Case report: unravelling the puzzle of unicuspid aortic valve with multimodality imaging.

Background: Unicuspid aortic valve (UAV) represents a rare congenital anomaly characterized by two subtypes: acommissural unicuspid aortic valve and unicommissural unicuspid aortic valve. Acommissural UAV is often diagnosed and corrected during the neonatal period due to haemodynamic instability. Unicommissural UAV leads to aortic stenosis (AS) in early adulthood. The diagnostic challenge associated with UAV primarily stems from its eccentric orifice opening and valvular calcification, resulting in difficult visualization of the commissures and localization of the orifice plane. This case report aims to demonstrate the unique morphological features of UAV through a comprehensive analysis using multimodality imaging. Case summary: A 61-year-old woman presented to the emergency department for recurrent episodes of dyspnoea. Severe AS was diagnosed on transthoracic echocardiography (TTE) by Doppler haemodynamic measurement. However, follow-up transesophageal echocardiography (TEE) and CT transcatheter aortic valve replacement showed moderate AS by planimetry. Following this, patient was monitored closely, but her dyspnoea kept worsening. Cardiovascular magnetic resonance (CMR) was performed due to persistent dyspnoea, identifying UAV with eccentric loophole orifice with unicommissural attachment and opposite free leaflet edge. The patient was managed medically. Discussion: TTE is the test of choice for AS that defines valvular morphology by direct visualization and grades the severity by haemodynamic measurement. However, the accuracy of TTE can be limited by poor acoustic windows and heavy valvular calcification. TEE measures aortic valve area (AVA) by planimetry that requires accurate localization of the AV orifice plane. Similarly, it applies to multi-detector computed tomography (MDCT). While CMR is expensive and mainly available in tertiary centres, it can provide additional information when there is discordance.

Unicommissural unicuspid aortic valve (UAV) presenting as ascending aortic aneurysm with aortic dissection.

We herein present an exceptionally rare instance of a unicommissural unicuspid aortic valve (UAV) manifesting with an aneurysmal ascending aorta complicated by a type A aortic dissection .

Unicuspid aortic valve by cardiac computed tomography: the best view is from the mountaintop-a case report.

Background: Unicuspid aortic valve (UAV) is a rare valvular heart disease and a challenging diagnosis. Advanced imaging techniques, particularly cardiac computed tomography (CT), appear to be invaluable tools to correctly identify this disease pre-operatively, as this may have an impact on the optimal surgical treatment. Case summary: We describe the case of a young patient admitted with heart failure, due to a severely stenotic UAV. Cardiac CT allowed adjusting the imaging plane to the best view in two orthogonal planes to identify the top of the 'dome' and to accurately measure the smallest valve opening by planimetry. Surgical inspection confirmed a rare case of acommissural UAV. Discussion: Cardiac CT angiography is crucial to understand the complexity of UAV disease and to differentiate the acommissural from the unicommissural type. Accurate positioning of the imaging plane through the smallest valve opening in systole reduces the risk of missing the diagnosis of this rare disease.

A rare and unique case: aortic valve replacement in a young adult with a stenotic unicommissural unicuspid aortic valve.

Unicuspid aortic valve (UAV) is a rare congenital cardiac anomaly. There are two forms of UAV, including unicuspid acommissural and unicuspid unicommissural. Definitive management for UAV is surgical intervention, but due to the rarity of UAV, the long-term surgical outcomes as well as overall prognosis are not known. Here, we present the case of a 19-year-old patient who was found to have a UAV prenatally and underwent a mechanical aortic valve replacement through an upper hemi-sternotomy due to elevated aortic stenosis gradients and presence of symptoms.

Functionally unicuspid aortic valve in an adult: Case report and literature review.

Aortic stenosis is one of the most prevalent cardiac valvular diseases throughout the world and has a significant impact on quality of life. While there are several etiologies, we will be discussing the case of a male in his mid-thirties of southeast Asian descent with a bicuspid aortic valve which was found to be functionally unicuspid and complicated by aortic dilation. Following a comprehensive review of literature, it appears this subset of aortic stenosis is not commonly encountered. In addition to presenting this fascinating case, we will review the epidemiology, classification and management of aortic stenosis. Furthermore, we will examine the latest evidence-based literature on bicuspid aortic valve and unicuspid aortic valve and discuss interventions and diagnostic tools that may improve clinical prognosis.

