Quadricuspid aortic valve diagnosed by echocardiography in two cases identical twins

Quadricuspid aortic valves (QAVs) are a rare but well-recognized congenital heart disease and a cause of aortic regurgitation. The first case was reported in 1862. Since then, there have been about 189 cases reported with advances in echocardiography, more cases are diagnosed by transesophageal echocardiography. Hurwitz and Roberts have classified QAV according to the size of the leaflets. Our case demonstrates 2 different valve classifications based on equal and unequal cusp size. In conclusion, to our knowledge, this is the first case of QAV in identical twins.     

An Interesting Case of Tricuspid Aortic Valve with a Quadricuspid Function

Quadricuspid aortic valve (QAV) is a rare congenital heart defect, often related to severe aortic regurgitation, and usually detected by echocardiography or at the time of aortic valve surgery. We report a case of an interesting and extremely rare variant of “false” QAV, detected preoperatively by transthoracic and transesophageal echocardiography, in a severely symptomatic patient, admitted to our hospital for dyspnea. Three leaflets of aortic valve appeared quadricuspid, because the left coronary cusp was divided into 2 parts, as confirmed by MRI and pathology. Most frequently, QAV presents with all 4 leaflets equal in size.     

Quadricuspid aortic valve as a cause of severe aortic regurgitation.

Quadricuspid aortic valves (QAVs) constitute a rare congenital malformation, with an incidence ranging from 0.008 to 0.048%. We report a case of severe aortic regurgitation associated with a QAV, which was diagnosed intraoperatively using transesophageal echocardiography. Since the first case described in 1862, 186 QAVs have been reported. In most cases, QAVs are associated with valve regurgitation, with a concurrent stenosis in some patients, while only a small number of QAVs are functionally normal. Once the diagnosis has been made, echocardiographic follow-up is recommended, as progression to severe valve regurgitation is common. Antibiotic prophylaxis is advisable for dental, and "dirty" surgical procedures, to minimize the risk of infective endocarditis.     

主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.     

Quadricuspid aortic valve: a case report and review of the literature

Quadricuspid aortic valve (QAV) is a rare congenital anomaly that can present as aortic insufficiency later in life. We report a case of aortic regurgitation associated with a QAV, treated by aortic valve replacement. The patient presented with breathlessness, lethargy and peripheral oedema. Echocardiography and cardiac magnetic resonance revealed abnormal aortic valve morphology and coronary angiography was normal. The presence of a quadricuspid aortic valve was confirmed intra-operatively. This was excised and replaced with a bioprosthetic valve and the patient recovered well postoperatively. Importantly, the literature indicates that specific QAV morphology and associated structural abnormalities can lead to complications. Hence, early detection and diagnosis of QAV allows effective treatment. Aortic valve surgery is the definitive treatment strategy in patients with aortic valve regurgitation secondary to QAV. However, the long-term effects and complications of treatment of this condition remain largely unknown.     

Intraoperative echocardiography and Doppler color flow mapping in freehand allograft aortic valve and root replacement

Intraoperative epicardial echocardiography and color flow Doppler were performed before and after cardiopulmonary bypass in 17 consecutive patients undergoing 20 freehand allograft aortic valve replacements. Native aortic valves were replaced in 12, and prostheses in 8 patients. Precardiopulmonary bypass echocardiography estimates of annular diameter guided allograft selection and predicted length of allograft aortic root required, defined coronary situs, and revealed other cardiac abnormalities. These included unanticipated severe mitral regurgitation (which precluded allograft aortic valve replacements in one patient), left-to-right shunts in the membranous septum, ascending aortic dissection, and aortic root pathology requiring coronary reimplantation or bypass. Postcardiopulmonary bypass echocardiography demonstrated acceptable competency of 18/19 allograft valves (mild or no aortic insufficiency), and successful repair of 3/4 shunts. Mild mitral regurgitation was detected more often at postcardiopulmonary bypass than precardiopulmonary bypass (15 vs 11 cases) and postcardiopulmonary bypass estimates of mitral regurgitation severity corollated well with subsequent postoperative follow-up. IOE allows selection and thawing of the allograft valves prior to aortic cross clamping, minimizing cross-clamp time. It detects important concomitant cardiac abnormalities, and predicts postoperative allograft valve and mitral competency. Intraoperative echocardiography Doppler, is therefore, a useful adjunct for allograft aortic valve replacements or aortic root replacement.     

Quadricuspid Aortic Valve: A Case Report and Review of the Literature

The quadricuspid aortic valve (QAV) is a rare malformation; often isolated, sometimes associated with other heart diseases. Before the era of echocardiography, the diagnosis was made incidentally at autopsy or during surgery of valve replacement. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic approach is suitable for the diagnosis but, transesophageal echocardiography is a tool for the accurate definition of the valve anatomy. This review analyzes, after the presentation of a clinical case, the current knowledge on embryogenesis, classification, diagnosis and clinical course of QAV.     

