Quadricuspid Aortic Valve: Imaging, Diagnosis, and Prognosis.

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.

Manifestations of Rheumatic Carditis, Regression of Valvular Regurgitation, and Independent Predictors of Mitral Regurgitation Improvement After Rheumatic Carditis in Thai Children.

Background: Acute rheumatic fever (ARF) with carditis can lead to the development of rheumatic heart disease in children and young adults. Objective: This study aimed to investigate the manifestations of rheumatic carditis, clinically significant regression of valvular regurgitation as assessed by echocardiography, and the independent predictors of mitral regurgitation (MR) improvement after rheumatic carditis in Thai children. Method: Children diagnosed with rheumatic carditis during 2005-2020 at Siriraj Hospital (Bangkok, Thailand) were retrospectively enrolled. Trivial, and mild regurgitation were grouped as non-clinically significant (NCS) regurgitation. Valvular regression was defined moderate-severe regurgitation improving to NCS regurgitation. Results: Eighty-one patients (mean age: 10 years, range: 8-12 years) were included. At presentation, 59 (72.8%) patients had combined mitral regurgitation (MR) and aortic regurgitation (AR), 20 (24.6%) patients had MR alone, and 2 (2.4%) patients had AR alone. Concerning severity, 28 (34.6%) and 30 (37%) patients presented with severe and moderate MR, respectively. Severe and moderate AR was found in 9 (11.1%) and 16 (19.8%) patients, respectively. At the one-year follow-up, 43.4% of moderate-severe MR, and 41.7% of moderate-severe AR improved to NCS regurgitation. Multivariate analysis revealed high erythrocyte sedimentation rate (ESR) (p = 0.01) and severe carditis (p = 0.05) at presentation to be independent predictors of MR improvement. Conclusion: Thai children with rheumatic carditis had a high incidence of valvular regurgitation; however, the valvular damage was improved in most patients. High ESR and severe carditis independently predict MR improvement.

Expert proposal to analyze the combination of aortic and mitral regurgitation in multiple valvular heart disease by comprehensive echocardiography.

The assessment of valvular pathologies in multiple valvular heart disease by echocardiography remains challenging. Data on echocardiographic assessment-especially in patients with combined aortic and mitral regurgitation-are rare in the literature. The proposed integrative approach using semi-quantitative parameters to grade the severity of regurgitation often yields inconsistent findings and results in misinterpretation. Therefore, this proposal aims to focus on a practical systematic echocardiographic analysis to understand the pathophysiology and hemodynamics in patients with combined aortic and mitral regurgitation. The quantitative approach of grading the regurgitant severity of each compound might be helpful in elucidating the scenario in combined aortic and mitral regurgitation. To this end, both the individual regurgitant fraction of each valve and the total regurgitant fraction of both valves must be determined. This work also outlines the methodological issues and limitations of the quantitative approach by echocardiography. Finally, we present a proposal that enables verifiable assessment of regurgitant fractions. The overall interpretation of echocardiographic results includes the symptomatology of patients with combined aortic and mitral regurgitation and the individual treatment options with respect to their individual risk. In summary, a reproducible, verifiable, and transparent in-depth echocardiographic investigation might ensure consistent hemodynamic plausibility of the quantitative results in patients with combined aortic and mitral regurgitation.

The Hemodynamic Effects of Aortic Regurgitation in Patients Supported by a HeartMate 3 Left Ventricular Assist Device.

