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We describe a 30-year-old Caribbean-Black woman with a clinical presentation suggestive of a transient ischemic attack (TIA) with no conventional cerebrovascular risk factors, albeit with a newly diagnosed quadricuspid aortic valve (QAV) with moderate aortic regurgitation (AR). Although QAV is a recognized congenital cardiac defect, its association with TIA remains elusive. This case highlights the importance of considering potential atypical etiologies, such as QAV, in the evaluation and management of young patients presenting with cerebrovascular events. [ABSTRACT FROM AUTHOR]

Abstract Background Developmental abnormalities of aortic valve cusps are relatively common with the bicuspid valve being the most frequently encountered congenital heart disease. However, the quadricuspid aortic valve (QAV) is an exceedingly rare abnormality. Case presentation We report a case involving a young, otherwise healthy male who presented with non-exertional syncope and was subsequently diagnosed with complete heart block (CHB). Further evaluation revealed the coexistence of a rare quadricuspid aortic valve and CHB. This combination, in the absence of surgery or infective endocarditis, has only been reported once before in the literature.The patient underwent successful permanent pacemaker implantation and continues to be monitored for aortic regurgitation. Conclusions The coexistence of a QAV with CHB, in the absence of infective endocarditis or aortic valve surgery, is extremely rare and necessitates careful evaluation and follow-up.

Abstract 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.

Background: Developmental abnormalities of aortic valve cusps are relatively common with the bicuspid valve being the most frequently encountered congenital heart disease. However, the quadricuspid aortic valve (QAV) is an exceedingly rare abnormality. Case presentation: We report a case involving a young, otherwise healthy male who presented with non-exertional syncope and was subsequently diagnosed with complete heart block (CHB). Further evaluation revealed the coexistence of a rare quadricuspid aortic valve and CHB. This combination, in the absence of surgery or infective endocarditis, has only been reported once before in the literature.The patient underwent successful permanent pacemaker implantation and continues to be monitored for aortic regurgitation. Conclusions: The coexistence of a QAV with CHB, in the absence of infective endocarditis or aortic valve surgery, is extremely rare and necessitates careful evaluation and follow-up. [ABSTRACT FROM AUTHOR]

Abstract Background Quadricuspid aortic valve (QAV) is a rare congenital anomaly characterized by the presence of four cusps instead of the usual three. It is estimated to occur in less than 0.05% of the population, with Type A (four equal-sized leaflets) accounting for roughly 30% of QAV subtypes. Based on limited clinical series, the usual presentation is progressive aortic valve regurgitation (AR) with symptoms occurring in the fourth to sixth decade of life. Severe aortic valve stenosis (AS) and acute AR are very uncommon. Case presentation We describe two cases of Type A QAV in patients who remained asymptomatic until their seventies with very uncommon presentations: one with severe AS and one with acute, severe AR and flail leaflet. In Case A, a 72-year-old patient with history of moderate AS presents to clinic with progressive exertional dyspnea. During work-up for transcatheter vs. surgical replacement pre-operative computed tomography angiogram (CTA) reveals a quadricuspid aortic valve with severe AS, and the patient undergoes surgical aortic valve replacement. Pre-discharge transthoracic echocardiography (TTE) shows good prosthetic valve function with no gradient or regurgitation. In Case B, a 76-year-old patient is intubated upon arrival to the hospital for acute desaturation, found to have wide open AR on catheterization, and transferred for emergent intervention. Intraoperative TEE reveals QAV with flail leaflet and severe AR. Repair is considered but deferred ultimately due to emergent nature. Post-operative TTE demonstrates good prosthetic valve function with no regurgitation and normal biventricular function. Conclusions QAV can present as progressive severe AS and acute AR, with symptoms first occurring in the seventh decade of life. The optimal treatment for QAV remains uncertain. Although aortic valve repair or transcatheter option may be feasible in some patients, aortic valve replacement remains a tenable option.

Quadricuspid aortic valve is a rare congenital cardiac anomaly with an incidence of 0.008% to 0.043%. Its clinical course varies depending on cusp anatomy, function, and associated cardiac malformations. It frequently progresses to aortic valve regurgitation that may require surgical valve replacement. Detection has shifted from incidental discovery during autopsies or cardiac surgeries in the early 20th century to various cardiac imaging methods in recent decades. In addition to contributing to the literature, this report supports the use of transesophageal echocardiography more liberally to detect aortic valve abnormalities. The case presents a 48-year-old female patient with an incidentally discovered quadricuspid aortic valve.

