二叶主动脉瓣的超声分析

目的 探讨二叶主动脉瓣的超声心动图特征。方法 采用回顾性方法分析了26例先天性二叶主动脉瓣,其中主动脉瓣狭窄17例（有或无主动脉瓣关闭不全）和单纯主动脉瓣关闭不全9例。结果 在主动脉瓣狭窄组、轻度狭窄14例（82％）;二叶主动脉瓣感染性心内膜炎的发生率为42％;主动脉瓣狭窄组与单纯主动脉瓣反流组相比,主动脉瓣增厚钙化有极显性差异（P＜0．00005）,结果表明,二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。结论：二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。二叶主动脉瓣感染性心内膜炎的发生率较高。彩色多普勒二维超声心动图对二叶主动脉瓣的诊断具有重要价值。     

Rare or unusual causes of chronic, isolated, pure aortic regurgitation

Six patients undergoing aortic valve replacement had rare or unusual causes of isolated, pure aortic regurgitation. Two patients had congenitally bicuspid aortic valves with a false commissure (raphe) displaced to the aortic wall ("tethered bicuspid aortic valve"), two had floppy aortic valves, one had a congenital quadricuspid valve, and one had radiation-induced valve damage.     

Bicuspid aortic valve morphology when associated with coarctation of the aorta

Thirteen children and young adults with coarctation of the aorta as their principal cardiovascular abnormality, 11 with bicuspid aortic valves, were evaluated by orifice-view aortography to evaluate their aortic valvular morphology. For comparison 30 individuals with aortic valvular deformities but without coarctation of the aorta were similarly studied. Two distinct forms of bicuspid valves could be identified characterized by either the appearance of gross inequality of size of the two valve leaflets or an appearance wherein each leaflet closely approximated the size of the other, thus equally bicuspid. Excepting two individuals with normal, tricuspid, aortic valves, all of the patients with coarctation of the aorta had equally bicuspid aortic valves which contrasted to the group without coarctation in which the unequally bicuspid type predominated. This difference in bicuspid aortic valve morphology associated with coarctation of the aorta suggests a different developmental process involving the aortic valve as opposed to the situation in individuals without coarctation.     

Pathology of aortic valve stenosis and pure aortic regurgitation a clinical morphologic assessment—part I

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal detect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.     

Diagnostic significance of three‐dimensional echocardiography in asymptomatic unicuspid aortic valve

Unicuspid aortic valve (UAV) is a rare congenital anomaly of aorta associated with a faster progress of valvular dysfunction, aortic dilatation and with necessity for more frequent controls and precise evaluation Asymptomatic 35 year old man had abnormal systolic diastolic murmur on aortic valve during routine examination. Initial diagnostic with transthoracic echocardiography (TTE) supposed bicuspid aortic valve, while three‐dimensional transesophageal echocardiography (3D TEE) and multidetector computed tomography defined unicuspid, unicomissural aortic valve with moderate aortic stenosis and regurgitation. This case report confirmed that 3D TEE gives us opportunity for early, improved and precise diagnosis of UAV.     

经胸和经食管超声对老年主动脉瓣退行性变早期诊断的研究

目的　评价和对比经胸 (TTE)和经食管 (TEE)超声心动图诊断老年人主动脉瓣周钙化的价值。方法　通过TTE和TEE超声对 5 0例老年主动脉瓣退行性变患者进行检查 ,并以自身前后对照 ,每位入选对象先后行TTE、TEE检查和螺旋CT检查各 1次。结果　螺旋CT示主动脉瓣周钙化阳性的 19例 ,阴性的 31例。以螺旋CT检查结果为对照 ,TTE的敏感性为 6 3% ,特异性为 71% ,准确性为 6 8% ;TEE的敏感性为 79% ,特异性为 6 5 % ,准确性为 70 % ;联合检查的敏感性为 84 % ,特异性为 5 8% ,准确性为 6 8%。结论　TEE超声诊断早期老年性主动脉瓣周钙化的敏感性显著高于TTE ,特异性和准确性接近 ;联合应用TTE和TEE可进一步提高敏感性。     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

Accuracy of two-dimensional echocardiography in determining aortic valve structure in patients >50 years of age having aortic valve replacement for aortic stenosis