BACKGROUND: Aortic regurgitation (AR) is a common complication following left ventricular assist device (LVAD) implantation. We evaluated the hemodynamic implications of AR in patients with HeartMate 3 (HM3) LVAD at baseline and in response to speed changes. METHODS AND RESULTS: Clinically stable outpatients supported by HM3 who underwent a routine hemodynamic ramp test were retrospectively enrolled in this analysis. Patients were stratified based on the presence of at least mild AR at baseline speed. Hemodynamic and echocardiographic parameters were compared between the AR and non-AR groups. Sixty-two patients were identified. At the baseline LVAD speed, 29 patients (47%) had AR, while 33 patients (53%) did not. Patients with AR were older and supported on HM3 for a longer duration. At baseline speed, all hemodynamic parameters were similar between the groups including central venous pressure, pulmonary capillary wedge pressure, pulmonary arterial pressures, cardiac output and index, and pulmonary artery pulsatility index (p > 0.05 for all). During the subacute assessment, AR worsened in some, but not all, patients, with increases in LVAD speed. There were no significant differences in 1-year mortality or hospitalization rates between the groups, however, at 1-year, ≥ moderate AR and right ventricular failure (RVF) were detected in higher rates among the AR group compared to the non-AR group (45% vs. 0%; p < 0.01, and 75% vs. 36.8%; p = 0.02, respectively). CONCLUSIONS: In a cohort of stable outpatients supported with HM3 who underwent a routine hemodynamic ramp test, the presence of mild or greater AR did not impact the ability of HM3 LVADs to effectively unload the left ventricle during early subacute assessment. Although the presence of AR did not affect mortality and hospitalization rates, it resulted in higher rates of late hemodynamic-related events in the form of progressive AR and RVF.

TAVR as an Alternative to SAVR for Pure Native Aortic Regurgitation.

Although transcatheter aortic valve replacement was originally fulfilling an unmet clinical need in the elderly population suffering from tricuspid aortic valve stenosis, its use has been progressively expanded to other groups of patients. In this review, we focus on pure native aortic valve regurgitation, which is in most cases a degenerative disease and therefore frequently diagnosed in elderly patients with comorbidities. Symptoms tend to appear late in the disease, when left ventricular dilation and systolic dysfunction are associated owing to excessive volume overload. It is often combined with a dilated aortic annulus and ascending aorta. Surgical aortic valve replacement remains the criterion standard treatment for severe aortic regurgitation. However, for patients at prohibitive surgical risk, transcatheter aortic valve replacement represents an attractive alternative. Various technical challenges are the absence of calcium at the level of the annulus, which means there are no anchoring points or fluoroscopic landmarks, the difficulty of valve sizing, and the increased stroke volume secondary to the aortic regurgitation, making valve deployment more unstable than in the setting of aortic stenosis. The first-generation transcatheter valves were associated with a higher mortality rate and lower procedural success related to increased risk of paravalvular leak and valve migration requiring a second valve or annular rupture than the more recent off-label or on-label transcatheter valves. Early studies with the dedicated on-label devices showed safety and promising results and will undoubtedly serve in the future a growing number of patients with native aortic regurgitation at prohibitive risk for surgery.

Predictors of outcome in patients with moderate mixed aortic valve disease.