Background: Quadricuspid aortic valve (QAV) is a rare congenital anomaly characterized by the presence of four cusps instead of the usual three. It is estimated to occur in less than 0.05% of the population, with Type A (four equal-sized leaflets) accounting for roughly 30% of QAV subtypes. Based on limited clinical series, the usual presentation is progressive aortic valve regurgitation (AR) with symptoms occurring in the fourth to sixth decade of life. Severe aortic valve stenosis (AS) and acute AR are very uncommon. Case presentation: We describe two cases of Type A QAV in patients who remained asymptomatic until their seventies with very uncommon presentations: one with severe AS and one with acute, severe AR and flail leaflet. In Case A, a 72-year-old patient with history of moderate AS presents to clinic with progressive exertional dyspnea. During work-up for transcatheter vs. surgical replacement pre-operative computed tomography angiogram (CTA) reveals a quadricuspid aortic valve with severe AS, and the patient undergoes surgical aortic valve replacement. Pre-discharge transthoracic echocardiography (TTE) shows good prosthetic valve function with no gradient or regurgitation. In Case B, a 76-year-old patient is intubated upon arrival to the hospital for acute desaturation, found to have wide open AR on catheterization, and transferred for emergent intervention. Intraoperative TEE reveals QAV with flail leaflet and severe AR. Repair is considered but deferred ultimately due to emergent nature. Post-operative TTE demonstrates good prosthetic valve function with no regurgitation and normal biventricular function. Conclusions: QAV can present as progressive severe AS and acute AR, with symptoms first occurring in the seventh decade of life. The optimal treatment for QAV remains uncertain. Although aortic valve repair or transcatheter option may be feasible in some patients, aortic valve replacement remains a tenable option. [ABSTRACT FROM AUTHOR]

Background: Quadricuspid aortic valve (QAV) is a rare congenital disease. The clinical characteristics of this disease remain unclear except for those in relatively young patients reported from tertiary referral hospitals. The aim of this study was to determine the clinical features of QAV in a regional population. Methods and Results: We retrospectively investigated 25 340 consecutive patients over middle age (median age, 73 (IQR 65-80) years; range, 45-102 years) who underwent transthoracic echocardiography (TTE) at our institute during the period from April 2008 to December 2023. Eight (0.032%) of the patients (median age, 65 years; range, 47-91 years) were diagnosed withQAV. Six patients suffered from aortic regurgitation (AR), and one patient had mild aortic stenosis at the time of QAVd iagnosis. Two patients who had severe AR at referral underwent aortic valve surgery. The severity of AR in the other patients was moderate or less. During a median follow-up period of 27 months (range, 1-171 months), none of the patients other than above two patients had cardiac events. One patient died from a non-cardiac cause at 94 years of age. Conclusions: Patients diagnosed with QAV after middle age, who do not exhibit severe valve insufficiency at the time of diagnosis, may not experience worse clinical outcomes. However, further research is required for a better understanding of the long-term outcomes. [ABSTRACT FROM AUTHOR]

Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes. Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROA CT was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3–62.8). ROA CT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm2, HR ​= ​4.25, 95%CI 1.49–12.17, p ​= ​0.007; as a continuous variable (per mm2 increment), HR ​= ​1.04, 95%CI 1.01–1.07, p ​= ​0.003). Additionally, ROA CT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004). QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROA CT may be a potential ancillary prognostic marker in patients with QAV. [Display omitted] [ABSTRACT FROM AUTHOR]

Background The presence of severe aortic stenosis in quadricuspid aortic valve (QAV) is an extremely rare combination, and it is unknown whether transcatheter aortic valve replacement (TAVR) is a safe option due to the low incidence. Case summary We present two patients diagnosed with severe aortic stenosis with QAV morphology type 1 (Nakamura classification). All patients presented to our hospital for evaluation because of worsening functional class, dyspnoea, or syncope. During tomographic planning, the aortic annulus was measured at the level of the deepest sinus for the selection of the number of devices. Due to the presence of four cusps, the smallest cusp was excluded, and three sinuses were virtualized for placement of the pigtail catheter during the procedure. Without complications, a 23 mm Edwards SAPIEN 3 was deployed through the femoral artery in both patients. Control aortography showed no valve leakage or regurgitation. Discussion In patients with QAV and aortic stenosis undergoing TAVR, similar to the tricuspid valve, tomographic planning can be used to ensure the success of the procedure. However, unlike the tricuspid valve, where the selection of the device number is based on the measurements of the aortic annulus at the level of the non-coronary sinus, in these QAV cases, we perform the measurements at the level of the deepest aortic sinus (right coronary sinus). [ABSTRACT FROM AUTHOR]

