Congenital Quadricuspid Aortic Valve Research Articles

“One-stop” interventional therapy for quadricuspid aortic valve combined with severe coronary artery disease: a case report

Congenital Quadricuspid Aortic Valve (QAV) malformation is a relatively rare cardiac valve malformation, especially with abnormal coronary opening and severe stenosis of Coronary Artery Disease (CAD). The patient underwent “one-stop” interventional treatment with transcatheter aortic valve replacement and percutaneous coronary stent implantation. Follow up for 12-month with good outcomes.

Fusion of Bovine Tissue Aortic Valve Leaflets in a Patient with Left Ventricular Assist Device: A Case Report

<h3>Introduction</h3> We present a case of a patient with prior bioprosthetic aortic valve replacement who underwent Left Ventricular Assist Device (LVAD) explant and was found to have fused valve leaflets at the time of surgery. <h3>Case Report</h3> The patient is a 30 year old woman with a history of a congenital quadricuspid aortic valve (frequency < 0.05%), complicated by myocardial infarction and cardiogenic shock with LVEF 5% due to occlusion of the left main coronary ostium by rupture of one of the leaflets. She required support via Veno-Arterial ExtraCorporeal Membrane Oxygenation (VA-ECMO) followed by Aortic Valve Replacement with #23 Bovine Tissue Valve and HeartMate 3™ (Abbot Laboratories) implantation in January 2019. Her LVEF improved to 55% after 6 months of mechanical circulatory support. The patient was scheduled for LVAD explant 9 months after implant. Intraoperative transesophageal echocardiogram showed leaflet fibrosis & severe functional closure of her prosthetic aortic valve and left ventricular outflow tract, not seen on prior echocardiograms (Figure 1). We performed an LVAD explant via redo sternotomy and left anterolateral thoracotomy, redo-aortic valve replacement with a #19 mechanical valve, and ventriculotomy closure using custom made synthetic plug. She is doing well 13 months after surgery. <h3>Summary</h3> Patients with prior tissue aortic valve replacement require careful preoperative evaluation before LVAD explant. Though we had a good outcome, pre-procedural knowledge of valve dysfunction is critical in surgical planning. We have developed an algorithm which may be utilized in the future to improve outcomes (Figure 2).

Severe Aortic Regurgitation Caused by Congenital Quadricuspid Aortic Valve With an Anomalous Cord

Summary An aortic valve in a 71-year-old female exhibited a quadricuspid valve morphology. Two-dimensional transesophageal echocardiography showed an incompletely separated non-coronary cusp and severe aortic regurgitation. Moreover, real time three-dimensional transesophageal echocardiography indicated the presence of an anomalous cord that appeared to be adhered to the tip of the two leaflets of the incompletely separated non-coronary cusp.

Congenital Quadricuspid Aortic Valve, a Rare Cause of Aortic Insufficiency in Adults: Case Report

Congenital Quadricuspid Aortic Valve, a Rare Cause of Aortic Insufficiency in Adults: Case Report

Type F Congenital Quadricuspid Aortic Valve: A Very Rare Case Diagnosed by 3-dimenional Transoesophageal Echocardiography

Congenital quadricuspid aortic valve (QAV) is a rare cardiac anomaly. Several different anatomical variations of a quadricuspid aortic valve have been described. Aortic regurgitation is the predominant valvular dysfunction associated with QAV and patients tend to present in their 5th or 6th decade of life. This anomaly is rarely picked up by transthoracic echocardiogram (TTE). A comprehensive transoesophageal echocardiography (TOE) study is more likely to diagnose it. We describe a very rare type of QAV – Type F in a 52-year-old lady who presented with symptoms of shortness of breath and pre-syncope. We include TOE images and intra-operative valve images.

Quadricuspid aortic valve complicated with infective endocarditis: report of a case

Congenital quadricuspid aortic valve is a rare cardiac malformation with an unknown risk of infective endocarditis. We report a case of quadricuspid aortic valve complicated with infective endocarditis. A 53-year-old Japanese woman was hospitalized with leg edema and a fever of unknown origin. Corynebacterium striatum was detected in the blood culture. Echocardiography demonstrated a quadricuspid aortic valve with vegetation and severe functional regurgitation. The condition was diagnosed as a quadricuspid aortic valve with infective endocarditis, for which surgery was performed. The quadricuspid aortic valve had three equal-sized cusps and one smaller cusp (type B according to Hurwitz classification). We dissected the vegetation and infectious focus and implanted a mechanical valve. Following the case report, we review the literature.