OBJECTIVES: Grading the severity of moderate mixed aortic stenosis and regurgitation (MAVD) is challenging and the disease poorly understood. Identifying markers of haemodynamic severity will improve risk stratification and potentially guide timely treatment. This study aims to identify prognostic haemodynamic markers in patients with moderate MAVD. METHODS: Moderate MAVD was defined as coexisting moderate aortic stenosis (aortic valve area (AVA) 1.0-1.5 cm2) and moderate aortic regurgitation (vena contracta (VC) 0.3-0.6 cm). Consecutive patients diagnosed between 2015 and 2019 were included from a multicentre registry. The primary composite outcome of death or heart failure hospitalisation was evaluated among these patients. Demographics, comorbidities, echocardiography and treatment data were assessed for their prognostic significance. RESULTS: 207 patients with moderate MAVD were included, aged 78 (66-84) years, 56% male sex, AVA 1.2 (1.1-1.4) cm2 and VC 0.4 (0.4-0.5) cm. Over a follow-up of 3.5 (2.5-4.7) years, the composite outcome was met in 89 patients (43%). Univariable associations with the primary outcome included older age, previous myocardial infarction, previous cerebrovascular event, atrial fibrillation, New York Heart Association >2, worse renal function, tricuspid regurgitation ≥2 and mitral regurgitation ≥2. Markers of biventricular systolic function, cardiac remodelling and transaortic valve haemodynamics demonstrated an inverse association with the primary composite outcome. In multivariable analysis, peak aortic jet velocity (Vmax) was independently and inversely associated with the composite outcome (HR: 0.63, 95% CI 0.43 to 0.93; p=0.021) in an adjusted model along with age (HR: 1.05, 95% CI 1.03 to 1.08; p<0.001), creatinine (HR: 1.002, 95% CI 1.001 to 1.003; p=0.005), previous cerebrovascular event (85% vs 42%; HR: 3.04, 95% CI 1.54 to 5.99; p=0.001) and left ventricular ejection fraction (LVEF) (HR: 0.97, 95% CI 0.95 to 0.99; p=0.007). Patients with Vmax ≤2.8 m/s and LVEF ≤50% (n=27) had the worst outcome compared with the rest of the population (72% vs 41%; HR: 3.87, 95% CI 2.20 to 6.80; p<0.001). CONCLUSIONS: Patients with truly moderate MAVD have a high incidence of death and heart failure hospitalisation (43% at 3.5 (2.5-4.7) years). Within this group, a high-risk group characterised by disproportionately low aortic Vmax (≤2.8 m/s) and adverse remodelling (LVEF ≤50%) have the worst outcomes.

Infective Endocarditis Complicated With a Large Left Coronary Cusp Aneurysm: A Condition With Undefined Natural History and Treatment.

The natural history and treatment of an aortic cusp aneurysm with or without rupture because of native aortic valve infective endocarditis (NAV-IE) have not been well defined. This may explain why current guidelines for the management of valvular heart disease do not include this complication as an indication for surgical aortic valve replacement or repair or transcatheter aortic valve replacement (TAVR). We describe herein the first case of a man aged 76 years with multiple co-morbidities with a NAV-IE associated large left coronary cusp aneurysm with subsequent rupture and consequent severe aortic regurgitation and heart failure for which he underwent an off-label successful TAVR. This patient's scenario suggests that a cusp aneurysm because of NAV-IE poses a high risk for subsequent rupture, severe aortic regurgitation, and heart failure. In conclusion, TAVR may be a reasonable alternative to high-risk surgical aortic valve replacement in patients with NAV-IE associated cusp aneurysms with or without but impending rupture.

Design and Application of Comprehensive Management System For Patients with Chronic Aortic Valve Disease-A Perspective From West China Hospital.

BACKGROUND: Chronic aortic valve disease (AVD) is a prevalent age-related health issue. Current guidelines recommend transthoracic echocardiography as the method of detection. Early screening is crucial to decrease mortality caused by aortic valve disease. However, a lack of consistent and effective guidelines for screening populations with asymptomatic AVD persists. Based on the comprehensive management system (CMS) for AVD developed by West China Hospital of Sichuan University, the objective of this study is to enhance the survival rate and overall quality of life for patients suffering from AVD by integrating early screening into the standardised diagnosis and treatment process. METHODS: Three primary study cohorts were established: a community-based population screening cohort, an outpatient and medical examination population cohorts and an AVD inpatient cohorts. For each cohort, important data on clinical diagnosis and treatment were systematically collected and analyzed, evaluating the effectiveness of early screening for AVD. RESULTS: The transthoracic echocardiography (TTE) findings of outpatients and medical examination population at West China Hospital of Sichuan University between 1st January 2020 and 31st December 2022 have been examined utilizing CVS. In total, 327,822 outpatients were investigated, identifying 986 patients with aortic stenosis (AS), 2,961 patients with aortic regurgitation (AR), and 549 patients with mixed AS and AR. CONCLUSION: The AVD system for comprehensive management facilitates efficient screening, diagnosis and follow-up. In the future, the CVS will fully cover the West China hospital healthcare system and extend to the south-western region of China by leveraging a medical-driven role.

