. Type 0 bicuspid aortic valve with asymmetric calcification was identified by dual source computed tomography, and the unfavorable anatomies increased the possibility of malposition and paravalvular leakage during TAVR. Therefore, we used the retrievable and repositionable VenusA-Plus valve for the patient.RESULTS: Transfemoral TAVR was performed under local anesthesia with sedation, and a 26-mm VenusA-Plus valve was successfully implanted. No transvalvular pressure gradient and trace paravalvular leakage were found.CONCLUSION: The successful first-in-man implantation indicates the retrievable and repositionable VenusA-Plus valve is feasible in complicated TAVR cases such as bicuspid aortic valve.     

Balloon valvuloplasty of aortic valve stenosis in childhood: early and medium term results

BACKGROUND: Isolated aortic valve stenosis in childhood is treated by balloon valvuloplasty. The role of independent risk factors for the outcome remains unclear. MATERIAL AND METHODS: We analysed the early and medium term outcome of balloon valvuloplasty in isolated aortic valve stenosis in 44 pediatric patients with isolated, severe aortic valve stenosis at an age younger than 18 years, who received a primary balloon valvuloplasty during the last 5 years in our institution. We evaluated the type of aortic valve morphology, age, clinical status, and myocardial function at the time of the intervention as independent risk factor. RESULTS: A significant early relief of the pressure gradient across the aortic valve (P < 0.001) after balloon valvuloplasty was found. This was independent of the aortic valve morphology. Two neonates with a highly stenotic tricuspid aortic valve and severely compromised haemodynamics died within the first 30 days after the intervention. During medium term follow up (mean 22.5 months) we observed a functional deterioration for the stenosis as well as for the insufficiency of the aortic valve. "Symptoms before intervention" is an independent risk factors (P < 0.001) for valvuloplasty failure. Patients at an "age at intervention 相似文献   

主动脉瓣病变合并升主动脉扩张的外科治疗

目的探讨主动脉瓣病变合并升主动脉扩张的外科处理方式。方法按照主动脉瓣瓣叶情况和外科处理方式将患者分组,利用心脏彩超随访测量不同方式处理后的升主动脉变化情况。结果三叶主动脉瓣行单纯置换瓣膜者的升主动脉年平均减少0.471 mm,同期行升主动脉成形者的升主动脉年平均减少0.27 mm;二叶主动脉瓣患者单纯置换瓣膜者其升主动脉年平均增加2.28 mm,同期行成主动脉成形者其升主动脉年平均增加2.923 mm。结论二叶主动脉瓣患者术后升主动脉扩张明显。二叶主动脉瓣患者术前升主动脉40 mm宜进行置换手术,三叶主动脉瓣患者应谨慎同期处理升主动脉。     

Intraoperative transesophageal echocardiographic assessment of acute prosthetic aortic valve regurgitation after mitral valve replacement: Value of the deep transgastric long-axis view

The transesophageal echocardiographic assessment of prosthetic aortic valve function is made more difficult by the presence of a mechanical mitral valve prosthesis because echocardiographic views conventionally used to assess the aortic valve function are obscured by acoustic shadowing and artifacts. We report the use of intraoperative transesophageal echocardiography in a patient who developed severe prosthetic aortic valve regurgitation after implantation of a mechanical mitral valve, in whom conventional multiplane midesophageal views failed to reveal aortic regurgitation owing to acoustic shadowing and artifacts from the prosthetic mitral valve. We report the value of the deep transgastric long-axis view of the aortic valve that provided an unobstructed view of the left-ventricular outflow tract, and clearly demonstrated severe aortic regurgitation as a result of interference with the prosthetic aortic valve mechanism by the implanted mitral valve prosthesis. This case also emphasizes the importance of a comprehensive intraoperative transesophageal examination, including that of surrounding structures, to detect iatrogenic complications during mitral valve replacement.     

