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Introduction: Partial heart transplants are a new type of pediatric transplant that replace defective heart valves with the parts of matched donor hearts containing the necessary valves. Short‐term outcomes of partial heart transplants are excellent, but long‐term outcomes are unknown. In order to predict the long‐term outcomes of partial heart transplants, we evaluated long‐term growth and function of semilunar heart valves transplanted in infancy as part of a heart transplant. Methods: All children who underwent infant heart transplantation at a single center from 1997 to 2014 were included in this study. Children in whom echocardiograms after heart transplantation and after 10 years were not available for review were excluded. The echocardiograms were reviewed by two authors to analyze semilunar valve annulus diameters, Z‐scores, peak valve gradients, and valve regurgitation. Statistical difference was determined using two‐tailed, paired sample t‐tests with Bonferroni correction for multiple comparisons. Results: Data from 15 patients were analyzed. The aortic valve annulus averaged 1.3 cm (range 0.7–1.8 cm) immediately after transplantation and grew to an average of 1.7 cm (range 1.4–2.3 cm) after 10 years (p <.001). After 10 years, the aortic valve peak gradient avereraged 5.1 mmHg (range 2.1–15.5 mmHg) and none of the valves had more than trivial regurgitation. The pulmonary valve annulus averaged 1.5 cm (range 1.1–2.5 cm) immediately after transplantation and grew to an average of 2.1 cm (range 1.0–2.9 cm) after 10 years (p <.001). After 10 years, the pulmonary valve peak gradient averaged 4.3 mmHg (range 1.1–13.8 mmHg), and 7% of valves had moderate regurgitation. Discussion: Semilunar heart valves transplanted in infancy as part of a heart transplant demonstrate statistically significant growth and excellent function after 10 years. This predicts excellent long‐term outcomes of partial heart transplants. [ABSTRACT FROM AUTHOR]

Hypereosinophilic syndrome (HES) is considered to be a rare myeloproliferative disease that is characterized by persistent eosinophilia with associated multiple-organ damage. The heart is often involved in HES, representing a major cause of morbidity and mortality. HES is a heterogeneous group of disorders; the majority of the cases are idiopathic. Summarizing the findings regarding myocardial, valvular, and vascular abnormalities in a series of patients with HES, most studies found normal left ventricular (LV) volumes with reduced LV global longitudinal strain and LV apical rotation and twist in HES cases, accompanied by increased left atrial (LA) volumes and stroke volumes, reduced peak LA circumferential strain (representing systolic abnormalities), and mitral annular dilation and functional deterioration. Regarding the right heart, preserved right ventricular volumes and functional properties, increased right atrial volumes, mild RA functional abnormalities, and dilated tricuspid annular dimensions without functional impairment could be seen in these studies. Aortic and pulmonary valves showed no specific disease-related alterations. Vascular abnormalities included increased aortic stiffness without dilation of the aorta and pulmonary hypertension in some cases. These results suggest disease-specific but relatively mild myocardial, valvular, and vascular abnormalities in HES. The present review aimed to summarize the available clinical data about cardiac mechanics and valvular and vascular abnormalities in a series of patients with HES. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12–19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31–63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10–53 mmHg) pre-procedure to 10 mmHg (IQR 5–16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5–44 min). The large sizes—mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120–300 mL). Patients were followed for a median period of 360 days (IQR 164–525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes. [ABSTRACT FROM AUTHOR]

Appropriate load‐bearing function of soft connective tissues is provided by their nonlinear and often anisotropic mechanics. Recapitulating such complex mechanical behavior in tissue‐engineered structures is particularly crucial, as deviation from native tissue mechanics can trigger pathological biomechanical pathways, causing adverse tissue remodeling and dysfunction. Here, a novel method combining computational modeling, melt electrowriting (MEW), and design of experiments (DOE) is reported to generate scaffolds composed of sinusoidal fibers with prescribed biaxial mechanical properties, recapitulating the distinct nonlinear, anisotropic stress–strain behavior of three model tissues: adult aortic valve, pediatric pulmonary valve, and pediatric pericardium. Finite element analysis is used to efficiently optimize scaffold architecture over a broad parameter space, representing up to 65 conditions, to define MEW print parameters to achieve polycaprolactone scaffolds with target mechanical properties. Architectural parameters are further optimized experimentally using DOE and regression to account for uncertainties involved in the simulation, yielding functional scaffolds with accurate, prescribed mechanics. The prescribed architecture also primarily governs the mechanics of hybrid structures generated by casting cell‐laden fibrin hydrogel within the scaffolds. This high‐fidelity approach recapitulates biaxial mechanical properties over a broad range of mechanical nonlinearity and anisotropy and is generalizable for programmed biofabrication in a variety of tissue engineering applications. [ABSTRACT FROM AUTHOR]

