A unicuspid aortic valve morphology is a relatively rare variant and may require surgical correction early in life for regurgitation. In the majority of cases, a patch is required for repair, which degenerates and thus limits the durability of valve repair. The bicuspidization of a unicuspid aortic valve without the use of patch material is possible in cases with a symmetric cusp nadir. Unicuspid aortic valves are often accompanied by root enlargement and require root stabilization. Whenever possible, we repair these valves without the use of patch material and with annular stabilization by using a suture annuloplasty.

Robotic aortic valve replacement is the latest advance in the field of aortic valve intervention and is increasingly being adopted by various centres with favourable early results. It allows surgeons to implant any commercially available aortic valve prosthesis (sutureless, biological, mechanical) in a surgically conventional manner, while offering minimal surgical trauma (sternum-free, rib-free, pectoralis muscle sparing). We present a step-by-step standardized procedure of robotic aortic valve replacement with a mechanical prosthesis for a young female patient with severe symptomatic aortic valve stenosis in a unicuspid aortic valve.

Bicuspidization of the Unicuspid Aortic Valve: Neocommissure Reconstruction Preserving the Native Free Margin

Unicuspid aortic valve (UAV) is a rare congenital defect. Compared with valve replacement, aortic valve repair is a better potential strategy to achieve a low rate of valve-related events and an enhanced quality of life. We herein report two cases of aortic valve repair for UAV accompanied by aneurysm of the ascending aorta. By retaining the free margin tissue and forming a neocommissure to the side of the left lateral commissure that is 180 degrees opposite and the same height, we were able to achieve bicuspidization of the UAV. A glutaraldehyde-treated autologous pericardium patch was placed along the cusp connection to the annulus to expand the aortic cusp. During the follow-up period of over 4 years, the patients showed no signs of aortic regurgitation or significant stenosis.

Description of case: A 39-year-old man with history of unicuspid aortic valve status-post bicuspidization (2017), subsequent severe aortic insufficiency prompting mechanical aortic valve replacement (2023), recent prosthetic valve thrombosis on therapeutic warfarin complicated by stenosis and shock prompting redo mechanical aortic valve replacement (2/2025, 23mm On-X), presented with acute dyspnea, chest pain, and loss of mechanical click. He was found to have SCAI-C cardiogenic shock, with exam notable for III/VI systolic crescendo-decrescendo and IV/IV decrescendo early diastolic murmurs, and absent mechanical click. Labs were notable for absolute eosinophils of 11.5 K/cu mm. On TTE, valve disks were poorly visualized. The mean gradient was 59mmHg and there was severe aortic insufficiency with preserved ventricular function. Due to concern for valvular obstruction, TEE was performed, revealing a well-seated valve with severe valvular regurgitation originating posteriorly and a reduction in systolic excursion of the disks. Valve fluoroscopy showed minimal movement of both discs suggesting thrombosis. The patient was deemed high risk for surgical intervention and therefore received systemic thrombolysis with alteplase 25mg over 6h x2. Repeat TTE showed mean gradient 29mmHg, and mild-moderate aortic insufficiency. Eosinophilia peaked at 16.7 K/cu mm and normalized with high-dose prednisone. Eosinophilia workup is ongoing. Discussion: Prosthetic valve thrombosis is a rare and feared complication without randomized controlled trials to support optimal treatment. The 2017 American Heart Association guidelines suggest surgery or fibrinolytics determined by individualized patient factors. Previously, surgery was preferred in patients with advanced NYHA symptoms or large thrombi. However, a novel strategy with echocardiogram-guided, low-dose infused fibrinolytics has demonstrated high success rates in this patient population. In our case, multimodal imaging was essential in the rapid diagnosis of prosthetic valve thrombosis. Our preference for fibrinolytics was based on surgical risk and to enable pre-operative workup and management of his hypercoagulable state. Few cases have been reported of eosinophilia-driven prosthetic valve thrombosis, with differential including eosinophilic granulomatosis polyangiitis and IgG4-related disease. Further data are needed to enable tailored treatment strategies in different patient populations.