Hemodynamic effects of aortic valve and heart rate on coronary perfusion

BackgroundReduced coronary flow reserve in aortic stenosis and after transcatheter aortic valve implantation is usually attributed to physiological factors taking place during systole, such as an increase in coronary resistance, and backward waves intensity. In this paper, we suggest an additional factor related to the diastolic hemodynamics in the aortic root.MethodsWe measured left ventricle, aortic and coronary pressure and coronary perfusion in in-vitro models of healthy, aortic stenosis and an artificial valve at different heart rates and cardiac output conditions, to isolate the effect of hemodynamic factors in the aortic root during diastole.FindingsOur results show that during diastole, coronary perfusion depends on the pressure gradient between the aorta and the coronary inlet. This aorta-coronary pressure gradient is influenced by the hemodynamic flow field in the aortic root. The ratio between the aorta-coronary pressure gradient magnitude in stress to that under rest conditions of a healthy model is ten times higher than the same ratio in the aortic stenosis model and twice higher as compared to the artificial valve model result. The coronary flow reserve of the healthy model is correspondingly higher compared to the artificial valve and the aortic stenosis models. These results are in agreement with the clinical evidence.InterpretationThis study supports the hypothesis of a hemodynamic mechanism in the aortic root that increases coronary flow during rest but reduces the coronary flow reserve in aortic stenosis and artificial valve cases. The results may provide valuable insights regarding valve design.     

Calcific aortic valve disease: imaging studies and therapeutic interventions.

Echocardiography is the predominant imaging method used for patients with aortic valve disease because of its excellent diagnostic accuracy, high reproducibility and noninvasive nature. Cardiac catheterization is typically reserved for patients in whom the diagnosis remains unclear, those requiring coronary angiography prior to valve replacement, and in the setting of complex valve disease. Cardiac computed tomography (CT) has recently been applied as a research tool to quantify the amount of aortic valve calcium (AVC), which has served as a clinical end point in several medical therapy trials. Medical therapy for aortic valve disease remains an active area of clinical research. Multiple retrospective studies have shown a benefit for 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (HMG-CoA reductase inhibitors or statins) in reducing disease progression. However, two recently completed prospective, randomized trials yielded conflicting results. The data for using angiotensin converting enzyme (ACE) inhibitors are in the preliminary stages. This review will focus on imaging methods that are available for patients with aortic valve disease and summarize the recent trials that have evaluated medical therapy aimed to reduce progression of aortic valve disease.     

主动脉瓣二叶式畸形及合并升主动脉瘤样扩张的外科治疗

目的：探讨主动脉瓣二叶式畸形（BAV）及合并升主动脉瘤样扩张的外科治疗方法。方法：2000年1月—2009年4月对28例主动脉瓣二叶式畸形患者行手术治疗,其中6例合并升主动脉瘤样扩张。其中,男性22例,女性6例,年龄23～65岁。心功能II级16例,III级9例,IV级3例。行主动脉瓣置换22例,行主动脉瓣置换和升主动脉置换（Wheats手术）2例,行主动脉瓣置换和外用Dacron人工血管展开后包绕升主动脉成形1例,3例应用带瓣管道行Bentall手术。同期行二尖瓣环置换术3例,三尖瓣成形术6例。结果：术后早期死亡1例,为Bentall手术后死于纵隔感染。术后早期并发低心输出量综合征6例,呼吸功能不全6例。痊愈出院27例,随访3个月～2年,无晚期死亡病例。结论：先天性主动脉瓣二叶式畸形可致主动脉瓣关闭不全或狭窄,主动脉瓣置换术是常用的手术方法。BAV合并升主动脉瘤样扩张要根据不同的病情作不同手术,可获得良好的结果。     