Association of aortic valve sclerosis and coronary artery disease in patients with severe nonischemic mitral regurgitation

BACKGROUND: The presence of aortic valve sclerosis accounts for a higher rate of ischemic events and increased cardiovascular mortality. It may reflect coronary artery disease (CAD) because of a shared pathologic background. HYPOTHESIS: We aimed to analyze whether the presence of aortic valve sclerosis might help in identifying patients with coronary atherosclerosis among those with severe nonischemic mitral regurgitation (MR), who undergo coronary angiography before surgery for screening, and not because of suspected ischemic heart disease. METHODS: In all, 84 patients (mean age 64 +/- 9 years; 71% men) with mitral valve prolapse and severe regurgitation underwent echocardiography and coronary angiography. Aortic valve sclerosis was defined as focal areas of increased echogenicity and thickening of the leaflets without restriction of leaflet motion on echocardiography. Coronary artery disease was defined by the presence/absence of atherosclerotic plaques, independent of the degree of stenosis. RESULTS: Coronary artery disease was diagnosed in 47.6% of patients with and 15.8% of those without aortic valve sclerosis (p = 0.008). On logistic regression analysis, the presence of aortic valve sclerosis predicted CAD (odds ratio 3.3, 95% confidence interval 1.03-10.5; p = 0.04) independent of age. In female patients, the risk ratio for CAD in the presence of aortic valve sclerosis was 9. CONCLUSIONS: Coronary artery atherosclerosis and aortic valve sclerosis are closely associated in patients with severe nonischemic MR.     

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.     

Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature.

A case is reported of a 38 year old woman without known cardiac congenital abnormality but a history of well controlled arterial hypertension who was admitted to hospital after successful resuscitation at home following cardiac arrest. There was no evidence of myocardial infarction on 12-lead electrocardiogram but there were signs of left ventricular hypertrophy. Transoesophageal echocardiography revealed a rare quadricuspid aortic valve (QAV) malformation with concomitant mild aortic regurgitation. The left ventricle showed a massive concentric hypertrophy without obstruction. The patient was eventually transferred in a persistent vegetative state to a home care facility. A review of the literature revealed 70 cases of QAV diagnosed by transthoracic or transoesophageal echocardiography (26 cases), at necropsy (25), during surgery (15), and during angiography (4). The present case is the first report of QAV associated with non-obstructive hypertrophic cardiomyopathy.     

Flail Aortic Valve in Systemic Lupus Erythematosus: Diagnosis by Transesophageal Echocardiography

This case report describes a patient with systemic lupus erythematosus who demonstrated by trans-esophageal echocardiography (TEE) a flail right coronary cusp of the aortic valve causing severe aortic regurgitation. This finding was confirmed at surgery, which showed near detachment of the right coronary leaflet near the commissure. Destruction of areas of the aortic valve has been reported in one previous case report in the absence of infective endocarditis or a traumatic process. This is the second case to our knowledge and the first to describe identification by TEE.     

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.     

Retinal embolization of bicuspid aortic valve calcification

We report the case of a 36-year-old man with calcified bicuspid aorta. Aortic disease was diagnosed after retinal embolism, which caused loss of vision affecting the left eye. Doppler examination did not identify carotid stenosis. Transthoracic echocardiography showed thickness and calcification of two leaflets, moderate aortic regurgitation, high normal left ventricular diastolic dimensions, and high normal interventricular septum thickness. Parasternal long-axis view in the systolic frame showed dilated left ventricular outflow tract, dilated ascending aorta, and calcification and thickening of aortic leaflets, causing an echo-lucent area, in the diastolic frame. It also showed the eccentric position of the closed valve leaflets, short axis in the diastolic frame, and the absence of the typical "Y" letter sign, tracked by the three leaflets (septal, right coronary, and left coronary) in the normal tricuspid aortic valve. On long-axis parasternal acquisition, we note high normal interventricular septum thickness and high normal diastolic diameter. Color Doppler analysis from the apical five-chamber view showed aortic regurgitation; regurgitant jet area was about 44% of the outflow tract, so aortic regurgitation was classified as moderate. Pulsed Doppler showed a high normal value of peak aortic velocity. Transesophageal echocardiography was performed, but no further findings were identified. We think that the likely retinal embolism sources were the aortic calcified leaflets, even if the aortic valve was not stenotic and with no signs of endocarditis. However, because we cannot exclude other potential causes, we put the patient on anticoagulant therapy. Furthermore, aortic valve replacement was not advised.     

Balloon valuloplasty prior to transcatheter valve‐in‐valve implantation in a degenerated mitroflow aortic bioprosthesis

Transcatheter valve‐in‐valve implantation is an emerging treatment option for high‐risk patients with failing aortic bioprostheses. The presence of the prosthesis stents is thought to prevent coronary artery obstruction, a known complication of transcatheter aortic valve implantation in the native aortic valve. The Sorin Mitroflow aortic bioprosthesis (Sorin Group, Saluggia, Italy) has a particular design in that the pericardial leaflets are mounted outside the valve stent. As a consequence, the pericardial leaflets of this prosthesis may be displaced well away from the stents during the deployment of transcatheter valves. This might explain why both the cases of coronary occlusion following valve‐in‐valve implantation reported to date occurred in patients with a malfunctioning Mitroflow bioprosthesis. We describe a patient with a malfunctioning 25 mm Mitroflow bioprosthesis successfully treated by percutaneous transcatheter valve‐in‐valve implantation, and discuss the role that balloon aortic valvuloplasty plays in the performance of this delicate procedure. © 2012 Wiley Periodicals, Inc.     