Congenital quadricuspid aortic valve associated with aortic insufficiency and mitral regurgitation

Congenital quadricuspid aortic valve is a rare cardiac anomaly. More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. It is vital that patients with quadricuspid aortic valve who present with progressive aortic regurgitation undergo valve replacement or repair at appropriate time. Here, we present two cases of quadricuspid aortic valve. We first describe a 58-year-old man who had mitral regurgitation and ascending aorta dilatation with quadricuspid aortic valve. He underwent aortic valve replacement and mitral valve plasty and recovered well. The second patient is a 20-year-old asymptomatic boy who has been closely followed up and has not received any surgical treatment.

Quadricuspid Aortic Valve: Multimodality Imaging

A 56-year-old man presented to the outpatient department with a history of atypical chest pain. He had no traditional risk factors. Cardiac auscultation revealed a 3/6 early diastolic murmur along the lower left sternal border. Electrocardiography was normal. Transthoracic echocardiography showed normal left-ventricular dimension with normal left-ventricular ejection fraction (55%). Transthoracic and transesophageal echocardiography showed unusual quadricuspid aortic valve with four equal size cusps (Fig. 1a, b, Video 1 in Supplementary material).Color Doppler examination showed mild central aortic regurgitation (Fig. 2a, b; Video 2 in Supplementary material). TEE in basal short-axis view clearly demonstrated anatomy of aortic valve. Three-dimensional TEE showed excellent visualization of aortic valve anatomy (Fig. 3a, b; Video 3 in Supplementary material). As a part of chest pain evaluation and to rule out associated coronary artery anatomy, the patient underwent 64-slice multidetector computed tomographic (CT) coronary angiography, which showed quadricuspid aortic valve with two separate origins of the right and left normal coronary arteries (Fig. 4a, b). Virtual intra-aortic endoscopy by multidetector CT showed a rare appearance of four equal-sized aortic cusps. The patient was advised to pursue medical follow-up. Congenital quadricuspid aortic valve is an uncommon developmental abnormality and is usually diagnosed incidentally at autopsies or during cardiac surgery. With

Congenital Quadricuspid Aortic Valve: A Rare Valvular Pathology Leading to Aortic Valve Insufficiency

We herein report the case of severe congenital quadricuspid aortic valve insufficiency in a 47 year-old, 2 m tall man. He noticed a drop in his exercise capacity, but initially declined to see a physician. Prior to admission to our clinic for aortic valve surgery, he was diagnosed with NYHA III congestive heart failure. Transthoracic echocardiography (TTE), though, detected grade III° aortic regurgitation, but the exact morphology of the aortic valve was not clear and was described as being bicuspid. Aortography suggested a quadricuspid aortic valve with four equal aortic cusps, which was confirmed by transoesophageal echocardiography (TEE) (Fig. 1).

Surgical treatment of congenital quadricuspid aortic valve

To improve the understanding of congenital quadricuspid aortic valve (QAV), explore its echocardiographic diagnostic value and summarize the methods and outcomes of surgical treatments. The clinical data of 11 QAV patients from January 2000 to December 2008 were retrospectively analyzed. There were 9 males and 2 females with a mean operative age of (32±16) years (range: 4-55). In 766 patients undergoing aortic valve surgery, 11 were of congenital quadricuspid aortic valve (1.4%); cardiac pathology: infective endocarditis (n=1), left superior vena cava (n=1), aortic aneurysm (n=1), mitral prolapse (n=1) and tricuspid insufficiency (n=1). The patients of congenital QAV deformity was diagnosed by echocardiography (n=7), misdiagnosed as single valve (n=1), misdiagnosed as bicuspid valve (n=1) and misdiagnosed as rheumatic heart disease (n=2). Type B (n=7), Type A (n=2), Type F (n=1) and Type G (n=1). Eleven patients underwent the procedure of aortic valve replacement. And the concomitant procedures were aortic root broadening (n=1), ascending aortoplasty (n=1), mitral valvuloplasty (n=1) and tricuspid valvuloplasty (n=1). Quadricuspid aortic valve is rare in clinical practice. And echocardiography plays an important diagnostic role. Surgical replacement of aortic valve is the first-line therapy for these patients.