ROSS手术的护理配合

ROSS手术又称为自体肺动脉瓣移植术，它是心脏瓣膜置换术的一种。由于主动脉瓣关闭不全和／或狭窄而造成血液返流，所以用病人本身的肺动脉瓣代替病变的主动脉瓣。因为主动脉瓣在器官、细胞、组织的营养供应上起重要作用，氧含丰富的血液泵出心脏通常通过主动脉瓣泵出主动脉而到身体其他部位。如果主动脉瓣病变，那么身体其他部位会因缺氧而造成损害。用病人本身健康的肺动脉瓣代替病变的主动脉瓣，病人的肺动脉瓣需要用代替品。在同一手术中病人的肺动脉瓣用来自供体的健康瓣膜代替。ROSS的最大优点是新的主动脉瓣会和病人本身一起生长，这对孩子来说是很重要的。尽管ROSS手术有一定难度，但最近研究表明ROSS手术是一种临床疗效好的矫治主动脉瓣疾病的手术方法。     

Beh?et's aortitis and aortic regurgitation: a report of two cases.

Beh?et's disease is a rare systemic vasculitis that may lead to neurologic complications and rare manifestations of aortitis and aortic regurgitation. We report 2 cases of Beh?et's aortitis and aortic regurgitation. The first patient presented with acute stroke. Recognition of acute aortitis on echocardiography led to the diagnosis of vasculitis as the cause of the cerebral event. This case highlights the echocardiographic features of aortic root pathology from acute aortitis to subsequent aortic valve perforation. In both cases, severe aortic regurgitation necessitated aortic valve replacement. Both were complicated by valve dehiscence requiring reoperation, illustrating the postoperative morbidity in this inflammatory condition.     

同种异体人心脏主动脉与肺动脉瓣膜的生物力学比较

背景:目前同种心脏瓣膜已成熟应用于瓣膜替换手术及复杂先天性心脏病矫治手术中,但人心脏肺动脉瓣膜与主动脉瓣膜生物力学特性的对比研究报道较少.目的:对比分析深低温液氮保存人心脏主动脉瓣膜和肺动脉瓣膜的生物力学特性.方法:对比观察试验于2004-03/07在中国医学科学院心血管病研究所先天性心脏病研究室完成.取深低温液氮保存的健康成年男性脑死亡6 h内志愿捐献者的人心脏主动脉瓣膜和肺动脉瓣膜各6例,符合医学伦理学要求.应用HD-10型厚度仪测定瓣膜组织的厚度,应用Instron拉伸机测定周向瓣膜组织的生物力学参数:瓣膜组织的断裂强度、应力应变曲线的斜率及伸长比.结果与结论:与人主动脉瓣膜相比,人肺动脉瓣膜组织厚度明显变薄;但二者的乍物力学参数瓣膜组织的断裂强度、应力应变曲线的斜率及伸长比差异均无显著性意义(P>0.05).结果提示,人主动脉瓣膜与肺动脉瓣膜的生物力学特性基本相似,从瓣膜水平上分析,人肺动脉瓣膜可满足主动脉瓣位生物力学的需要.     

Prospective study of routine perioperative transesophageal echocardiography for elective valve replacement: clinical impact and cost-saving implications.

Transesophageal echocardiography (TEE) is widely used during heart valve replacement operations, but its clinical impact and cost-saving profile have not been studied prospectively for this indication. We investigated the clinical benefits and cost-savings of routine TEE for elective valve replacement at a regional tertiary center. We prospectively studied 300 patients (140 men; mean age [+/-SD], 66 +/- 9 years) undergoing aortic valve, mitral valve, or double-valve replacements. Transesophageal echocardiography with a biplane (in 161 patients) or a multiplane probe was performed before and after surgery. We assessed whether the TEE findings changed the operation or the postoperative treatment and the cost of TEE either as an extension of a preexisting service or as a new development. In 2 patients undergoing aortic valve replacement, significant mitral regurgitation on TEE led to additional mitral valve replacement, and in 1 patient undergoing mitral valve replacement, aortic regurgitation also required aortic valve replacement. Immediate reoperation (dehisced mitral valve prosthesis) and delayed extubation (suspected obstruction of an aortic valve prosthesis) were prompted by postoperative TEE. Extending an existing TEE service to routine intraoperative use saved up to $109 (US) per patient per year. Routine intraoperative TEE can provide major clinical benefit to a small proportion of patients undergoing elective valve replacement, and this can lead to cost savings, but only if the service can be provided without major capital investment.     