We sought to measure the accuracy of 2-dimensional transthoracic echocardiography in determining aortic valve structure in patients with aortic stenosis (AS) undergoing aortic valve replacement (AVR). Few studies have compared aortic valve structure determined by echocardiogram to that determined by examination of the operatively excised stenotic aortic valve. Two-dimensional echocardiograms were reviewed and interpreted by an expert echocardiographer in blinded fashion in 100 patients >50 years of age (mean 70) who had undergone AVR for isolated AS ± aortic regurgitation and the aortic valve structure (unicuspid, bicuspid, tricuspid) was compared to that from examination of the operatively excised stenotic valve. After excluding 14 cases in which echocardiograms were uninterpretable because of heavy calcium and/or poor image quality, congenitally malformed valves were present in 44 patients (51%) and tricuspid valves in 42 of the 86 patients (49%). Ten of the 14 patients (71%) with uninterpretable echocardiograms had congenitally malformed valves. Valve structure by echocardiogram was concordant with morphologic interpretation in 57 of 86 patients (66% accuracy, kappa = 0.33). Accuracy trended toward improvement as degree of AS decreased. In patients with valve areas similar to those enrolled in the recent transcatheter aortic valve implantation trial (PARTNER; 0.7 ± 0.2 cm(2)), aortic valve structure was accurately determined by echocardiography in 21 of 35 patients (60%). In conclusion, aortic valve structure was interpretable by transthoracic echocardiogram in 86 of 100 patients and accurate in 57 of these 86 patients (66%).     

The congenital bicuspid aortic valve with post-inflammatory disease--a neglected pathological diagnosis of clinical relevance

It is unusual to find surgically excised congenital bicuspidaortic valves with post-inflammatory disease. We have analysedretrospectively all surgically excised aortic valves over a6-year period for this particular diseased state in relationto relevant clinical data. There were 181 congenital bicuspid aortic valves, and the characteristicgross pathology of the tricuspid aortic valve with post-inflammatorydisease served as reference. Twelve of the 181 congenital bicuspidaortic valves were identified as post-inflammatory (6–6%);one with clinically established rheumatic disease. The meanage at operation of these 12 patients was significantly lowercompared to ‘other’ aortic valves. The study indicates that post-inflammatory involvement of acongenital bicuspid aortic valve can be identified, which isof clinical relevance since these patients have a significantlyyounger age at operation.     

Dissection of the aorta associated with congenital malformation of the aortic valve

The association of congenital aortic valve malformation and aortic dissection is analyzed. Over a 30 year period, 186 patients with non-iatrogenic aortic dissection were studied at necropsy. The aortic valve was tricuspid in 170 (91.4%), bicuspid in 14 (7.5%) and unicuspid in 2 (1.1%). Among the 16 patients with aortic dissection and a congenitally malformed valve, the age at death ranged from 17 to 82 years (mean 52) and 13 (81%) were men. The entrance tear of the aortic dissection was located in the ascending aorta in all 16 patients with a malformed valve but in only 68% of those with a tricuspid aortic valve. The aortic valve was stenotic in 6 of the 16 patients with a congenitally malformed valve. Fatal rupture of the false channel occurred after acute ascending aortic dissection in each of the 11 patients (none with healed dissection) who did not have operative therapy for the dissection. Two of the 16 patients with a malformed valve compared with no patient with a tricuspid aortic valve had aortic isthmic coarctation. Histologic sections of aorta from 10 patients disclosed severe degeneration of the elastic fibers of the media in 9 patients. Thus, a congenitally malformed aortic valve appears to be present at least 5 times more frequently in adults with than in those without aortic dissection, and in our patients the entrance tear was always in the ascending aorta, which usually had severe loss of elastic fibers in its media.     

A rare mechanism of aortic regurgitation in a young patient

A 19‐year‐old male patient was admitted to our institute with dyspnea. His medical history had no rheumatic fever or infective endocarditis. Physical examination revealed a diastolic murmur over the aortic area, rales of bilateral lungs. Bedside transthoracic echocardiography (TTE) revealed a severe aortic regurgitation (AR) without aortic valve stenosis and a moderately dilated left ventricle accompanied by an ejection fraction of 55%. The aortic valve could not be clearly demonstrated as either bicuspid or tricuspid. Congenital AR typically occurs in conjunction with an additional cardiac abnormality or aortic valve stenosis. Furthermore, bicuspid aortic valves are observed in the majority of patients. The aortic valve is created from the truncus ridge of the truncus arteriosus while the embryological development.     