Abstract Aortic valve malformation is a common congenital abnormality reported in human medicine. The malformation is characterised by an increased or decreased number of cusps. Anatomical variations of the aortic valve that have been documented in humans include unicuspid, bicuspid, quadricuspid and quinticuspid valves. Two reports described a quadricuspid aortic valve in horses associated with either a ventricular septal defect (VSD) or tetralogy of Fallot. In this case report we describe the clinical and echocardiographic findings of a horse with a quadricuspid aortic valve as single congenital abnormality, referred with history of exercise intolerance and an episode of paroxysmal atrial fibrillation. Limitations and risks of misdiagnosis that can be encountered with transthoracic echocardiography are also discussed. The reported case highlights the importance of echocardiographic screening in asymptomatic patients as congenital heart disease can be present without obvious cardiac signs. As advanced imaging on the equine thorax is still far from future possibilities for adult horses, this report may help to reach an accurate diagnosis with similar cases.

The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography.

The article in the Oxford Medical Case Reports discusses a case of successful implantation of a balloon-expandable transcatheter aortic valve in an 83-year-old man with quadricuspid aortic valve stenosis. The patient had severe aortic stenosis and regurgitation, and due to his advanced age and high surgical risk, the heart team decided to perform transcatheter aortic valve implantation. The procedure resulted in significant improvement in the patient's condition, with no clinical events reported during a two-year follow-up period. The article emphasizes the importance of selecting the appropriate transcatheter heart valve based on the characteristics of the aortic valve for safe performance of the procedure. [Extracted from the article]

A quadricuspid aortic valve is a rare congenital anomaly whose diagnosis is mostly incidental. The overall incidence in the population is less than 0.00033% in autopsies and less than 0.0065% in transthoracic echocardiography. Most patients present with valvular pathologies, the most prominent of this being aortic regurgitation. The following case report presents a 60-year-old female patient with severe aortic insufficiency. Her symptoms were rapidly progressing and she had a clear indication for valvular replacement. In the preoperative transthoracic and transesophageal echocardiography there is no evidence of a four aortic cusp. However, during the surgical procedure a native aortic quadricuspid valve was found. During surgery a fourth aortic cusp is identified. The native aortic valve was exchanged for a biological prosthetic valve. Postoperative ecography demostrates a prosthetic aortic valve with normal function. A quadricuspid aortic valve is a rare anatomic variation that can result in aortic insufficiency due to mechanical valvular anomalies. [ABSTRACT FROM AUTHOR]

Aortic valve malformation is a common congenital abnormality reported in human medicine. The malformation is characterised by an increased or decreased number of cusps. Anatomical variations of the aortic valve that have been documented in humans include unicuspid, bicuspid, quadricuspid and quinticuspid valves. Two reports described a quadricuspid aortic valve in horses associated with either a ventricular septal defect (VSD) or tetralogy of Fallot. In this case report we describe the clinical and echocardiographic findings of a horse with a quadricuspid aortic valve as single congenital abnormality, referred with history of exercise intolerance and an episode of paroxysmal atrial fibrillation. Limitations and risks of misdiagnosis that can be encountered with transthoracic echocardiography are also discussed. The reported case highlights the importance of echocardiographic screening in asymptomatic patients as congenital heart disease can be present without obvious cardiac signs. As advanced imaging on the equine thorax is still far from future possibilities for adult horses, this report may help to reach an accurate diagnosis with similar cases. [ABSTRACT FROM AUTHOR]

Abstract Background Quadricuspid aortic valve is a rare congenital heart disease that may be associated with a different anatomical relationship between the coronary artery ostium and the commissure. 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 the commissure between the right coronary cusp and one of the two non-coronary cusps. 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. A one-step smaller-sized prosthesis was selected to avoid an oversized prosthetic valve potentially compressing the right coronary ostium. Conclusions When performing aortic valve replacement for a quadricuspid aortic valve, careful observation of the coronary location and means to avoid coronary ostium obstruction are essential.

