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 .

Valvular Heart Disease in Pregnancy.

Valvular heart disease is a common cause of peripartum cardiovascular morbidity and mortality. The hemodynamic changes of pregnancy and their impact on preexisting valvular lesions are described in this paper. Tools for calculation of maternal and fetal risk during pregnancy are also discussed. The pathophysiology and management of valvular lesions, both obstructive and regurgitant, are then described, followed by discussion of mechanical and bioprosthetic valve complications during pregnancy.

Cardiac Computed Tomography of Native Cardiac Valves.

Valvular heart disease (VHD) is a significant clinical problem associated with high morbidity and mortality. Although not being the primary imaging modality in VHD, cardiac computed tomography (CCT) provides relevant information about its morphology, function, severity grading, and adverse cardiac remodeling assessment. Aortic valve calcification quantification is necessary for grading severity in cases of low-flow/low-gradient aortic stenosis. Moreover, CCT details significant information necessary for adequate percutaneous treatment planning. CCT may help to detail the etiology of VHD as well as to depict other less frequent causes of valvular disease, such as infective endocarditis, valvular neoplasms, or other cardiac pseudomasses.

A Female in Her 50s With Caseating Mitral Annulus Calcification.

This case report describes a diagnosis of caseating mitral annulus calcification in a female patient with a history of peritoneal dialysis who presented with confusion and disorientation.

Association between echocardiographic indexes and urinary Neutrophil Gelatinase-Associated Lipocalin (uNGAL) in dogs with myxomatous mitral valve disease.

Neutrophil gelatinase-associated lipocalin (NGAL) is a biomarker of tubular damage, and its elevation has been described in human and canine cardiorenal syndrome. The aim was to evaluate the association between echocardiographic indexes and urine NGAL (uNGAL) and uNGAL normalized to urine creatinine (uNGALC) in dogs with MMVD. This is a multicentric prospective cross-sectional study. A total of 77 dogs with MMVD at different ACVIM stages were included. All dogs underwent echocardiography, serum chemistry, and urinalysis. Echocardiographic data analyzed were shortening fraction (SF), left ventricular diastolic (LVIDDn) and systolic (LVIDSn) diameters normalized for body weight, left atrium to aortic root ratio (LA/Ao), maximal (LAVMax) and minimal (LAVMin) left atrial volumes, LA stroke volume (LASV), early diastolic mitral peak velocity (EVmax), EVmax to tissue Doppler E' wave (E/E'), aortic (VTIAo) and mitralic (VTIMit) velocity time integrals and their ratio (VTIMit/VTIAo), and tricuspid regurgitation velocity (TRVmax). In the univariate analysis LASV, TRVmax, LAVMax, LVIDDn, and VTIMit/VTIAo were independent predictors of increased uNGAL and uNGALC; however, only LASV [(OR: 1.96, 95% CI: 1.16 to 3.31) P = 0.01 for NGAL, and (OR: 2.79, 95% CI: 1.50 to 5.17) P < 0.001 for NGALC] and TRVmax [(OR: 1.73, 95% CI: 1.20-2.51) P = 0.002 for NGAL, and (OR: 1.50, 95% CI: 10.07-2.10) P = 0.015 for NGALC] remained statistically significant in the multivariable analysis. Based on our results, LASV and TRVmax are associated with increased uNGAL and uNGALC. These parameters might detect dogs with MMVD at higher risk of developing kidney damage.

Patient-specific analysis of bicuspid aortic valve hemodynamics using a fully coupled fluid-structure interaction model.

Bicuspid aortic valve (BAV), the most common congenital heart disease, is prone to develop significant valvular dysfunction and aortic wall abnormalities such as ascending aortic aneurysm. Growing evidence has suggested that abnormal BAV hemodynamics could contribute to disease progression. In order to investigate BAV hemodynamics, we performed 3D patient-specific fluid-structure interaction (FSI) simulations with fully coupled blood flow dynamics and valve motion throughout the cardiac cycle. Results showed that the hemodynamics during systole can be characterized by a systolic jet and two counter-rotating recirculation vortices. At peak systole, the jet was usually eccentric, with asymmetric recirculation vortices and helical flow motion in the ascending aorta. The flow structure at peak systole was quantified using the vorticity, flow rate reversal ratio and local normalized helicity (LNH) at four locations from the aortic root to the ascending aorta. The systolic jet was evaluated with the peak velocity, normalized flow displacement, and jet angle. It was found that peak velocity and normalized flow displacement (rather than jet angle) gave a strong correlation with the vorticity and LNH in the ascending aorta, which suggests that these two metrics could be used for clinical noninvasive evaluation of abnormal blood flow patterns in BAV patients.

Coarctation of Aorta With Tricuspid Aortic Valve Is Not Associated With Ascending Aortic Aneurysm.

BACKGROUND: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension. OBJECTIVES: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms. METHODS: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years. RESULTS: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups. CONCLUSIONS: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD.

Validation of fluid-structure interaction simulations of the opening phase of phantom mitral heart valves under physiologically inspired conditions.

