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Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Background Quadricuspid aortic valve (QAV) is a rare congenital heart defect usually accompanied with different hemodynamic abnormalities. Due to the rarity of QAV, treatment and prognosis of QAV patients with aortic regurgitation still remain challenging. We here present the first case of a patient with severe QAV regurgitation who underwent successful treatment and performed favorable prognosis with transapical aortic valve implantation (TAVI) using J-Valve system. Case presentation A 62-year-old man experienced intermittent palpitation, shortness of breath and chest pain. Echocardiography revealed congenital QAV with massive aortic regurgitation and mild aortic stenosis, left ventricular enlargement. Aortic valve replacement was successfully performed with TAVI using J-Valve system. The postoperation and follow-up was uneventful. Conclusion TAVI using J-Valve system has emerged as a new high success rate method for treatment of patients with simple non-calcified aortic valve insufficiency.

Quadricuspid aortic valve, a rare congenital cardiac defect, manifests most commonly as aortic regurgitation. Clinical presentation mainly depends on the functional status of the aortic valve, myocardium and associated cardiovascular abnormalities. Aortic valve replacement or repair is usually warranted in the 5th or 6th decade.

A quadricuspid aortic valve is a very rare congenital heart condition that can present as aortic regurgitation in the 5th and 6th decade of life. The following case report will describe a patient who presented with symptoms of severe aortic regurgitation and was found to have a quadricuspid aortic valve on echocardiography. The case will describe the clinical manifestations in which the patient presented and the subsequent diagnosis of the quadricuspid aortic valve. The rationale for the surgical approach will also be discussed, along with the patient’s clinical response.

Graphical abstract, Highlights • Quadricuspid aortic valve is a rare finding in patients with aortic valvulopathy. • Patients over age 40 with this finding are more likely to have valve dysfunction. • Transesophageal echocardiogram is preferred to evaluate quadricuspid aortic valves.

Quadricuspid aortic valve (QAV) is a rare congenital valvular abnormality with less than 200 cases reported to date. The first QAV was reported in 1862 by Balington. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated cardiac disorders. Most QAV are asymptomatic and are incidentally found. Severe aortic regurgitation and/or stenosis can develop overtime, requiring aortic valve replacement or repair. Transesophageal echocardiography is the preferred modality to diagnose QAV. We present a case of QAV which was incidentally diagnosed in a 43-year-old woman who presented with gastroenteritis.

Background We present the case of a patient with severe quadricuspid aortic valve (QAV) stenosis who underwent treatment with transcatheter aortic valve implantation (TAVI). Case summary An 87-year-old woman was referred to our department with severe aortic stenosis. Computed tomography angiography revealed a QAV with four separate equal-sized leaflets, similar in appearance to a four-leaf clover. The patient underwent successful implantation of a 23 mm Edwards Sapien 3 transcatheter valve via left transfemoral access. The valve positioning across the aortic annulus was fluoroscopically challenging due to difficulty visualizing all four cusps in only one two-dimensional view and to the position of the left main coronary artery, which was very low. The immediate result of the TAVI was good, with no aortic regurgitation and no coronary damage. Follow-up to 6 months was free of event. Conclusion This case demonstrates that QAV stenosis can be treated using TAVI with good clinical outcomes.

No abstract available

Quadricuspid aortic valve (QAV) is a relatively rare valve abnormality. Patients with aortic valve regurgitation or stenosis associated with congenital abnormalities often require surgery at a relatively young age. In most cases, patients with QAV undergo aortic valve replacement. We report on a 58-year-old patient with QAV and aortic insufficiency due to enlarged aortic root. Valve-sparing aortic root replacement was performed without any procedure on the aortic leaflets.

Background: Quadricuspid aortic valve is a rare congenital heart disease that may be associated with coronary ostium anomalies. Care should be taken to avoid occluding or compressing the coronary ostium while performing aortic valve replacement.Case presentation: Herein, we report a case of a 59-year-old woman who underwent aortic valve replacement for a quadricuspid aortic valve with severe aortic regurgitation. Intraoperatively, the aortic valve had four cusps of almost equal size and the right coronary artery arose adjacent to one of the commissures. The annular stitches were placed in a non-everting mattress fashion with pledgets on the ventricular side, and stitches near the right coronary ostium were transitioned to the subannular ventricular myocardium to maintain the distance from the ostium. Further, we selected a small prosthesis because oversized prosthetic valve could potentially compress the right coronary ostium.Conclusions: While performing aortic valve replacement for a quadricuspid aortic valve associated with a right coronary ostium anomaly, careful selection of the size of the prosthesis and modification of the annular stitches are essential to prevent obstruction of the coronary ostium.

Quadricuspid aortic valve complicated with infective endocarditis is an uncommon clinical scenario. The indications for surgery and medical management do not differ from other types of aortic valve endocarditis. Commonly present structural abnormalities pose an increased risk of complete heart block and coronary occlusion during valve replacement. We present a case of quadricuspid aortic valve complicated with infective endocarditis, with surgical images of the valve.