The hidden unicuspid: Recurring aortic stenosis post-valvuloplasty from a unicuspid aortic valve masquerading as bicuspid.

Unicuspid aortic valves (UAV) account for 0.2 % of cardiac valvular disorders and present with early-onset aortic stenosis (AS) during adolescence or early adulthood. We present a case of a 17-year-old male with recurring AS. He was diagnosed with bicuspid aortic valve (BAV) two years previously and treated with balloon valvuloplasty, which relieved symptoms before that. Multimodality imaging work-up revealed the precise morphology of UAV, consistent with the surgical findings. The patient received a St. Jude mechanical valve (St Paul; MN, USA) which resolved his symptoms. A thorough radiologic evaluation is therefore required for the accurate diagnosis of UAVs. While 2-dimensional (2D) transthoracic echocardiography (TTE) often constitutes the initial modality for evaluating UAVs, 3D-TTE, transesophageal echocardiography, and cardiac computed tomography are used as confirmatory diagnostic tools. Balloon valvuloplasty reports good outcomes in BAV but is associated with an increased rate of symptom recurrence, repeated surgical procedures, and higher mortality in UAVs, underscoring the importance of an accurate pre-operative diagnosis. Learning objectives: 1.Unicuspid aortic valve (UAV) should be considered in the differential diagnosis of severe aortic stenosis during childhood.2.3D-transthoracic echocardiogram, transesophageal echocardiogram, and cardiac computed tomography (CT) can confirm the diagnosis of a UAV.3.Cardiac CT can additionally assess for accompanying abnormalities of the great vessels in UAV patients.4.Balloon valvuloplasty reports good outcomes in bicuspid aortic valve but is associated with an increased rate of symptom recurrence in UAV patients.

Ozaki Procedure Plus Aortic Annular Enlargement in an Extremely Narrow Aortic Annulus: A Surgical Challenge.

We report a 19-year-old woman with a history of percutaneous aortic valve (AV) balloon valvuloplasty 3 years ago. She was admitted with severe symptomatic AV stenosis with narrow aortic annulus. We decided to perform AV reconstruction with autologous pericardium. However, surgical findings revealed a very narrow AV annulus with a unicuspid valve; therefore, an AV enlargement was added.

A new alternative to the Bentall procedure: aortic valve neocuspidization (Ozaki procedure) with the Florida sleeve aortic root reconstruction.

Aortic valve operations for the sinus of Valsalva aneurysm are challenging. Several techniques are described in the literature for these kinds of pathologies, such as the David procedure, the Yacoub procedure and the Bentall procedure. The Florida sleeve technique was described in the last decade as a valve-sparing technique for a sinus of Valsalva aneurysm. More recently, the J-Mart technique was described: It combines the Florida sleeve technique with aortic valve replacement. Our goal was to describe our new technique, which depends mainly on combining the Florida sleeve technique and the Ozaki procedure in a small group of patients who had aortic valve disease and a sinus of Valsalva aneurysm.

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients.

INTRODUCTION: Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. MATERIALS AND METHODS: Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher's exact test and overlapping 83.7% confidence intervals. RESULTS: Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1-14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. DISCUSSION: In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

Diagnosis of unicuspid aortic valve after loss of consciousness during cesarean section: A case report.

Loss of consciousness is a rare but potentially serious complication of delivery by cesarean section under spinal anesthesia. Here, we report the case of a pregnant woman with unicuspid aortic valve that was incidentally diagnosed during aortic valve replacement after transient loss of consciousness during cesarean section.

Aortic Valve Repair in Neonates With Aortic Stenosis and Reduced Left Ventricular Function.