Quadricuspid aortic valve (QAV) and sinus of Valsalva aneurysm (SVA) are rare congenital anomalies. We report an elderly patient with QAV associated with a ruptured SVA to the right atrium. Transthoracic echocardiographic and computed tomographic images are presented. We emphasize the important role of computed tomography angiography in establishing and confirming the diagnosis and facilitating treatment planning. The patient was successfully operated by a minimally invasive approach. [ABSTRACT FROM AUTHOR]

Background: We present the case of a patient who underwent successful transapical aortic valve implantation in a severe quadricuspid aortic valve (QAV) with severe regurgitation and multiorgan failure. Case Summary: A 57‐year‐old man experienced intermittent palpitation and shortness of breath for 6 months. The condition deteriorated in the past month and caused multiorgan failure. The echocardiography and computed tomography angiography revealed severe aortic regurgitation due to congenital QAV. The aortic valve replacement was successfully performed in this high‐risk patient using a J‐Valve system. Postoperation and follow‐up were uneventful. Conclusion: The J‐Valve system effectively treated QAV regurgitation with good clinical outcomes in this case. [ABSTRACT FROM AUTHOR]

Subvalvular aortic stenosis typically manifests at a young age and rarely presents in adulthood. It may cause left ventricular outflow tract stenosis, which requires surgical treatment in severe cases. The coexistence of discrete subvalvular aortic stenosis and quadricuspid aortic valve is a highly unusual finding. We present a case of a patient with coexisting discrete subvalvular aortic stenosis and quadricuspid aortic valve, who underwent aortic valve plasty and subvalvular aortic stenotomy. A 63-year-old woman with a history of shortness of breath on exertion was referred to our hospital after being diagnosed with discrete subvalvular aortic stenosis and quadricuspid aortic valve at another hospital. Echocardiography revealed membranous tissue below the aortic valve and a quadricuspid aortic valve. Dobutamine stress echocardiography showed a mean pressure gradient of 75 mmHg and Vmax of 5.9 m/s in the subaortic membranous area. The subaortic valve membranous structures were first resected during surgery to release the subaortic stenosis. The aortic valve had four cusps with an accessory cusp between the right and left coronary cusps. Next, the right coronary cusp was sutured to the accessory cusp and converted to a single valve. Intraoperative transesophageal echocardiography showed trivial aortic regurgitation. The intraoperatively resected subvalvular tissue contained fibrous connective tissue with fibrous thickening and mucinous degeneration. At one-year postoperative follow-up, there is no subvalvular aortic stenosis and aortic regurgitation recurrence, and the patient is doing well. Long-term outcomes of aortic valvuloplasty for quadricuspid aortic valves are not well-characterized in the literature. Owing to the high relapse rate of subvalvular aortic stenosis, rigorous follow-up with echocardiography every six to twelve months is essential to evaluate the long-term success of aortic valve plasty., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Research ethics review committee of ayase heart hospital issued approval Approval No. 2024-0002-1. This study was approved by the Research Ethics Review Committee of Ayase heart hospital in Japan (Approval No. 2024-0002-1). Written informed consent was obtained from patient. . Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Saitoh et al.)

Introduction: Quadricuspid aortic valve (QAV) is an extremely rare developmental abnormality with an incidence of 0.006%. QAV is an incidental finding that in some patients (23%) may determine aortic regurgitation (AR). Altogether 16% of patients indeed require surgery with AR being the most frequent indication. Methods and results: We describe a case report of a 46 year‐old female affected by severe aortic regurgitation due to QAV successfully treated with a modified‐tricuspidization technique associated with cusp extension, prolapsing commissure suturing, and sub‐commissural annuloplasty. Discussion: QAV repair represents an attractive perspective to overcome the drawbacks of either mechanical or biological prosthesis. [ABSTRACT FROM AUTHOR]