An 84-year-old woman with severe aortic stenosis and regurgitation was referred to our institution for transcatheter aortic valve replacement (TAVR). Pre-procedural computed tomography (CT) revealed a type A quadricuspid aortic valve (QAV) (valve leaflets equally divided into 4) without severe

While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three-leaflet aortic valve is characterized by engineering advantages with superior long-term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age. AIM Evaluation of long-term performance, similarities, and differences between QAV and BAV. METHODS Screening of 19 000 consecutive echocardiographic studies was performed. RESULTS BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P

Quadricuspid aortic valve is rare and requires surgery when symptomatic severe regurgitation/stenosis is present. Associated anomalous coronary ostia location demands accurate diagnosis to avoid intraoperative complications, and several imaging techniques have been used, with drawbacks of low sensitivity, radiation and contrast exposure. We report a pre-operative assessment using 3-dimensional echocardiography. (Level of Difficulty: Intermediate.), Central Illustration

Aneurysms of the sinus of Valsalva are rare, with dissecting aneurysms of the sinus of Valsalva that extend into the interventricular septum being even more rare. This report describes a young patient with syphilis and a quadricuspid aortic valve who experienced a spontaneously dissecting aneurysm of the sinus of Valsalva and the basal interventricular septum.

Normal aortic valve has three leaflets. Quadricuspid aortic valve(QAV) is a rare congenital anomaly of aortic valve in which there are four cusps. These patients should be diagnosed and clinically monitored as they are prone to developing aortic regurgitation in their life. Other structural anomalies and aortic dilation are also common in these patients. Advances in cardiac imaging have resulted in more and earlier detection of QAV.

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. Resumo: A válvula aórtica quadricúspide é uma malformação congênita rara. A progressão para insuficiência aórtica com significado clínico é frequente na idade adulta. O tratamento cirúrgico, quando indicado, tem habitualmente lugar por volta da quinta ou sexta décadas de vida em cerca de um quinto dos doentes.Descrevemos o caso clínico de uma doente de 64 anos com diagnóstico de regurgitação valvular aórtica severa e deterioração clínica e ecocardiográfica, reunindo critérios para cirurgia convencional. Durante a cirurgia, observou-se uma válvula aórtica composta por quatro folhetos independentes, retraídos e com má coaptação central. O procedimento decorreu sem intercorrências.

We present an exceptional case of a quadricuspid aortic valve associated with a left atrial myxoma. Both are rare conditions, and this association has not been reported yet. These conditions can be silent but may lead to several complications. This case highlights importance of a careful echocardiographic evaluation for early management. (Level of Difficulty: Beginner.), Graphical abstract

A 39-year-old male patient from a county health facility presented to our department with a one-year history of progressive exertional chest tightness (NYHA class III-IV), which resolved completely within five to six minutes of rest. Both motion and static imaging results revealed a rare solitary Quadricuspid aortic valve (QAV) with severe aortic valve regurgitation. The QAV was replaced with mechanical prosthetic valve via mini-superior sternotomy on cardiopulmonary bypass machine. The patient was reviewed a month after operation, and assessment revealed that he had reverted to NYHA class-I. A rare congenital lesion such as QAV, repair may not be the best option due to lack of long-term data on longevity. Additionally, choice of a mechanical prosthetic valve guarantees unwanted re-operations associated with possible failure of bioprosthetic valves.

A 70-year-old male underwent transthoracic (TTE) and transesophageal (TEE) echocardiography for a stroke. A bicuspid aortic valve was suspected by TTE, but TEE revealed a normally functioning quadricuspid aortic valve. A quadricuspid aortic valve may be more common than generally thought, as it may not be readily diagnosed by TTE, and may remain functionally normal.

The quadricuspid aortic valve is an exceedingly rare congenital valvular anomaly, characterized by a tetrad of leaflets that typically presents with aortic regurgitation. Almost one third of cases are associated with coexisting cardiac defects with one fifth warranting surgical intervention. In this article, we describe the first documented-in-Caribbean case and present a brief clinical review of its pathophysiology, diagnosis, and management.

Quadricuspid aortic valve is a rare entity. Left ventricular noncompaction is an uncommon cause of cardiomyopathy. Combination of these two conditions is very rare. We report a case of quadricuspid aortic valve and moderate aortic regurgitation with left ventricular noncompaction who had presented with ventricular tachycardia.

This case report describes a 55-year-old male who presented with acute Type A aortic dissection. He underwent emergent surgical repair, and his intraoperative transesophageal echocardiography revealed a quadricuspid aortic valve. His aortic root measured 45 mm. Quadricuspid aortic valves have previously been associated with aortic root dilation. This case illustrates the possible association of quadricuspid aortic valves with aortic dissection, similar to what is described with bicuspid valves.

Graphical abstract, Highlights • QAV is a rare congenital cardiac malformation. • QAV with ostium secundum atrial septal defect. • Hurwitz and Roberts's anatomic classification of QAV. • More than 50% of people with QAV require valve repair or replacement.