Bicuspid and unicuspid aortic valves represent the most common congenital aortic valve malformations and pose unique challenges in clinical management across the lifespan. These anomalies are associated with progressive valvular dysfunction and aortopathy, often necessitating early intervention. Multiple publications have described life-long management of aortic stenosis (AS), affecting tricuspid valve. This review outlines the embryologic basis, natural history, and clinical spectrum of uni- and bicuspid aortic valve, highlighting diagnostic strategies, surveillance protocols, and surgical - transcatheter interventions. Emphasis is placed on longitudinal care, including transition from pediatric to adult congenital cardiology, multimodality imaging, and timing of surgical or transcatheter interventions. In conclusion, the article aims to provide a framework for evidence-informed, individualized management of these complex valvulo-aortic disorders.

Unicuspid Aortic Valve Stenosis in a Child.

Pulmonary valve repair at the time of the Ross procedure: A safe and durable strategy to address postimplantation aortic regurgitation.

Right Atrial Appendage for Aortic Valve Reconstruction.

Unicuspid aortic valve diagnosed by transthoracic 3-dimensional echocardiography with rapidly progressing aortic stenosis.

Abstract Introduction Aortic valve stenosis (AS) is a leading form of cardiovascular disease in the developed world, affecting 0.3-0.5 % of the general population.The etiologies of AS are different, the most frequent is the degenerative form in elderly population,followed by congenital malformation (unicuspid or bicuspid aortic valve) and rheumatic heart disease (RHD) especially in young population. Echocardiographic exam allows to assess early stage of disease before the onset of symptoms and to orientate the adequate timing of aortic–valve replacement. Case Report A 53 years old female admitted to our medical centre with history of dyspnea for mild exercise and chest pain. On his physical examination body temperature was 37,4 °C, blood pressure was 100/60 mmHg,and heart rate 100 beats/min. ECG showed sinus rhythm, high voltage QRS and no changes of ST–T waves suggestive for ischemia.On admission,hematologic findings, renal and liver function tests were within normal limits. C–reactive protein was slightly increased. Myocardionecrosis enzymes were elevated. Echocardiographic examination documents reduced ejection fraction (EF 45%) with diffuse hypokinesia, severe aortic valve stenosis and moderate aortic insufficiency and pericardial effusion. Diuretic supportive therapy was first initiated. On the suspicion of peri–myocarditis was treated whit antiinflammatory drugs. All autoimmune tests were negative.To better evaluated aortic valve disease a transoesophageal echocardiogram was perfomed hat showed leaflet thickening and calcification with circular valve opening (Figure 2). The peak and mean aortic valve gradients were recorded to be 66 mmHg and 42 mmHg, respectively (Figure 3).The valve area calculated by planimetry was 0.874 cmq in 2D (Figure 1). Calculated valve area by continuity equation was 0.562 cm2/m2. A unicuspid aortic valve was suspected. Coronary angiography was negative for stenosis. A cardiac magnetic resonance imaging was performed and no diagnostic criteria for myocardial inflammation was detected. After stabilizing the clinical condition, the patient underwent surgical replacement of the aortic valve. A diagnosis of rheumatic aortic valve disease was made because the valve was tricuspid and had fusion of the cusps. Conclusion RHD and congenital aortic valve malformation have a different prevalence in the general population. Today there are many tools available for a reliable diagnosis. Cardiac MRI, thanks to better image quality, can help in the diagnosis and specific treatment for our patientFigure 1:Short axis aortic valveFigure 3:Continuous wave doppler (CW) aortic valveFigure 2:3D color aortic valve

The incidence of thoracic aortic aneurysms (TAAs) is approximately 10.4 cases per 100,000 person-years. Although most cases of TAA are caused by degenerative disease, associated aortic valve abnormalities have been heavily linked to this condition. These include unicuspid, bicuspid and quadricuspid aortic valves. These non-tricuspid aortic valves occur sporadically but can occur in familial clusters with variable penetrance. The presence of non-tricuspid aortic valves has significant implications for patients, as they become prone to valvular dysfunction and aortic dissection. Therefore, understanding of the pathophysiology and natural history of this condition is imperative for early diagnosis, regular surveillance and timely intervention. In this review article, we discuss the normal anatomy of the aortic valve, non-tricuspid aortic valves and their association with TAAs. We also highlight the role of various cardiac imaging modalities in the management of affected patients.