Aortic valve insufficiency: an unrecognized complication of the surgical repair of ostium primum atrial septal defect

Between 1974 and 1989, eight children undergoing surgical repair of a partial atrioventricular canal comprising an ostium primum atrial septal defect, mitral regurgitation due to a cleft mitral valve (6 cases) and a small ventricular septal defect (1 case) developed aortic regurgitation. This complication was diagnosed on the finding of a diastolic murmur 1 day to 9 years after the repair of the atrial septal defect. The degree of regurgitation was Grade I in 4 cases but in the other patients it was severe from the outset requiring emergency surgery the next day (1 case), or moderate at the beginning but rapidly progressive (1 case) or slowly progressive (2 cases) leading to aortic valvuloplasty in these 3 patients. The cause of the regurgitation was iatrogenic: perforation of the non coronary cusp secondary to repair of the ostium primum atrial septal defect. None of the patients required aortic valve replacement. After surgical valvuloplasty, all children had Grade 1 aortic regurgitation. Color coded Doppler echocardiography confirmed the diagnosis and helped assess the clinical course of this lesion during follow-up.     

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.     

Quadricuspid aortic valve with a hidden left ostium: Case report and literature review

Quadricuspid aortic valve (QAV) is a rare congenital condition that frequently progresses to aortic regurgitation with clinical impact in adulthood. Surgical treatment is required in the fifth to sixth decade of life in about one fifth of patients.We describe the case of a 64-year-old woman with regular cardiological follow-up for severe aortic valve regurgitation who had suffered recent clinical and echocardiographic deterioration. Conventional open surgery was indicated. During the procedure, a QAV with leaflet retraction and central orifice was observed. The aortic valve was successfully replaced.     

Obstruction of the right coronary artery ostium by an aortic valve papillary fibroelastoma

Aortic valve papillary fibroelastomas are rare benign tumours which may present with the sequele of embolisation, coronary occlusion or sudden death. We report a case of a 68 year old lady presenting with unstable angina who appeared at coronary angiography to have thrombus in the right coronary ostium. Increasing right territory ischaemia prompted echocardiography, which diagnosed a mass in the right coronary ostium. Urgent surgery was undertaken, demonstrating chronic impaction of an aortic valve fibroelastoma in the right coronary ostium. Aortic valve replacement and coronary bypass grafting to the distal right coronary artery (RCA) was performed. This case illustrates a common cardiology presentation caused by the complication of a rare tumour.     

Echocardiographic versus Histologic Findings in Marfan Syndrome

This retrospective study attempted to establish the prevalence of multiple-valve involvement in Marfan syndrome and to compare echocardiographic with histopathologic findings in Marfan patients undergoing valvular or aortic surgery.We reviewed echocardiograms of 73 Marfan patients who underwent cardiovascular surgery from January 2004 through October 2009. Tissue histology was available for comparison in 29 patients.Among the 73 patients, 66 underwent aortic valve replacement or the Bentall procedure. Histologic findings were available in 29 patients, all of whom had myxomatous degeneration. Of 63 patients with moderate or severe aortic regurgitation as determined by echocardiography, 4 had thickened aortic valves. The echocardiographic findings in 18 patients with mitral involvement included mitral prolapse in 15. Of 11 patients with moderate or severe mitral regurgitation as determined by echocardiography, 4 underwent mitral valve repair and 7 mitral valve replacement. Histologic findings among mitral valve replacement patients showed thickened valve tissue and myxomatous degeneration. Tricuspid involvement was seen echocardiographically in 8 patients, all of whom had tricuspid prolapse. Two patients had severe tricuspid regurgitation, and both underwent repair. Both mitral and tricuspid involvement were seen echocardiographically in 7 patients.Among the 73 patients undergoing cardiac surgery for Marfan syndrome, 66 had moderate or severe aortic regurgitation, although their valves manifested few histologic changes. Eighteen patients had mitral involvement (moderate or severe mitral regurgitation, prolapse, or both), and 8 had tricuspid involvement. Mitral valves were most frequently found to have histologic changes, but the tricuspid valve was invariably involved.     

A case of prosthetic aortic valve dehiscence due to infective endocarditis without paravalvular regurgitation

Aortic prosthetic valve endocarditis is often a challenging disease process that carries high morbidity and mortality. Echocardiography is widely used to identify infected valves and associated complications. One major complication of an infection involving the aortic annulus is dehiscence of the prosthetic valve from the aortic root and is usually associated with paravalvular regurgitation. Here, we present a rare case of complete prosthetic valve dehiscence without paravalvular regurgitation on transthoracic and transesophageal echocardiography.