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.

Severe aortic insufficiency-induced cardiogenic shock treated with left atrial VA-ECMO and emergent valve-in-valve TAVR.

Conventional venoarterial extracorporeal membrane oxygenation (VA-ECMO) places a functional afterload burden on the left ventricle. In the setting of acute severe aortic insufficiency-induced cardiogenic shock, the utility of VA-ECMO in combination with a failing valve may result in catastrophic haemodynamic consequences. This challenge is compounded when the culprit is a failing surgical bioprosthetic valve. We present a case of severe rapid-onset bioprosthetic aortic insufficiency-induced cardiogenic shock successfully resuscitated with left atrial VA-ECMO promptly followed by emergent percutaneous valve-in-valve transaortic valve replacement. We discuss the logistics, implications, and associated haemodynamic manifestations in utilizing this strategy for such disease processes.

Surgical Management of Giant Unruptured Left Sinus of Valsalva Aneurysm With Severe Aortic Regurgitation.

Left sinus of Valsalva aneurysms are extremely rare. Concomitant aortic valve regurgitation is a comorbidity in this pathology. This case report summarizes successful surgical treatment with aortic root replacement with a modified Bentall procedure in a 49-year-old female patient who had an unruptured huge left sinus of Valsalva aneurysm with severe aortic valve regurgitation. The intraoperative assessment showed severe adhesion between the left main trunk of the coronary artery and the left sinus of Valsalva aneurysm, and meticulous adhesion detachment was required.

Tertiary Cardiovascular Syphilis Presenting as Aortic Regurgitation, Aortitis, Thrombus, and Coronary Artery Occlusion, Requiring Percutaneous Coronary Intervention.

BACKGROUND Heart failure is caused by coronary artery disease, valvular disease, and arrhythmias and is highly treatable with recent technology. However, the incidence of syphilis is increasing worldwide. This case report describes tertiary cardiovascular syphilis, accompanied by aortic regurgitation, syphilitic aortitis complicated by thrombus of the ascending aorta, and coronary artery occlusion, requiring percutaneous coronary artery intervention. CASE REPORT A 51-year-old Japanese man with no significant medical history was admitted to the hospital for worsening shortness of breath on exertion. On physical examination, there was no edema in either lower leg. Chest X-rays showed an enlarged heart and pulmonary congestion, and echocardiography showed a left ventricular ejection fraction of 18%, with full circumferential wall motion impairment. Heart failure was diagnosed, and the patient was found to have severe coronary artery disease and aortic regurgitation. He underwent percutaneous coronary intervention (PCI) for his coronary artery occlusion and was treated with medications for heart failure. Two months later, his condition improved, and PCI was performed for the revascularization of the remaining coronary artery. After PCI was completed, the patient was evaluated for vasculitis. The aortic wall lesion was likely a result of non-active syphilitic aortitis, and the results of serological tests of syphilis were positive. Therefore, we concluded that the diagnosis was cardiovascular syphilis. CONCLUSIONS This case report has highlighted the need for clinicians to be aware of the cardiovascular findings in syphilis, including syphilitic aortitis, particularly at this time, when the global incidence of syphilis is increasing.

Aortic Root Dilatation Measured by Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot.