BackgroundQuadricuspid aortic valve (QAV) is a rare congenital malformation that can present with aortic regurgitation or aortic stenosis (AS)), requiring surgical treatment. Transcatheter aortic valve replacement (TAVR) is an alternative treatment for older patients and its prognosis for QAV therapy remains challenging. We sought to examine our early experience with TAVR in patients with QAV.Materials and methodsProspectively collected data were retrospectively reviewed in patients with QAV undergoing TAVR in our institution.ResultsFive patients with QAV and AR or AS were treated with TAVR between January 2016 and January 2022. The mean age was 73.8 years (range 69–82 years), and the median Society of Thoracic Surgeons score was 7.51% (range 2.668–18.138%). Two patients had type B and three had either type A, D, or F according to the Hurwitz and Roberts classification for QAV. Four patients with pure aortic regurgitation underwent transapical TAVR using the J-Valve system, and the patient with severe AS underwent transfemoral TAVR using the Venus-A system. Procedural success was achieved in all five patients. Trivial paravalvular leak was only detected in one case after the procedure, and one patient received a permanent pacemaker due to high-degree atrioventricular block three days later. The median follow-up period was 18 (12–56) months. After discharge, no deaths occurred during the 1 year follow-up. All patients improved by ≥1 New York Heart Association functional class at 30 days; four patients were in functional class ≤II later in the follow-up period. All patients’ heart failure symptoms improved considerably.ConclusionOur early experience with TAVR in QAV demonstrates these procedures to be feasible with acceptable early results. Further follow-up is necessary to determine the long-term outcomes of this modality.Clinical trial registration[ClinicalTrials.gov], identifier [NCT02917980].

Abstract 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.

Association of quadricuspid aortic valve (QAV) with ascending aortic aneurysms (AsAA) is rare. A 63-year-old female with hypertension was found (on MRI) to have an ascending aortic aneurysm (52 mm in maximum diameter) and dilatation at the level of the sinotubular junction (38 mm in diameter) associated with quadricuspid aortic valve. An ascending aortic wall replacement surgery was performed. In this study, we focus on the behavior of the aorta associated with QAV considering the in vitro biomechanical characteristics and histology. The properties of QAV are closer to bicuspid aortic valve than tricuspid aortic valve, but with higher wall thickness. [ABSTRACT FROM AUTHOR]

Background: Quadricuspid aortic valve is a rare congenital heart disease that may be associated with a different anatomical relationship between the coronary artery ostium and the commissure.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 the commissure between the right coronary cusp and one of the two non-coronary cusps. 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. A one-step smaller-sized prosthesis was selected to avoid an oversized prosthetic valve potentially compressing the right coronary ostium.Conclusions: When performing aortic valve replacement for a quadricuspid aortic valve, careful observation of the coronary location and means to avoid coronary ostium obstruction are essential. [ABSTRACT FROM AUTHOR]

The presence of a quadricuspid aortic valve is extremely rare. It is a congenital anomaly that is recognized relatively late, approximately in the fifth or sixth decade of life. Although it is an anatomically differently structured valve, it does not necessarily represent a disease. However, it can be a prerequisite for the development of a serious valvular defect. Given the long-preserved functional status of the heart, the exact incidence is unclear. This is evidenced by the fact that it is most often recognized during cardiac surgery. The most commonly described quadricuspid aortic valve defect is aortic regurgitation, and the most common concomitant anomaly is an aneurysm of the ascending aorta. We present the case of a 68-year-old patient with paroxysmal atrial fibrillation for whom we demonstrated a quadricuspid aortic valve by echocardiography. [ABSTRACT FROM AUTHOR]

The quadricuspid aortic valve (QAV) is a rare congenital anomaly that typically is unrelated to other cardiac anomalies. It usually is discovered incidentally through imaging modalities such as echocardiogram or computed tomography angiogram, during surgery, or autopsy. Some patients with QAV develop aortic regurgitation and, rarely, other cardiovascular complications like aortic aneurysm. Due to its rarity, it is difficult to characterize these patients or standardize management. However, review of case reports can be very useful in rare medical conditions such as QAV. In this manuscript, in addition to presenting two cases of QAV, the authors reviewed 149 cases of QAV from 2010 to 2020 from published case studies and series in order to gain a better insight into the characteristics of the patient population with QAV and its management. [ABSTRACT FROM AUTHOR]

