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2.
J Cardiovasc Comput Tomogr ; 18(2): 179-186, 2024.
Article En | MEDLINE | ID: mdl-38262851

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.


Aortic Diseases , Aortic Valve Insufficiency , Quadricuspid Aortic Valve , Humans , Male , Adult , Middle Aged , Aged , Female , Retrospective Studies , Predictive Value of Tests , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/etiology , Aortic Valve Insufficiency/surgery , Hemodynamics
3.
J Invasive Cardiol ; 35(9)2023 Sep.
Article En | MEDLINE | ID: mdl-37983112

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.


Aortic Valve Insufficiency , Quadricuspid Aortic Valve , Male , Humans , Middle Aged , Angina Pectoris , Aortic Valve Insufficiency/diagnosis , Aortic Valve Insufficiency/etiology , Aortic Valve Insufficiency/surgery , Dyspnea , Electrocardiography
8.
J Cardiothorac Surg ; 18(1): 63, 2023 Feb 07.
Article En | MEDLINE | ID: mdl-36750908

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.


Aortic Valve Insufficiency , Endocarditis, Bacterial , Endocarditis , Quadricuspid Aortic Valve , Male , Humans , Adult , Endocarditis, Bacterial/surgery , Aortic Valve/surgery , Endocarditis/complications , Aortic Valve Insufficiency/surgery
9.
Clin Genet ; 104(1): 133-135, 2023 07.
Article En | MEDLINE | ID: mdl-36756699

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.


Aortic Valve , Quadricuspid Aortic Valve , Humans , Aortic Valve/diagnostic imaging , Aortic Valve/abnormalities , Frameshift Mutation/genetics , Phenotype , Transcription Factors/genetics
11.
Vet Med Sci ; 9(2): 712-716, 2023 03.
Article En | MEDLINE | ID: mdl-33713550

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.


Heart Defects, Congenital , Horse Diseases , Quadricuspid Aortic Valve , Humans , Animals , Horses , Aortic Valve/diagnostic imaging , Aortic Valve/abnormalities , Aortic Valve/surgery , Quadricuspid Aortic Valve/veterinary , Echocardiography/veterinary , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/veterinary , Thorax , Horse Diseases/diagnostic imaging
12.
Curr Cardiol Rev ; 19(2): e220322202505, 2023.
Article En | MEDLINE | ID: mdl-35319379

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.


Endocarditis , Heart Defects, Congenital , Pulmonary Valve , Quadricuspid Aortic Valve , Infant , Humans , Aortic Valve/diagnostic imaging , Pulmonary Valve/diagnostic imaging , Constriction, Pathologic , Cross-Sectional Studies
14.
Braz J Cardiovasc Surg ; 38(1)2023 02 10.
Article En | MEDLINE | ID: mdl-35072405

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.


Aortic Aneurysm , Quadricuspid Aortic Valve , Sinus of Valsalva , Humans , Aged , Sinus of Valsalva/diagnostic imaging , Sinus of Valsalva/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Aneurysm/complications , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/surgery , Echocardiography
15.
J Card Surg ; 37(12): 5595-5598, 2022 Dec.
Article En | MEDLINE | ID: mdl-36423255

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.


Aortic Valve Insufficiency , Heart Valve Prosthesis Implantation , Quadricuspid Aortic Valve , Male , Humans , Middle Aged , Aortic Valve Insufficiency/complications , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/abnormalities , Quadricuspid Aortic Valve/surgery , Echocardiography , Heart Valve Prosthesis Implantation/adverse effects
17.
Echocardiography ; 39(10): 1363-1366, 2022 10.
Article En | MEDLINE | ID: mdl-36138549

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 Insufficiency , Cardiac Surgical Procedures , Quadricuspid Aortic Valve , Female , Humans , Middle Aged , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/surgery , Treatment Outcome , Aortic Valve/diagnostic imaging , Aortic Valve/surgery
18.
J Cardiothorac Surg ; 17(1): 146, 2022 Jun 07.
Article En | MEDLINE | ID: mdl-35672828

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.


Aortic Valve Insufficiency , Heart Valve Prosthesis , Quadricuspid Aortic Valve , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/etiology , Aortic Valve Insufficiency/surgery , Coronary Vessels , Female , Humans , Middle Aged
19.
Article En | MEDLINE | ID: mdl-35671233

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.


Aortic Valve Insufficiency , Quadricuspid Aortic Valve , Aorta/surgery , Aortic Valve/surgery , Aortic Valve Insufficiency/surgery , Humans , Retrospective Studies , Treatment Outcome
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