Occlusion of Left Coronary Ostium With a Rudimentary Aortic Cusp

A 74-year-old woman with exertional angina was diagnosed with occlusion of the left coronary ostium associated with a rudimentary aortic valve cusp. A transesophageal echocardiogram, a multi-detector computed tomographic scan, and a coronary angiographic scan revealed the rudimentary aortic cusp covering the small left coronary aortic sinus leading to occlusion of the ostium of the left coronary artery, despite the intact coronary arteries. After excision of the rudimentary left coronary cusp, the left coronary ostium appeared intact. An aortic valve replacement with annular enlargement using a bioprosthetic valve was performed. The patient uneventfully recovered without angina.

Congenital quadricuspid aortic valve detected on chest CT

A quadricuspid aortic valve is a very rare congenital anomaly, previously detected at autopsy, or more recently, on echocardiography. This case is only the second to be reported following detection on chest CT.

Congenital quadricuspid aortic valve in children

Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.

Congenital quadricuspid aortic valve associated with obstructive hypertrophic cardiomyopathy

Quadricuspid aortic valve is a rare congenital abnormality; it is usually an isolated lesion, but several concomitant congenital abnormalities have been described. We report a case of congenital quadricuspid aortic valve associated with obstructive hypertrophic cardiomyopathy. Two-dimensional (2D) transthoracic and transesophageal echocardiography and real-time three-dimensional (3D) echocardiography clarified the morphological and functional status of the aortic valve. To our knowledge, the association between quadricuspid aortic valve and obstructive hypertrophic cardiomyopathy has never been described before.

Endocarditis complicating a congenital quadricuspid aortic valve

The most common aortic valve congenital abnormality is observed in bicuspid aortic valve. Only a few cases of aortic valve quadricuspidy have been reported in the literature. We report a new case of endocarditis complicating a congenital quadricuspid aortic valve.

Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.

Ross Procedure in a Quadricuspid Aortic Valve

A quadricuspid aortic valve is a rare congenital cardiovascular abnormality, and when present, it is associated with aortic valve regurgitation. If aortic valve replacement is required, mechanical or biological prostheses are used. We report the case of a patient with a severely regurgitant quadricuspid aortic valve in whom a Ross procedure was performed.

Acute Occlusion of Left Coronary Ostium Associated With Congenital Quadricuspid Aortic Valve

A 10-year-old girl suffered from chest discomfort during exercise. She experienced an acute myocardial infarction accompanied by an obstruction in an orifice to the left coronary artery. An emergency operation revealed that the aortic valve was quadricuspid, and a left-sided small cusp had adhered to the aortic wall resulting in an unusual sac with a tiny slit. Resection of the sac disclosed the ostium to left coronary artery. Blood toward the left coronary artery passed through the tiny slit. The mechanisms of obstruction in the coronary artery were uncertain. She recovered well after the aortic valve replacement with resection of the aortic cusps.

Congenital Quadricuspid Aortic Valve Associated with Congenital Complete Heart Block

Congenital quadricuspid aortic valve is very uncommon, and is often associated with other cardiac disorders, such as patent ductus, ventricular septal defect, pulmonary stenosis, mitral valve malformation, hypertrophic cardiomyopathy and coronary abnormalities. We report a patient with congenital quadricuspid aortic valve associated with congenital complete heart block. To our knowledge, this association has not been reported so far. Copyright 2002 The European Society of Cardiology. Published by Elsevier Science Ltd. All rights reserved .

Embryological evidence for the formation of a quadricuspid aortic valve in the Syrian hamster

Congenital quadricuspid aortic valve is a rare anomaly, the morphogenesis of which remains unclear. In this study we report the case of a Syrian hamster embryo that presented an aortic valve with four valve cushions instead of three. The embryo, aged 12 days and 2 hours postcoitum, was at an early stage of valvulogenesis. It was examined using semithin sections of the heart. Two of the four valve cushions were located, one in the dorsal and the other in the left-ventral position, corresponding, respectively, to the dorsal and left valve cushions of a normal aortic valve. The remaining two cushions were situated in the right-ventral position. They were joined at the level of their basal portions and were less developed in size than the other cushions. This report seems to be the first embryological evidence for the formation of a quadricuspid aortic valve. The present findings strongly suggest that the anomalous valve originated from three mesenchymal anlagen and that the supernumerary valve cushion resulted from the division of the anlagen that normally gives rise to the right valve cushion.