Aortic stenosis (AS) is a significant and growing concern, with a prevalence of 2-3% in individuals aged over 65 years. Moreover, with an aging global population, the prevalence is anticipated to double by 2050. Indeed, AS can arise from various etiologies, including calcific trileaflets, congenital valve abnormalities (e.g., bicuspid and unicuspid valves), and post-rheumatic, whereby each has a distinct influence that shapes the onset and progression of the disease. The normal aortic valve has a trilaminar structure comprising the fibrosa, spongiosa, and ventricularis, which work together to maintain its function. In calcific AS, the disease begins with early calcification starting in high mechanical stress areas of the valve and progresses slowly over decades, eventually leading to extensive calcification resulting in impaired valve function. This process involves mechanisms similar to atherosclerosis, including lipid deposition, chronic inflammation, and mineralization. The progression of calcific AS is strongly associated with aging, with additional risk factors including male gender, smoking, dyslipidemia, and metabolic syndrome exacerbating the condition. Conversely, congenital forms of AS, such as bicuspid and unicuspid aortic valves, result in an earlier disease onset, typically 10-20 years earlier than that observed in patients with a normal tricuspid aortic valve. Rheumatic AS, although less common in developed countries due to effective antibiotic treatments, also exhibits age-related characteristics, with an earlier onset in individuals who experienced rheumatic fever in their youth. The only curative therapies currently available are surgical and transcatheter aortic valve replacement (TAVR). However, these options are sometimes too invasive for older patients; thus, management of AS, particularly in older patients, requires a comprehensive approach that considers age, disease severity, comorbidities, frailty, and each patient's individual needs. Although the valves used in TAVR demonstrate promising midterm durability, long-term data are still required, especially when used in younger individuals, usually with low surgical risk. Moreover, understanding the causes and mechanisms of structural valve deterioration is crucial for appropriate treatment selections, including valve selection and pharmacological therapy, since this knowledge is essential for optimizing the lifelong management of AS.

Restoration of 3-dimensional aortic hemodynamics after the Ross procedure for unicuspid aortic valve disease using 4-dimensional flow magnetic resonance imaging

The unicuspid unicommisural aortic valve is an uncommon congenital malformation that often manifests as stenosis with or without regurgitation in adults in their third to fifth decades of life. This report characterizes the morphological features of surgically excised unicuspid valves in adults with clinical correlation. Among the surgically excised aortic valves over a period of 10 years, the clinical data and morphological features of unicuspid aortic valves were analyzed. The patients were grouped by the type of valvular function. Pathological features noted were the shape of the orifice, the status of the commissure and raphe, presence of fibrous thickening, calcification, and other complications. Twenty-three UAVs, excised over a 10-year period, represented 4.16% of the excised diseased aortic valves. There was a male preponderance with a mean age of 47.7 years. Majority of the patients (22 cases) had moderate to severe stenosis with varying degrees of regurgitation, and the valvular disease had been clinically attributed to rheumatic heart disease, bicuspid aortic valve, or senile degenerative changes. Most of the valves (18) had been cut at their commissural regions, and 2 rudimentary commissures or raphes were seen in 21 valves. Cuspal fibrosis/calcification was often associated with complications like ulceration, hemorrhage, and bland vegetations. Aneurysm of the ascending aorta had been present in 1 patient. The unicuspid unicommisural aortic valve while rare is usually clinically classified with the more common, congenitally bicuspid aortic valve. Clinical or imaging diagnosis can be challenging since calcification may obscure the morphology creating difficulties in distinguishing such valves from other congenital or acquired valvular pathologies, especially in older patients. Often it is only the gross examination that leads to the differentiation as was our observation.

