Quadricuspid aortic valve in a neonate with Fallot's tetralogy.

A 3-day-old male neonate was referred to our hospital for a cardiac echocardiogram.

Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment.

BACKGROUND: 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. METHODS: Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 â€‹mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). RESULTS: Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 â€‹± â€‹13.6 years, 61.1 â€‹% male). Isolated significant aortic regurgitation (AR) was present in 75.6 â€‹% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 â€‹%) and type II (3 larger and 1 smaller leaflets, 42.2 â€‹%) QAV. Fused raphe was present in 26.7 â€‹% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 â€‹%, n â€‹= â€‹37). Among patients without AVR at baseline (n â€‹= â€‹60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 â€‹mm2, HR â€‹= â€‹4.25, 95%CI 1.49-12.17, p â€‹= â€‹0.007; as a continuous variable (per mm2 increment), HR â€‹= â€‹1.04, 95%CI 1.01-1.07, p â€‹= â€‹0.003). Additionally, ROACT 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 â€‹= â€‹0.004). CONCLUSION: QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.

Quadricuspid aortic valve.

A 56-year-old man presented with typical angina for 1 day and a prior history of exertional dyspnea of 6 months duration. Clinically, he was diagnosed with severe aortic regurgitation (AR). Electrocardiogram showed left ventricular hypertrophy with volume overload.

Infective endocarditis of quadricuspid aortic valve.

BACKGROUND: Infective endocarditis of the aortic valve is a relatively common disease presentation, with surgical intervention a mainstay of treatment in severe cases. Quadricuspid aortic valves are a rare spontaneous developmental anomaly that are more likely to be asymptomatic, and less likely to require a full valve replacement than their hypocuspid counterparts. However, there is very little literature addressing infective endocarditis of this valve variant. CASE PRESENTATION: This case report presents a case of infective endocarditis of a quadricuspid aortic valve that required replacement with a surgical bioprosthetic valve. The patient is a 30 year old male with a history of polysubstance use, upper extremity aneurysm, and prior tricuspid valve endocarditis. Surgical aortic valve replacement was performed with a 25 mm tissue valve via median sternotomy. CONCLUSIONS: The patient made a full recovery after surgical aortic valve replacement and a course of antibiotics and was discharged home without any complications. This supports that surgical aortic valve replacement is feasible and safe in patients with polycuspid aortic valve endocarditis.

Broad spectrum of anomalies including quadricuspid aortic valve associated with a novel frameshift SALL4 variant.

Each family member had a SALL4 variant. This is the first report of quadricuspid aortic valve and a genetic variant. The variation in phenotype caused by SALL4 mutations questions the division of SALL4-related phenotypes in three different entities.

Quadricuspid Aortic Valve with Ruptured Sinus of Valsalva Aneurysm: a Case Report.

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 Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve.

BACKGROUND: Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV). CASE PRESENTATION: We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained. RESULTS: In QPV cases diagnosed at autopsy, Hurwitz's type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz's type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients. CONCLUSION: Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.

Transthoracic echocardiography and its limitations in the diagnosis of congenital supernumerary aortic valve in a Thoroughbred.

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.

Transapical aortic valve replacement for quadricuspid aortic valve with severe aortic regurgitation which caused multiorgan failure.

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.

Quadricuspid aortic valve repair with a modified-tricuspidization technique.

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.

Aortic valve replacement of a quadricuspid aortic valve with right coronary artery ostium adjacent to one of the commissures.

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.

The David procedure for quadricuspid aortic valve repair.

Quadricuspid aortic valves are rare but may result in significant aortic regurgitation requiring surgery. To date, valve-sparing root replacement is frequently done in patients with bi- and tricuspid aortic valve pathologies with preserved cusp tissue. Nevertheless, little is known about quadricuspid repair, especially in regard to the optimal surgical technique for long-term durability. We hereby provide a surgical guide to valve-sparing aortic root replacement using the David technique in a young patient with severe aortic regurgitation.