This study assessed outcomes of neonatal aortic valve (AoV) repair in presumed high-risk patients with depressed left ventricular (LV) function. A retrospective analysis of all neonates who underwent isolated AoV repair for severe aortic stenosis (AS) was performed. Patients with moderate or severe LV dysfunction were compared to those with normal or mild LV dysfunction. From 1980-2021, 43 neonates underwent isolated AoV repair for AS. Of these, 16 patients (37.2%) had ≥moderate LV dysfunction. Mean LV ejection fraction (EF) was 32.8 ± 9.1%. Valve morphology was mostly unicuspid (68.75%, 11/16). Median age at surgery was 6.5 days (IQR 1-17.5). An optimal repair result with ≤mild AS or aortic regurgitation was achieved in 75% (12/16). There was no early death. One patient (6.25%) required postoperative extracorporeal membrane oxygenation (ECMO) support for 3 days. LVEF improved after surgery to 56.4 ± 12.6% before discharge (P < 0.0001) and normalized in 87.5% (14/16) with a median time of 6.4 days (IQR 3.4-39). Freedom from AoV reoperation was 45.1% (95%CI 17.9-69.3%) and 27.1% (95%CI 6.8-53%) at 5 and 10 years, respectively. Freedom from AoV replacement was 59% (95%CI 27-80.8%) and 31.4% (95%CI 6-62.2%) at 5 and 10 years, respectively. While survival was similar, freedom from AoV reoperation and replacement tended to be lower compared to neonates with preserved LVEF. AoV repair was associated with a low incidence of postoperative ECMO and mortality. LV function normalized after relief of obstruction in most patients before discharge. Late reoperation remained common for those with severely dysplastic valves.

Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

OBJECTIVE: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. METHODS: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization. Survival, valve-related reoperation, and the development of moderate or greater aortic or truncal valve regurgitation were assessed. RESULTS: There were 23 patients who underwent symmetric bicuspidizing repair and 18 who underwent another form of bicuspidization. Preoperative aortic regurgitation was present in 87.0%. Patients who underwent symmetric bicuspidizing repair more commonly underwent suture annuloplasty (100% vs 55.6%; P = .002) and ascending aortoplasty (78.3% vs 27.8%; P = .004). There was 1 operative mortality (2.4%) in the entire cohort and 1 late mortality. Freedom from moderate aortic regurgitation was 87.5% at 21 months after symmetric bicuspidizing repair compared with 43.5% for patients who underwent other types of bicuspidization; P = .03. Freedom from valve-related reoperation was 100% in the symmetric bicuspidizing repair group compared with 64.4%; P = .02. CONCLUSIONS: The symmetric bicuspidizing repair may be safely extended to patients with various forms of congenital aortic and truncal valve disease. Longer term follow-up will be necessary to determine the comparative effectiveness of this technique compared with neocuspidization and the Ross procedure.

Unicuspid Aortic Valve With Severe Aortic Stenosis and Progressive Heart Failure During Pregnancy.

A 39-year-old gravida 7 para 6 woman with unicuspid aortic valve and severe symptomatic stenosis was admitted to the hospital at 15 weeks gestation. We describe maternal cardiovascular complications and their implication on obstetric and fetal care. We also describe our multidisciplinary approach to caring for this complex patient.

Comparison of bicuspidization and Ross procedure in the treatment of unicuspid aortic valve disease in adults - Insight from the AVIATOR registry.

Background: Unicuspid aortic valve (UAV) is the second most common underlying cause of aortic valve dysfunction in young adults after the bicuspid valve. The valve may be replaced (for example by pulmonary autograft) or repaired using the bicuspidization technique. The aim of our study was to compare short- and mid-term outcomes of Ross procedure with bicuspidization in patients with severe UAV dysfunction. Methods: This was a multi-center retrospective observational cohort study comparing data from two dedicated Ross centers in the Czech Republic with bicuspidization outcomes provided by AVIATOR registry. As for the Ross group, only the patients with UAV were included. Primary endpoint was mid-term freedom from reintervention. Secondary endpoints were mid-term freedom from major adverse events, endocarditis and pacemaker implantation. Results: Throughout the study period, 114 patients underwent the Ross procedure (years 2009-2020) and 126 patients underwent bicuspidization (years 2006-2019). The bicuspidization group was significantly younger and presented with a higher degree of dyspnea, a lower degree of aortic valve stenosis and more often with pure regurgitation. The primary endpoint occurred more frequently in the bicuspidization group than in the Ross group - 77.9 vs. 97.9 % at 5 years and 68.4 vs. 75.2 % at 10 years (p < 0.001). There was no difference in secondary endpoints. Conclusion: Ross procedure might offer a significantly lower mid-term risk of reintervention than bicuspidization in patients with UAV. Both procedures have comparable survival and risk of other short- and mid-term complications postoperatively.