Discovery of a parachute mitral valve complex (Shone's anomaly) in an adult

Parachute mitral valve complex is an unusual congenital anomaly described by Shone et al. It is characterized by a parachute deformity of the mitral valve associated with additional forms of left heart anomalies, such as aortic valvular stenosis and coarctation of the aorta. Fewer than 50 cases of Shone's complex have been reported in the literature, and it has only been observed in children. We report the case of a 33-year-old man who was referred to our department because of atrial fibrillation. Echocardiographic evaluation and aortogram evidenced a Shone's complex, including a parachute mitral valve anomaly, an aortic bicuspid valvular anomaly, and a coarctation of the aorta.     

Aortic valvular malformation associated with coincident cardiovascular anomalies: morphologic considerations

The association of a bicuspid aortic valve with other congenital cardiac malformations, prominently coarctation of the aorta, is widely accepted with estimates of frequency reported to be 20% overall and ranging between 25-50% to as high as 85% when there is coarctation. The anatomic configuration of the valve has received little comment and, excepting those individuals requiring aortic valve surgery coincident with repair of other malformations, has been limited to postmortem examination. Recently, however, an angiographic technique for accurately evaluating the morphology of the aortic valve has been utilized allowing for the determination of aortic valvular appearance at routine evaluation by cardiac catheterization. We have currently examined 17 individuals with various forms of congenital cardiac malformations in whom an aortic valvular abnormality was suspected as a coexistent abnormality employing the technique of orifice-view aortography. The present report concentrates on the aortic valvular anatomy of these patients.     

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.     

接受手术的二瓣化和三瓣化主动脉瓣患者的临床差异

目的研究需要接受主动脉瓣膜或者升主动脉手术的二瓣化或者三瓣化主动脉瓣膜患者的临床差异.方法将1996-2001年的2 570例主动脉瓣膜病变患者,根据主动脉瓣膜形态分为二组,2 015例(78.4%)是三瓣化瓣膜,555例(21.6%)是二瓣化主动脉瓣膜,具体研究统计各项相关数据.结果男性患者在二组中均明显多于女性,在二瓣化组中尤其明显.在二组中早期导致手术的瓣膜病变均是瓣膜狭窄.二瓣化瓣膜的患者接受主动脉瓣膜或者升主动脉手术的年龄平均比三瓣化瓣膜的患者年轻10岁.在二瓣化患者中的升主动脉扩张的发生率明显高于三瓣化的患者.但是在A型主动脉夹层动脉瘤的患者中,三瓣化瓣膜明显比二瓣化瓣膜多.三瓣化主动脉瓣膜的患者同时接受冠状动脉搭桥手术的概率明显高于二瓣化的患者.结论经临床资料对比显示,主动脉瓣先天性二瓣化畸形会加速瓣膜自身的纤维化、钙化导致瓣膜狭窄和关闭不全的早期出现,并且增加升主动脉扩张的危险性.     

Coexisting bicuspid aortic and pulmonary valves diagnosed by 3D transthoracic echocardiography

Coexisting bicuspid aortic and pulmonary valves is an extremely rare condition, and there have been few published cases. Diagnosis of bicuspid aortic valve is straightforward with 2D echocardiography; however, analysis of the morphology of the pulmonary valve is challenging. In this study, we report on a case of a 32‐year‐old man with bicuspid aortic and pulmonary valves diagnosed by 2D and 3D transthoracic echocardiography. The enlarged pulmonary artery without any obvious etiology led us to suspect a pulmonary valve anomaly; thus, we comprehensively evaluated it with 2D and 3D echocardiography, which confirmed the diagnosis of bicuspid pulmonary valve.     

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.     

Impact of valvular calcification on the diagnostic accuracy of transesophageal echocardiography for the detection of congenital aortic valve malformation

BACKGROUND: Degeneration of congenital bicuspid or unicuspid aortic valves can progress more rapidly than that of tricuspid valves, and an early diagnosis significantly impacts decision making and outcome. We hypothesized that the extent of valvular calcification would negatively influence the diagnostic accuracy of multiplane transesophageal echocardiography (TEE) for the diagnosis of congenital aortic valve disease. METHODS: TEE was performed in 57 patients undergoing aortic valve replacement surgery for aortic stenosis (n = 46), pure regurgitation (n = 9), or significant regurgitation with less than severe aortic stenosis (n = 2). The degree of aortic valve calcification and the number of valve cusps were determined at surgery. RESULTS: Surgical inspection confirmed 14 bicuspid and 43 tricuspid aortic valves. Sensitivity and specificity of TEE for the diagnosis of congenital aortic valve malformation was 93% (13/14) and 91% (39/43) (P = 0.0001), respectively. In patients with no or mild aortic valve calcification (n = 13), sensitivity and specificity of TEE for the diagnosis of congenitally malformed aortic valve was 100% (5/5) and 100% (8/8) (P = 0.001), respectively. In patients with moderate or marked aortic valve calcification (n = 44), sensitivity and specificity of TEE for the diagnosis of congenitally malformed aortic valve was 89% (8/9) and 89% (31/35) (P<0.0001), respectively. In this subgroup of 44 patients, there were four false-positive and one false-negative diagnoses due to valvular calcification. CONCLUSIONS: Although TEE is highly sensitive and specific for the detection of congenital aortic valve malformations, presence of moderate or marked calcification of the aortic valve may result in false positive and false negative diagnoses.     

Echocardiographic studies of abnormalities associated with coarctation of the aorta.