Abstract Background Dengue fever is endemic and a leading health problem in Sri Lanka. Increased incidence of concurrent bacteremia in patients with dengue infection is a recognized complication. However, Staphylococcal endocarditis following dengue fever is uncommon. Quadricuspid aortic valve (QAV) is a rare congenital anomaly and few cases of infective endocarditis have been reported in QAV. Case presentation A 32-year-old Sri Lankan male presented to the National Hospital of Sri Lanka with recurrence of fever and acute left hemiplegia following an uncomplicated recovery of dengue fever. He was diagnosed to have Staphylococcal infective endocarditis of quadricuspid aortic valve, with septic emboli to brain and spleen. He was managed with intravenous vancomycin initially, however, due to inadequate response, intravenous linezolid was added. He developed rhabdomyolysis with very high creatine phosphokinase leading to acute kidney injury, which settled with the cessation of linezolid. The patient succumbed to his illness despite aggressive antimicrobial therapy and maximum supportive care while being assessed for aortic valve replacement. Conclusions This case illustrates three clinical issues that a clinician should be aware of. Firstly, the possibility of a serious secondary bacterial infection as a cause for recurrence of fever following dengue infection. Secondly, this case highlights the importance of identifying QAV as a cause for complicated infective endocarditis of increased severity. The report also denotes the value of being vigilant of linezolid induced rhabdomyolysis which had a causal relationship with the commencement of the drug and its cessation.

Abstract Quadricuspid aortic valve (QAV) and sinus of Valsalva aneurysm (SVA) are rare congenital anomalies. We report an elderly patient with QAV associated with a ruptured SVA to the right atrium. Transthoracic echocardiographic and computed tomographic images are presented. We emphasize the important role of computed tomography angiography in establishing and confirming the diagnosis and facilitating treatment planning. The patient was successfully operated by a minimally invasive approach.

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. [ABSTRACT FROM AUTHOR]

We report a rare case of quadriscuspid aortic valve in a 28-year-old healthy man admitted to hospital after a resuscitated cardiac arrest. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aims Quadricuspid aortic valve (QAV) is a rare abnormality, which may cause aortic regurgitation (AR) requiring surgical intervention in some patients. The characteristics associated with aortic valve functional degeneration in patients with QAV are still unknown. The aim of this study is to describe QAV prevalence, characterize the disease by multimodality imaging, evaluate predictors of severe AR, and assess mid-term prognosis. Methods and results Retrospective search in imaging exams database of one tertiary centre, for patients diagnosed with QAV between January 2007 and September 2019. QAV was characterized by cardiac computed tomography, transthoracic/transoesophageal echocardiography, and cardiac magnetic resonance. A total of 160 004 exams were reviewed and eight patients with QAV were identified (50% men, mean age 53.5 ± 10.7 years). The prevalence of QAV was 0.005%. During a median follow-up of 52 months (interquartile range 16–88), there were no deaths. Seven patients (88%) had pure AR (three severe, one moderate, and three mild) and one patient (12%) had moderate AR and moderate aortic stenosis. Three patients (38%) with severe AR underwent valve surgery (two replacements and one repair). Analysis of predictors of severe AR was not statistically significant. Conclusion QAV is a rare congenital cardiac defect, with a prevalence of 0.005% in our study. Its predominant functional abnormality was regurgitation and about one-third of the patients required aortic valve surgery. Multimodality imaging may play a pivotal role in assessing patients with QAV with significant valve dysfunction or associated congenital heart disease and improve their treatment strategy. [ABSTRACT FROM AUTHOR]

The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography [ABSTRACT FROM AUTHOR]

We report the case of an 80-year-old woman with known quadricuspid aortic valve (QAV), severe aortic stenosis, and moderate-to-severe regurgitation who recently underwent a transcatheter aortic valve replacement (TAVR).

Abstract Background Integrated transthoracic and transesophageal echocardiography enables identification and characterization of a quadricuspid aortic valve anomaly. Case presentation A totally asymptomatic 40-year-old white man was referred to our Division of Cardiology after accidental finding of a heart murmur. Transesophageal echocardiography detected a quadricuspid aortic valve characterized by four cusps of equal size and severe aortic valvular regurgitation, without any further anomalies. He underwent a successful aortic valve repair. Conclusions Quadricuspid aortic valve anomaly is a rare congenital cardiac defect that can cause progressive valvular complications.

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.

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.