Graphical abstract, Highlights • Quadricuspid aortic valve is an uncommon congenital cardiac malformation often discovered incidentally at the time of valve surgery or at autopsy. • It is commonly associated with progressive aortic regurgitation, with nearly half of affected patients requiring aortic valve replacement surgery during their lifetime. • Dilatation of the aortic root and ascending aorta has also been reported in a significant number of affected patients.

Infective endocarditis complicating a quadricuspid aortic valve (QAV) is rare. Previous reports highlight the increased risk for complications, including progressive aortic regurgitation, decompensated heart failure, and valve perforation. Thus, cardiologists must be able to quickly identify QAVs to guide rapid evaluation and treatment. We report a case of infective endocarditis in a QAV identified on echocardiography and effectively managed with medical therapy alone without complications over an 8-year follow-up period. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:145-148, 2018.

Quadricuspid aortic valve has been classified based on leaflet size. However, no association is seen between classification and severity of aortic regurgitation (AR). Bicuspid aortic valve is classified according to the number of cusps, with significantly higher prevalence of AR in cases with a raphe. We classified cases according to raphe number. In 1 patient with no raphe, AR severity did not change into the eighth decade. However, AR severity worsened in patients with a raphe, in 1 case requiring aortic valve replacement in the fifth decade. Unequal shear stress may lead to leaflet fibrosis and progressive AR.

Key Clinical Message The quadricuspid aortic valve (QAV) is a malformation that leads to severe valve failure later in life. Malformation and displacement of coronary ostia are found in 10% of patients. In this context, transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are the essential imaging tests for the preoperative assessment for cardiac surgery.

A quadricuspid aortic valve (QAV) is an exceedingly rare congenital heart anomaly with around 200 cases reported in the literature since the first case was reported in 1862. To the best of our knowledge, there has not been any case of QAV associated with pregnancy. We report a case of a 29-year-old female with new-onset palpitations, diagnosed with QAV and mild aortic regurgitation during pregnancy. The patient presented with new-onset intermittent palpitations at the 37th week of pregnancy. Electrocardiogram (EKG) showed normal sinus rhythm, and transthoracic echocardiography revealed a quadricuspid aortic valve with three equal-sized cusps and one smaller cusp and mild aortic regurgitation without any additional anomalies. QAV morphology falls under the category type b Hurwitz & Roberts classification. She underwent normal vaginal delivery without any peripartum cardiac complications. In conclusion, QAV is a rare congenital anomaly. It is not uncommon to be associated with aortopathies. The presence of QAV and associated anomalies in pregnancy makes it a high-risk state. Close monitoring, especially during the second and third trimesters, remain of utmost importance. Because of its rarity, the characteristics, natural history, and long-term outcomes of QAV are poorly defined.

Sinus of Valsalva rarely ruptures due to congenital causes. When it comes to ruptured sinus of Valsalva combined with quadricuspid aortic valve, no cases have so far been reported in Japan. Here, we describe the case of 32-year-old female who developed ruptured sinus of Valsalva with tumor-like appearance. Some of the cases of ruptured sinus of Valsalva show aorta to right atrial tunnel with a windsock aneurysm looking like a tumor by echocardiography. Learning objective: Sinus of Valsalva rarely ruptures due to congenital causes such as aortic valve anomalies such as quadricuspid aortic valve. Ruptured sinus of Valsalva sometimes shows tumor-like appearance by echocardiography. In such a case, we need to keep in mind that the sinus of Valsalva with aortic valve anomalies rarely ruptured showing a fistulous pouch-like “windsock” in the edge of aneurysm by echocardiography.>

Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect characterized by the presence of four aortic valve leaflets of equal or varying sizes. Even rarer is its clinical presentation with aortic stenosis. Diagnosis of QAV could be challenging but is of great importance as patients often present with progressive aortic regurgitation. We present 2 cases of QAV presenting differently: one with aortic stenosis requiring valve replacement and the other with aortic regurgitation requiring close monitoring.

A47-YEAR-OLD MAN presented to the authors’ institution for an elective surgical closure of an atrial septal defect (ASD) after a failed attempt at percutaneous closure (Fig 1, Panel A). He was known to have a peri-membranous ventricular septal defect (VSD) and a quadricuspid aortic valve (Fig 1, Panels B and C; Video 1). On the day of surgery, after uneventful induction of general anesthesia, an intraoperative transesophageal echocardiography (TEE) examination was performed, which confirmed the presence of an ASD, a nonrestrictive peri-membranous VSD, and a quadricuspid aortic valve. These findings were confirmed with real-time, 3-dimensional (3D) TEE imaging (Fig 2; Video 2).

Quadricuspid aortic valve is a very rare congenital valvular anomaly. Most of these cases present with aortic insufficiency. We present a 38 year old male patient with aquadricuspid aortic valve with severe aortic regurgitation.