Key Points: Question: Do partial heart transplants deliver growing heart valve implants for neonates? Findings: The first-in-human partial heart transplant demonstrated valve growth during follow-up for more than 1 year. Meaning: Partial heart transplant is a new approach to treat neonates with irreparable heart 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. This case report documents the first partial heart transplant in a neonate with irreparable heart valve dysfunction to deliver aortic and pulmonary valves that grow with the developing child. [ABSTRACT FROM AUTHOR]

This article discusses the use of the Ross/Ross-Konno procedure in pediatric cardiac surgery for aortic valve diseases and left ventricle outflow tract obstruction. The study analyzes the mid- and long-term clinical results of over 20 years of experience with this procedure. The results show successful long-term durability, low risk of complications, good hemodynamic performance, and potential for growth. The procedure offers a longer-lasting solution and avoids the need for lifelong anticoagulant therapy. However, there is a relatively common occurrence of homograft degenerations that require interventions, especially after the Ross-Konno procedure. [Extracted from the article]

The most performed percutaneous valve replacement procedure is for the aortic valve. In recent years, there have been developments in percutaneous valve replacement methods for other valves, including the pulmonary valve. Computed tomography plays a crucial role in various stages of procedure planning and provides essential information regarding potential complications after the procedure. We present images documenting step by step how to evaluate cardiac computed tomography in the qualification procedure for transcatheter pulmonary valve implantation in patients with dysfunctional native right ventricular outflow tract. [ABSTRACT FROM AUTHOR]

The pulmonary autograft in the Ross procedure, where the aortic valve is replaced by the patient's own pulmonary valve, is prone to failure due to dilatation. This is likely caused by tissue degradation and maladaptation, triggered by the higher experienced mechanical loads in aortic position. In order to further grasp the causes of dilatation, this study presents a model for tissue growth and remodeling of the pulmonary autograft, using the homogenized constrained mixture theory and equations for immuno- and mechano-mediated mass turnover. The model outcomes, compared to experimental data from an animal model of the pulmonary autograft in aortic position, show that inflammation likely plays an important role in the mass turnover of the tissue constituents and therefore in the autograft dilatation over time. We show a better match and prediction of long-term outcomes assuming immuno-mediated mass turnover, and show that there is no linear correlation between the stress-state of the material and mass production. Therefore, not only mechanobiological homeostatic adaption should be taken into account in the development of growth and remodeling models for arterial tissue in similar applications, but also inflammatory processes. [ABSTRACT FROM AUTHOR]

To determine reference curves for fetal aortic and pulmonary valve annulus area by three-dimensional ultrasonography using the spatio-temporal image correlation (STIC) in the rendering mode, and to ascertain its applicability in congenital heart disease (CHD). We performed a retrospective cross-sectional study of 328 normal fetuses and 42 fetuses with CHD between 20 and 33 weeks 6 days of gestation. The outflow plane view of the great vessels was used to measure the areas of the valvar annuli, and the measurements were performed in systole. A linear regression model adjusted according to the determination coefficient (R2) was utilized to construct the reference intervals. The concordance correlation coefficient (CCC) was used to calculate the reproducibility of the mitral and tricuspid valve areas. The mean ± standard deviation (SD) of the aortic and pulmonary valve annulus areas ranged from 6.6 ± 1.2 to 32.9 ± 1.1 mm2 and 10.7 ± 1.3 to 40.3 ± 1.2 mm2, respectively. We observed a linear relationship and strong positive correlation between the area of the aortic and pulmonary valve annuli with r=0.97 and 0.96, respectively. Good intra (CCC=0.99) and interobserver agreement (CCC=0.98) was observed for the measurement of the aortic valve annulus area. A good intra (CCC=0.99) and interobserver (CCC=0.97) agreement was also observed for the measurement of the pulmonary valve annulus area. The mean ± SD of the difference of the areas of the aortic and pulmonary valve annuli between the normal fetuses and those with CHD were −1.801 ± 1.429 mm2 (p=0.208) and −1.033 ± 1.467 mm2 (p<0.0001), respectively. The reference curves for the areas of the aortic and pulmonary valve annuli of fetal hearts were determined, and showed good inter and intraobserver reproducibility. The constructed reference curves showed applicability in different types of CHD. [ABSTRACT FROM AUTHOR]