Case 1: A 54-yr-old man reported exertional dyspnea for 3 months, with a systolic murmur on exam. TTE noted severe aortic stenosis, moderate insufficiency and possible bicuspid valve morphology. Coronary angiogram showed no obstructive disease. CT chest showed a 5-cm ascending aortic aneurysm. CT surgery evaluation was sought, and patient had a Ross procedure with aortic hemiarch repair. Intra-operative TEE confirmed a congenitally unicommissural, unicuspid aortic valve (UAV). A 7-month follow-up TTE showed a normal aortic and pulmonic prosthetic gradient. Case 2: A 46-yr-old lady reported progressive exercise intolerance, with a systolic murmur and faint S2 noted on exam. TTE showed severe aortic stenosis. MR angiogram showed a unicuspid valve and a mildly dilated aortic root. The patient was evaluated by CT surgery and underwent Ross procedure. TTE 4 years later showed a normal aortic and a moderately elevated pulmonic prosthetic gradient. Patient continues to exercise without limitations. Discussion: UAV is a rare entity with an incidence of 0.02%. It has a bimodal distribution, in infants and adults, with the unicommissural type more common in adults. Due to rapid progression of valvular disease, the mean age at diagnosis is 42 years. Concurrent aortic dilatation is present in 48% patients. Management of UAV is a matter of ongoing investigation. Classically, young patients with aortic valve disease underwent mechanical AVR. Ross procedure was under-utilized due to concerns about the surgical complexity and potential need for re-operation of the aortic and pulmonic grafts. However, recent studies show promising results. Patients with UAV have lower reoperation rates after a Ross procedure compared to a repair alone. Furthermore, a Ross procedure confers mortality benefit over mechanical AVR with no significant difference in immediate perioperative complications and mortality, although, this needs further investigation in a UAV cohort. Our cases support the feasibility of a Ross procedure for UAV, in providing a durable solution while avoiding anticoagulation in young patients. As Ross procedure is performed in limited centers, patients with UAV should be referred to such centers for definitive management.

Unicuspid aortic valve, accurate detection for timely intervention

Combined simulation and ex vivo assessment of free-edge length in bicuspidization repair for congenital aortic valve disease

A unicuspid aortic valve (UAV) in adults is a very rare form of aortic valve (AV) malformation. UAV has two distinct subtypes, acommissural UAV and unicommissural, and can be differentiated by anatomical features, imaging modalities, and clinical presentation. With the development of significant AV lesion (s), surgical or transcatheter intervention will be required. The first part is a summarized review of UAV (anatomical features, clinical presentation, diagnostic modalities, and management). In the second part, we present a series of four patients diagnosed with UAV (3 unicommissural and 1 acommissural). The first case underwent balloon aortic valvuloplasty during childhood and surgical AV replacement later, with the progression to severe aortic stenosis (AS). The second case underwent a Ross procedure. The third and fourth cases were asymptomatic with moderate AS and mild-to-moderate AR and were kept on follow-up. In all the cases, transesophageal echocardiography confirmed the diagnosis of UAV with detailed morphological and functional assessment of AV.

BackgroundInfective endocarditis of the aortic valve can result in a wide range of destructive pathology beyond the valve leaflets and annulus which require careful surgical planning to provide appropriate debridement and reconstruction. Failure to do so can result in a failure of surgical treatment, recurrent infection and cardiac failure with concomitant high morbidity and mortality.Case reportWe describe the case of a 45-year-old male with previous patch repair of a ventricular septal defect, who was diagnosed with sub-acute bacterial endocarditis of the native aortic valve and developed a new fistula from the aorta to the right ventricular outflow tract which. This was managed surgically.ConclusionThis unique case highlights another spectrum of infective endocarditis with a unique approach to repair and management.