超声心动图评价二叶主动脉瓣畸形与升主动脉扩张的关系

目的　使用超声心动图评价二叶主动脉瓣畸形 (BAV)与升主动脉扩张的关系。方法　使用二维超声在四个部位检测 42例主动脉瓣功能正常的BAV患者 (病例组 1)、3 7例主动脉瓣狭窄的BAV患者 (病例组 2 )和 40例年龄、体表面积、血压等匹配的健康人 (对照组 )的升主动脉内径 :瓣环、瓦氏窦、主动脉上嵴及升主动脉近端。结果　病例组 2升主动脉瓦氏窦、主动脉上嵴及近端内径最大 ,其次为病例组 1(P <0 .0 5～P <0 .0 0 1)。但病例组 1中有一部分BAV患者的升主动脉内径与对照组无显著差异。结论　BAV常合并升主动脉扩张 ,提示两者可能为同一发育异常 ,即先天性主动脉壁发育缺陷的不同表现形式。     

Ultrasound evaluation of aortic valve anatomy in the fetus.

OBJECTIVE: To assess the feasibility of ultrasound identification of aortic valve anatomy in the fetus, with particular emphasis on the detection of bicuspid aortic valve. METHODS: This study was a prospective analysis of 21 fetuses with prenatally diagnosed congenital left heart obstructive lesions and 45 normal fetuses undergoing routine ultrasound evaluated at a tertiary referral center. These fetuses underwent detailed echocardiography, including the study of the aortic valve on a targeted short-axis view of the right ventricle. Necropsies or postnatal echocardiograms were available for confirmation of the diagnosis in all cases. RESULTS: Aortic cusps and commissures were satisfactorily visualized in 38/45 (84%) normal fetuses and in 18/21 (86%) fetuses with congenital heart disease. The aortic valve was correctly defined as bicuspid in one normal fetus and in six fetuses with congenital heart disease. In two fetuses with a positive family history, the bicuspid aortic valve was isolated. There was one incorrect diagnosis (a unicuspid unicommissural valve diagnosed prenatally as a bicuspid aortic valve in a fetus with severe aortic stenosis) and one false-positive diagnosis in a fetus diagnosed with a coarctation and a bicuspid aortic valve late in the third trimester of pregnancy and in which both anomalies were not confirmed at neonatal echocardiography. CONCLUSIONS: This study demonstrated that aortic valve anatomy can be satisfactorily assessed in fetuses with and without left heart obstructive lesions. We believe that a detailed search for a bicuspid aortic valve should be attempted in all patients referred for a positive family history of congenital heart disease, in general, and of left ventricle outflow tract obstruction or bicuspid aortic valve, in particular. In fact, the presence of an asymptomatic bicuspid aortic valve has been demonstrated to represent an important factor predisposing to the development of bacterial endocarditis and dissecting aortic aneurysm late in adult life. Therefore, an early detection of such an anomaly may contribute to ensure a longer symptom-free lifespan of individuals with the most common cardiac anomaly at birth.     

室间隔缺损合并主动脉瓣脱垂及瓦氏窦瘤破裂的外科治疗

目的总结室间隔缺损合并主动脉瓣脱垂及瓦氏窦瘤破裂的治疗体会.方法 118例室间隔缺损患者中合并主动脉瓣脱垂80例,主动脉瓣关闭不全为（＋～＋＋＋）;合并瓦氏窦瘤破裂37例;合并动脉导管未闭3例,三尖瓣关闭不全4例;合并亚急性细菌性心内膜炎7例,合并主动脉-右心室通道1例.结果本组无术后早期死亡,无室间隔缺损残余分流,复查部分病例仍有主动脉瓣关闭不全（＋、＋＋）,2例亚急性细菌性心内膜炎术后3个月再次行主动脉瓣置换术.结论室间隔缺损合并主动脉瓣脱垂及瓦氏窦瘤破裂及早手术治疗总体效果好.术中加强心肌保护、正确处理是治疗