Three-dimensional echocardiographic evaluation of an incidental quadricuspid aortic valve

Background: Quadricuspid aortic valve is one of the rare forms of congenitalcardiac valvular disease. Its diagnosis is often missed, evenwith the transthoracic echocardiogram. Many of these patientsprogress to aortic incompetence later in life requiring surgicalintervention. In addition, quadricuspid aortic valve can beassociated with other congenital cardiac deformities. Henceearly recognition and follow-up is critical in these patients. Case presentation: We report a patient with quadricuspid aortic valve identifiedon intraoperative transesophageal 3-D echocardiography. This66-year-old male presented with the features of congestive heartfailure. The preoperative transthoracic echocardiogram (TTE)disclosed, moderately severe aortic valve insufficiency alongwith severe mitral and tricuspid regurgitation, but failed toreveal the quadricuspid anomaly of the aortic valve. Interestingly,this patient had undergone transthoracic echocardiography ontwo previous occasions during the past seven years for the evaluationof his valvular heart disease, which all failed to documentthis anomaly. Intraoperatively, transesophageal echocardiography(TEE) displayed an aortic valve composed of three medium andone small cusps. Conclusion: Our patient's case demonstrates the usefulness of transesophagealechocardiography in detection of this uncommon congenital malformation.     

Contrasting histoarchitecture of calcified leaflets from stenotic bicuspid versus stenotic tricuspid aortic valves

Preliminary findings from clinical trials of percutaneous balloon aortic valvuloplasty and intraoperative debridement of calcific deposits in patients with aortic stenosis have suggested that calcified, congenitally bicuspid aortic valves may be less amenable to these techniques than are calcified tricuspid aortic valves. Accordingly, we evaluated the histoarchitecture of calcific deposits in 30 operatively excised aortic valves. Light microscopic sections taken through the calcified aortic valve leaflets disclosed two principal types of histoarchitecture. In 11 aortic valves nodular calcific deposits were superimposed on an underlying fibrotic aortic valve leaflet (type A); in 17 valves calcific deposits were diffusely distributed throughout the body (spongiosa) of the aortic valve leaflets (type B). Two aortic valves could not be classified histologically. These histologic subtypes were not randomly distributed with regard to gross valvular morphology. All 14 bicuspid valves (100%) were type B; in contrast, 11 (69%) of 16 tricuspid aortic valves were type A, and only 3 (19%) of 16 tricuspid valves were type B (p less than 0.01). Both valves with nonclassifiable histologic features were tricuspid on the basis of gross examination. Thus, the histoarchitectural distribution of calcific deposits is different for bicuspid than for tricuspid stenotic aortic valves. The more diffuse distribution of calcium throughout the body of calcified bicuspid aortic valve leaflets may render these valves less amenable to operative and percutaneous valvuloplasty than are calcified tricuspid aortic valve leaflets on which calcific deposits are typically superimposed in nodular form.     

Anatomically isolated aortic valve disease. Morphologic study of 100 cases at autopsy

Anatomically isolated aortic valve disease accounted for 1.1% of all and 5.2% of cardiac autopsies over a 20 year period. Among a total of 100 symptomatic cases, 52% had congenitally bicuspid, 43% had tricuspid and 5% had congenitally unicuspid valves. Nineteen percent had undergone fibrous sclerosis, including 1% unicuspid, 3% tricuspid and 15% bicuspid valves. Thirty nine percent had been affected by infective endocarditis, including 20 bicuspid and 19 tricuspid valves. The remaining 42 showed fibrocalcification, including 4 unicuspid, 17 bicuspid and 21 tricuspid valves. The nature of the valvular disease showed a correlation with the age of the patient. Infective endocarditis, fibrosis and calcific disease occurred in an ascending age pattern, at average ages of around 30 years, mid to late thirties and mid to late forties, respectively. The lesions occurred much earlier on the bicuspid than on the tricuspid valves, except for infective endocarditis. The bicuspid deformity was not found to make the aortic valve more prone to infection, nor did infection occur earlier on it than on the tricuspid valve. Four of the 100 cases, all tricuspid, were considered to be of rheumatic origin, the reasons for which are discussed. Certain well established associated cardiac lesions were identified.     

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。     

The role of transesophageal echocardiography in the diagnosis and management of patients with aortic perivalvular abscesses

Aortic valve abscesses (AVAs) are a devastating complication of aortic valve endocarditis. Over 8 years, 25 patients were diagnosed with AVA by transesophageal echo (TEE). Management and outcomes were then analyzed. Eleven (44%) AVAs involved prosthetic valves, and 6 (24%) occurred in congenitally malformed valves. Twenty patients (80%) underwent surgical intervention; the rest were treated medically. Eleven (44%) of the patients died [6 (30%) surgery patients and all the medical patients]. Eight of 11 (73%) patients who died were culture positive for Staphylococcus aureus. All patients with congenitally malformed aortic valves underwent surgical intervention and survived. We conclude that: (1) despite advances in therapy and diagnosis, patients with AVAs have a high mortality rate; (2) prognosis with AVA is especially poor when S aureus is the infectious organism; (3) patients with AVAs in congenitally malformed valves have a great outcome with surgery; (4) patients treated medically have a very poor prognosis; earlier identification by TEE may be critical to improving survival.     