BACKGROUND: Aortic root dilatation (AoD) frequently occurs following repaired tetralogy of Fallot (rTOF). The objective of this study was to assess aortic dimensions, investigate the prevalence of AoD, and identify predictors of AoD in rTOF patients. METHODS: A cross-sectional retrospective study was conducted in repaired TOF patients from 2009 to 2020. Aortic root diameters were measured by cardiac magnetic resonance (CMR). Severe AoD of the aortic sinus (AoS) was defined as a Z-score (z) of >4, reflecting a mean percentile ≥99.99%. RESULTS: Two hundred forty-eight patients, with a median age of 28.2 years (10.2-65.3 years), were included in the study. The median age at the time of repair was 6.6 years (0.8-40.5 years) and the median interval between the repair and CMR study was 18.9 years (2.0-54.8 years). The prevalence of severe AoD was found to be 35.2% when defined by an AoS z greater than 4 and 27.6% when defined by a AoS diameter ≥40 mm, respectively. A total of 101 patients (40.7%) had aortic regurgitation (AR), with 7 patients (2.8%) having moderate AR. Multivariate analysis revealed that severe AoD was only associated with the left ventricular end diastolic volume index (LVEDVi) and a longer duration after repair. The age at the time of repair for TOF was found not to be correlated with the development of AoD. CONCLUSIONS: After repair of TOF, severe AoD was found to be prevalent, but no fatal complications were observed in our study. Mild AR was also commonly observed. Larger LVEDVi and a longer duration after repair were identified as factors associated with the development of severe AoD. Therefore, routine monitoring of AoD is recommended.

Chronological comparison of TAVI and SAVR stratified to surgical risk: a systematic review, meta-analysis, and meta-regression.

BACKGROUND: Transcatheter aortic valve implantation (TAVI) has been established as a reasonable alternative to surgical aortic valve replacement (SAVR) in patients with severe aortic stenosis. However, long-term outcomes including valve durability and the need for reintervention are unanswered, especially in younger patients who tend to be low surgical risk. We performed a meta-analysis comparing clinical outcomes after TAVI and SAVR over 5 years stratified to low, intermediate, and high surgical risks. METHODS: We identified propensity score-matched observational studies and randomised controlled trials comparing TAVI and SAVR. Primary outcomes, including all-cause mortality, moderate or severe aortic regurgitation, moderate or severe paravalvular regurgitation, pacemaker placement, and stroke, were extracted. Meta-analyses of outcomes after TAVI compared to SAVR were conducted for different periods of follow-up. Meta-regression was also performed to analyse the correlation of outcomes over time. RESULTS: A total of 36 studies consisting of 7 RCTs and 29 propensity score-matched studies were selected. TAVI was associated with higher all-cause mortality at 4-5 years in patients with low or intermediate surgical risk. Meta-regression time demonstrated an increasing trend in the risk of all-cause mortality after TAVI compared with SAVR. TAVI was generally associated with a higher risk of moderate or severe aortic regurgitation, moderate or severe paravalvular regurgitation, and pacemaker placement. CONCLUSIONS: TAVI demonstrated an increasing trend of all-cause mortality compared with SAVR when evaluated over a long-term follow-up. More long-term data from recent studies using newer-generation valves and state-of-the-art techniques are needed to accurately assign risks.

Transcatheter aortic valve implantation (TAVI) was associated with increased all-cause mortality at longer periods of follow-up irrespective of surgical risk. Aortic regurgitation, paravalvular regurgitation, major vascular complications, and pacemaker placement favoured surgical aortic valve replacement (SAVR) over TAVI. TAVI remained superior to SAVR in major bleeding and renal failure events. Long-term data on newer generation valves and up-to-date implantation techniques may provide better durability and improved outcomes after TAVI.

Aortic Regurgitation and Heart Failure: Advances in Diagnosis, Management, and Interventions.

This review discusses the contemporary clinical evaluation and management of patients with comorbid aortic regurgitation (AR) and heart failure (HF) (AR-HF). Importantly, as clinical HF exists along the spectrum of AR severity, the present review also details novel strategies to detect early signs of HF before the clinical syndrome ensues. Indeed, there may be a vulnerable cohort of AR patients who benefit from early detection and management of HF. Additionally, while the mainstay of operative management for AR has historically been surgical aortic valve replacement, this review discusses alternate procedures that may be beneficial in high-risk cohorts.