We present a successful case of the management of a 46-year-old woman with an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp; type B according to the Hurwitz and Roberts classification) with a marked loss of coaptation that caused a severe aortic insufficiency (AI). The patient underwent an aortic valvuloplasty of the defect using the glutaraldehyde (GA)-pretreated autologous pericardium, restoring the subnormal function and competency of the aortic valve. The postoperative course was unremarkable. The early follow-up showed a trivial AI and a significant reduction of regurgitant volume was identified in a cardiac magnetic resonance imaging (MRI) when compared to the preoperative state (27.2 vs. 2.1 ml). The follow-up transthoracic echocardiography 1 year after the surgery showed mild AI. [ABSTRACT FROM AUTHOR]

Background: Dengue fever is endemic and a leading health problem in Sri Lanka. Increased incidence of concurrent bacteremia in patients with dengue infection is a recognized complication. However, Staphylococcal endocarditis following dengue fever is uncommon. Quadricuspid aortic valve (QAV) is a rare congenital anomaly and few cases of infective endocarditis have been reported in QAV.Case Presentation: A 32-year-old Sri Lankan male presented to the National Hospital of Sri Lanka with recurrence of fever and acute left hemiplegia following an uncomplicated recovery of dengue fever. He was diagnosed to have Staphylococcal infective endocarditis of quadricuspid aortic valve, with septic emboli to brain and spleen. He was managed with intravenous vancomycin initially, however, due to inadequate response, intravenous linezolid was added. He developed rhabdomyolysis with very high creatine phosphokinase leading to acute kidney injury, which settled with the cessation of linezolid. The patient succumbed to his illness despite aggressive antimicrobial therapy and maximum supportive care while being assessed for aortic valve replacement.Conclusions: This case illustrates three clinical issues that a clinician should be aware of. Firstly, the possibility of a serious secondary bacterial infection as a cause for recurrence of fever following dengue infection. Secondly, this case highlights the importance of identifying QAV as a cause for complicated infective endocarditis of increased severity. The report also denotes the value of being vigilant of linezolid induced rhabdomyolysis which had a causal relationship with the commencement of the drug and its cessation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Bicuspid aortic valve (BAV) is the most common human congenital cardiac malformation. Although the etiology is unknown for most patients, formation of the 2 main BAV anatomic types (A and B) has been shown to rely on distinct morphogenetic mechanisms. Animal models of BAV include 2 spontaneous hamster strains and 27 genetically modified mouse strains. To assess the value of these models for extrapolation to humans, we examined the aortic valve anatomy of 4340 hamsters and 1823 mice from 8 and 7 unmodified strains, respectively. In addition, we reviewed the literature describing BAV in nonhuman mammals. The incidences of BAV types A and B were 2.3% and 0.03% in control hamsters and 0% and 0.3% in control mice, respectively. Hamsters from the spontaneous model had BAV type A only, whereas mice from 2 of 27 genetically modified strains had BAV type A, 23 of 27 had BAV type B, and 2 of 27 had both BAV types. In both species, BAV incidence was dependent on genetic background. Unlike mice, hamsters had a wide spectrum of aortic valve morphologies. We showed interspecific differences in the occurrence of BAV between humans, hamsters, and mice that should be considered when studying aortic valve disease using animal models. Our results suggest that genetic modifiers play a significant role in both the morphology and incidence of BAV. We propose that mutations causing anomalies in specific cardiac morphogenetic processes or cell lineages may lead to BAV types A, B, or both, depending on additional genetic, environmental, and epigenetic factors. [ABSTRACT FROM AUTHOR]

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.)

Abstract Background The presence of a fourth aortic valve cusp (quadricupsid aortic valve) is a rare congenital malformation and is often accompanied by other anomalies of the adjacent cardiovascular structures. Among these concomitant anomalies, simultaneous association of both a single coronary ostium and aneurysmal ascending aortic dilation in combination with the quadricupsid aortic valve has not been reported yet. Case presentation We experienced the case of a 56-year-old female patient presenting as aortic regurgitation resulted from malcoaptation of quadricupsid aortic valve. The patient had also accompanying aneurysmal ascending aortic dilatation and coronary ostial anomaly. Surgical correction (aortic valve replacement with mechanical devices and supracoronary aortic replacement with prosthetic graft) was performed without any complications. Conclusions The technological development of preoperative imaging studies enable the physician to encounter the quadricuspid aortic valve with other associated malformations more often unlike previous reports. With review on the quadricuspid aortic valve, we discussed a surgical considerations for the treatment of this anomaly.