Presence of a congenitally bicuspid aortic valve among patients having combined mitral and aortic valve replacement

Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various types of dysfunctioning mitral valves. Eleven of the 16 patients had aortic stenosis (AS): 5 of them also had mitral stenosis, of rheumatic origin in 4 and secondary to mitral annular calcium in 1; the other 6 with aortic stenosis had pure mitral regurgitation (MR) secondary to mitral valve prolapse in 3, to ischemia in 2, and to unclear origin in 1. Of the 5 patients with pure aortic regurgitation, each also had pure mitral regurgitation: in 1 secondary to mitral valve prolapse and in 4 secondary to infective endocarditis. In conclusion, various types of mitral dysfunction severe enough to warrant mitral valve replacement occur in patients with bicuspid aortic valves. A proper search for mitral valve dysfunction in patients with bicuspid aortic valves appears warranted.     

Aortic valvuloplasty of calcific aortic stenosis with monofoil and trefoil balloon catheters: practical considerations

Summary In order to evaluate the relation between balloon design (monofoil, trefoil) and valvular configuration, experimental aortic valvuloplasty was performed in four post-mortem hearts with calcific aortic stenosis of various morphology. The degree of obstruction of the aortic orifice was assessed by computed axial tomography during inflation of monofoil 15 and 19 mm and trefoil 3×12 mm balloon catheters. We also evaluated the hemodynamic repercussion of balloon inflation (fall in systolic aortic pressure) in four elderly patients with acquired aortic stenosis who underwent a percutaneous transluminal aortic balloon valvuloplasty, with stepwise increasing balloon sizes of 15 mm, 19 mm and 3×12 mm, as during ourin vitro experiments, and who underwent aortic valve replacement later on. In these patients, we correlated the anatomy of the excised aortic valves with the retrospective analysis of aortic pressure curves recorded during previous valvuloplasty procedures.Our experimental and clinicopathological observations showed that the degree of obstruction of the aortic orifice in post-mortem specimens and the tolerance to balloon inflation in live patients are dependent of the valvular configuration. Although trefoil balloons have the theoretical advantage to avoid complete obstruction of the aortic orifice during inflation, we observed that in presence of a tricuspid configuration, they could be potentially more occlusive than monofoil balloons since each of the 3 individual components of the trefoil balloon occupied the intercommissural spaces while inflated. However, they offered more residual free space when inflated in aortic valves with a bicuspid configuration (i.e. congenitally bicuspid valves or tricuspid valves with one fused commissure).In our opinion, these observations are relevant, since degenerative disease of the aortic valve (i.e. tricuspid valve without commissural fusion) is now recognized as the most common etiology of aortic stenosis in the elderly.Abbreviations AS aortic stenosis - AVA aortic valve area - PTAV percutaneous transluminal aortic balloon valvuloplasty A video supplement to this article has been published in Cardiac Imaging Video Journal volume 2, nr. 4, 1989, see Reference 29.     

Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography

The prevalence of valvular regurgitation in a large population with structurally normal hearts remains unknown. From the computer database of the echocardiography laboratory of the hospital, 7209 records containing results of both two-dimensional and Doppler echocardiographic examinations were identified, from which 867 (12%) with no structural abnormality on two-dimensional echocardiograms were obtained for analysis. Of these 867 records, 291 (34%) had evidence of regurgitation by Doppler technique in at least one cardiac valve. Mitral regurgitation was found in 167 (19%), tricuspid regurgitation in 151 (17%), pulmonic regurgitation in 45 (5%), and aortic regurgitation in 29 records (3%). Regurgitation of just one valve was the most common and occurred in 207 records (24%). This was followed by regurgitation of two valves (69 records, 8%), three valves (13 records, 2%), and four valves (two records, 0.2%). The prevalence of mitral, tricuspid, and aortic regurgitation was found to increase significantly with increasing age, as was the prevalence of regurgitation involving multiple valves. In 98% and 95% of mitral and tricuspid regurgitations, respectively, the jets were confined to the proximal one fourth of the atria, suggesting only trivial or mild regurgitation. Thus valvular regurgitation occurs not uncommonly in patients with structurally normal hearts referred for echocardiographic evaluation. These findings caution against the inappropriate diagnosis of clinical disease in the many patients who fall into this category. The increasing prevalence of valvular regurgitation with increasing age suggests that a wear-and-tear phenomenon rather than a congenital cause is involved in most instances.