Echocardiograms were performed in thirty-six patients (aged 4 to 36 years) with proven coarctation of the aorta. Nineteen patients (53%) were found to have marked diastolic eccentricities of their aortic valves (Eccentricity Index greater 1.5), indicating the presence of bicuspid aortic valves. One of these patients also had multilayered aortic root echoes in diastole. Five patients had angiographic proof of their aortic valve morphologies which corroborated the echo findings. Five patients with bicuspid aortic valves showed mitral valve diastolic flutter indicative of aortic regurgitation. Idiopathic hypertrophic subaortic stenosis (IHSS) was suspected in four patients (11%) with abnormal systolic anterior motion of the mitral valve; three of these patients also had asymmetric septal hypertrophy. There was catheterization proof of IHSS in one patient. Two patients (5.6%) demonstrated mitral valve proplapse.     

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.     

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.     

Cardiovascular abnormalities associated with adult polycystic kidney disease

In a combined retrospective and prospective study, 11 patients with adult polycystic kidney disease were found to have one or more cardiac or aortic lesions. Seven patients had primary dilatation of the aortic root and annulus with aortic regurgitation. The severity of the aortic regurgitation necessitated aortic valve replacement in 2. Mitral regurgitation was present in 3 patients, of whom 2 had documented redundant mitral leaflets and ruptured chordae tendinae, and the third had mitral valve prolapse. Histologic analysis of available aortic and mitral valve tissue from these acquired lesions showed myxomatous degeneration with loss and disruption of collagen. Four patients had congenital bicuspid aortic valves with aortic regurgitation; 1 of these patients also had mild valvular stenosis, and 1 had coarctation of the aorta.     

Three-Dimensional Echocardiographic Evaluation of Aortic and Mitral Valve Stenosis

Dynamic volume rendered three-dimensional echocardiography allows the spatial recognition of anatomy and function of the aortic and mitral valves with acceptable image quality. The aortic valve can be best visualized in a view from the ascending aorta down to the valve level, thus allowing an overview of the aortic aspect of the valve in a surgeon's perspective in ∼ 80% of patients. Planimetric measurement of the aortic valve area was possible in 88% of patients, and there is no systematic overestimation or underestimation of aortic valve area compared with two-dimensional echocardiography and catheterization. The entire valvular circumference of the mitral valve can be assessed from both a left atrial and a left ventricular perspective. Advantages of the three-dimensional transesophageal echocardiography mitral valve area determination compared with transthoracic two-dimensional planimetry and Doppler-derived pressure half-time method are present in patients with severely calcified mitral valves and in those with combined aortic regurgitation.     

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.     

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.     

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.     

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.     

A guide to fluoroscopic identification and design of bioprosthetic valves: A reference for valve‐in‐valve procedure

Surgical aortic valve replacement remains the therapy of choice in majority of patients with aortic stenosis. Bioprosthetic heart valves are often preferred over mechanical valves as they preclude the need for anticoagulation with its associated risks of bleeding and thromboembolism. However, bioprosthetic heart valves undergo structural deterioration and eventually fail. Reoperation is the standard treatment for structural failure of the bioprosthetic valve, stenosis or regurgitation but can carry a significant risk, especially in elderly patients with multiple comorbidities. Transcatheter aortic valve implantation has recently been established as a feasible alternative to conventional valve surgery for the management of high‐risk elderly patients with aortic stenosis. This treatment modality has also been shown to be of benefit in the management of degenerated aortic bioprosthesis as a valve‐in‐valve procedure. The success of this procedure depends on a good understanding of the failing bioprostheses. This not only includes the device design but its radiological/fluoroscopic appearance and how it correlates with the implanted valve, as transcatheter aortic valve implantation is performed under fluoroscopic guidance. Here we illustrate the fluoroscopic appearance of 11 commercially available surgical bioprostheses and two commercially available transcatheter heart valves and discuss important aspects in their design which can influence outcome of a valve‐in‐valve procedure. We have also collated relevant information on the aspects of the design of a bioprosthetic valve, which are relevant to the valve‐in‐valve procedure. © 2012 Wiley Periodicals, Inc.     

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.     

Anomalous origin of the left coronary artery from the pulmonary trunk and its relationship with the morphology of the cardiac semilunar valves in Syrian hamsters

Summary The conditions of the aortic and pulmonary valves and the arrangement in the origin of the coronary arteries were studied in 247 Syrian hamsters belonging to a single family subjected to high endogamous pressure. Most specimens (n=216) were examined using a stereomicroscope. The remaining 31 were studied histologically. In 110 specimens both cardiac semilunar valves were normal. The aortic valve was bicuspid and the pulmonary valve was normal in 79 animals, while a normal aortic valve and a bicuspid pulmonary valve occured in 37. In the remaining 21 specimens both semilunar valves were bicuspid. In 34 cases, the left coronary artery originated from the pulmonary trunk. Statistical analyses indicate that there is no significant relationship between the bicuspid condition of the pulmonary valve and the fact that a coronary artery arises from the pulmonary trunk. On the other hand, they substantiate that the frequency of this coronary artery anomaly significantly increases when the aortic valve is bicuspid. The present findings agree with the hypothesis that abnormal migration of the neural crest cells may be responsible for the combined occurrence of bicuspid aortic valve and anomalous origin of the left coronary artery from the pulmonary trunk. In addition, they suggest that the neural crest cells involved in the formation of the pulmonary valve diverge from those migrating into the aortic valve and those imposing spatial order upon the development of the proximal coronary arteries.