BACKGROUND AND OBJECTIVE: Atrioventricular valve disease is a common cause of heart failure, and successful surgical or interventional outcomes are crucial. Patient-specific fluid-structure interaction (FSI) modeling may provide valuable insights into valve dynamics and guidance of valve repair strategies. However, lack of validation has kept FSI modeling from clinical implementation. Therefore, this study aims to validate FSI simulations against in vitro benchmarking data, based on clinically relevant parameters for evaluating heart valve disease. METHODS: An FSI model that mimics the left heart was developed. The domain included a deformable mitral valve of different stiffnesses run with different inlet velocities. Five different cases were simulated and compared to in vitro data based on the pressure difference across the valve, the valve opening, and the velocity in the flow domain. RESULTS: The simulations underestimate the pressure difference across the valve by 6.8-14 % compared to catheter measurements. Evaluation of the valve opening showed an underprediction of 5.4-7.3 % when compared to cine MRI, 2D Echo, and 3D Echo data. Additionally, the simulated velocity through the valve showed a 7.9-8.4 % underprediction in relation to Doppler Echo measurements. Qualitative assessment of the velocity profile in the ventricle and the streamlines of the flow in the domain showed good agreement of the flow behavior. CONCLUSIONS: Parameters relevant to the diagnosis of heart valve disease estimated by FSI simulations showed good agreement when compared to in vitro benchmarking data, with differences small enough not to affect the grading of heart valve disease. The FSI model is thus deemed good enough for further development toward patient-specific cases.

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction.

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. Objective: To deliver the first heart valve implant that grows. Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury. Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves. Main Outcomes and Measures: Transplanted valve growth and hemodynamic function. Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves. Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

Long-term outcome of bicuspid aortic valve disease.

AIMS: Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. METHODS AND RESULTS: A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). CONCLUSION: BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.

Right ventricle assessment before tricuspid valve interventions.

In the latest ESC/EACTS Guidelines for the Management of Valvular Heart Disease, right ventricular dilatation and dysfunction, severe pulmonary hypertension and tricuspid annulus dilatation were reported to be the most important parameters to consider in patient selection for tricuspid valve interventions. Indeed, comprehensive right ventricular assessment is crucial in patients with severe tricuspid regurgitation who may benefit from transcatheter or surgical procedures. However, the only guideline parameter considered for intervention has been tricuspid annular dilatation in the presence of at least mild to moderate tricuspid regurgitation, with no other right ventricular markers used in the decision-making process for invasive treatment. Notably, challenges in the assessment of right ventricular function may limit establishing thresholds for defining right ventricular dysfunction. The aim of this review is to summarize current evidence on the prognostic significance of right ventricular function in patients with tricuspid regurgitation undergoing percutaneous or surgical interventions.

Filamin A heart valve disease as a genetic cause of inherited bicuspid and tricuspid aortic valve disease.

OBJECTIVE: Variants in the FLNA gene have been associated with mitral valve dystrophy (MVD), and even polyvalvular disease has been reported. This study aimed to analyse the aortic valve and root involvement in FLNA-MVD families and its impact on outcomes. METHODS: 262 subjects (37 (18-53) years, 140 male, 79 carriers: FLNA+) from 4 FLNA-MVD families were included. Echocardiography was performed in 185 patients and histological analysis in 3 explanted aortic valves. The outcomes were defined as aortic valve surgery or all-cause mortality. RESULTS: Aortic valve alterations were found in 58% of FLNA+ compared with 6% of FLNA- (p<0.001). 9 (13.4%) FLNA+ had bicuspid aortic valve compared with 4 (3.4%) FLNA- (p=0.03). Overall, the transvalvular mean gradient was slightly increased in FLNA+ (4.8 (4.1-6.1) vs 4.0 (2.9-4.9) mm Hg, p=0.02). The sinuses of Valsalva and sinotubular junction diameters were enlarged in FLNA+ subjects (all p<0.05). 8 FLNA+ patients underwent aortic valve surgery (0 in relatives; p<0.001). Myxomatous remodelling with an infiltration of immune cells was observed. Overall survival was similar between FLNA+ versus FLNA- subjects (86±5% vs 85±6%, p=0.36). There was no statistical evidence for an interaction between genetic status and sex (p=0.15), but the survival tended to be impaired in FLNA+ men (p=0.06) whereas not in women (p=0.71). CONCLUSION: The patients with FLNA variants present frequent aortic valve disease and worse outcomes. Bicuspid aortic valve is more frequent in patients carrying the FLNA-MVD variants. These unique features should be factored into the management of patients with dystrophic and/or bicuspid aortic valve.

Cardiac valvular involvement in granulomatosis with polyangiitis in long-term observation.

INTRODUCTION AND OBJECTIVES: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is characterized by inflammation of blood vessels. The aim of the present study was to assess cardiac valvular changes in patients with GPA in a cohort of 105 patients followed for a mean of six years. METHODS: We followed 105 patients (mean age 50.4 years, 67 female) for a mean of 6.2±1.3 years. Echocardiography and laboratory tests were performed in all patients. RESULTS: At baseline, 43% of patients were diagnosed with aortic regurgitation (AR), which was the most common valvular lesion. Moreover, it was the only valvular involvement that significantly increased during observation (p=0.01). In a multivariate model, only D-dimer level was a predictor of AR in this group of patients (OR 8.0 (95% CI: 1.7-38.2, p=0.01). CONCLUSIONS: Involvement of the heart valves is a common finding in patients with GPA, but significant valvular disease is a rare complication. The most common valvular disease in this group of patients is AR. Aortic valves are also the most prone to degeneration in the course of the vasculitis.