Introduction: The influence of hypertension on the diagnostic assessment of aortic stenosis (AS) severity is unclear, yet clinically relevant. To clarify the effect of hypertension on transvalvular gradients, requires a better understanding of the impact that blood pressure change has on mean flow rate. Also, the effect of various degrees of AS severity, the valve geometry and intrinsic left ventricular contractile function (elastance) on this interaction, needs to be clarified. The current work aims to assess this interaction and the magnitude of these effects. Methods: A validated, zero-dimensional electro-hydraulic analogue computer model of the human cardiovascular circulatory system was generated. It was used to assess the impact of blood pressure changes on left ventricular pressure and transvalvular gradients at various flow rates, left ventricular elastances, a range of aortic valve areas and for different aortic valve morphologies. Results and discussion: The magnitude of the impact of hypertension induced changes on the mean gradient (MG) is influenced by the mean flow rate, the AS severity, the hydraulic effective valve orifice area and the left ventricular elastance. Generally, for a given change in systemic arterial pressure, the impact on MG will be the most marked for lower flow rate states such as is expected in more severe degrees of AS, for worse intrinsic left ventricular (LV) contractility, shorter ejection times and lower end diastolic LV volumes. Given the above conditions, the magnitude of the effect will be more for a larger aortic sinus diameter, and also for a typical degenerative valve morphology compared to a typical rheumatic valve morphology. Conclusion: The interaction between hypertension and mean gradients in AS is complex. The current work places previous recommendations in perspective by quantifying the magnitude of the effect that the changes in blood pressure has on mean gradient in various pathophysiological states. The work creates a framework for the parameters that should be considered in future clinical research on the topic. [ABSTRACT FROM AUTHOR]

The Ross procedure allows replacement of a diseased aortic valve with pulmonary root autograft, possibly avoiding the highly thrombotic mechanical valves and immunologic deterioration of tissue valves in antiphospholipid syndrome (APS). Here, we present the use of the Ross procedure in a 42‐year‐old woman with mild intellectual disability, APS, and a complex anticoagulation history after she presented with thrombosis of her mechanical On‐X aortic valve previously implanted for non‐bacterial thrombotic endocarditis. [ABSTRACT FROM AUTHOR]

This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7 years (interquartile range: 0–17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery were factors associated with post-operative residual aortic regurgitation. Mild pulmonary regurgitation was observed in 12%, 30%, and 40% of patients 5, 10, and 15 years after surgery, respectively. There were no significant differences in age and weight at which surgery was performed between patients with mild pulmonary regurgitation and those with less than mild pulmonary regurgitation. However, the number of sutures across the pulmonary valve was associated with post-operative pulmonary regurgitation (P < 0.01). As some patients with mild pre-operative aortic regurgitation may not improve even after surgery, early surgical intervention is necessary when aortic regurgitation appears. Some patients may develop post-operative pulmonary regurgitation in the long term, suggesting the need for careful follow-up. [ABSTRACT FROM AUTHOR]

Aortic valve replacement early in life may be inevitable. Ross operation, until present day, remains the favorite surgical option in pediatrics with irreparable aortic valve disease. Nonetheless, the necessity for re-operation was always its principal limitation due to aortic valve failure or homograft degeneration. We present our 25 years of experience in the pediatric population. From August 1994 until June 2018, 157 children below 18 years underwent the Ross operation. This retrospective review aims at assessing the long-term outcomes, as well as the risk factors for re-operation after Ross procedure. Median age was 10.9 years, of which seven patients were infants, 79 children, and 71 adolescents. The median follow-up time was 14 years. Hospital mortality was 0.6%. Freedom from autograft re-operation for children was 96.7% and 94.1% at 10 and 20 years, respectively; whereas for adolescents, it was 92.6% and 74.9% at 10 and 20 years. For children, freedom from homograft re-operation was 92.5%, 83.5%, and 56.2% at 10, 15, and 20 years; while for adolescents, it was 96.8%, 91.8%, and 86.7% at 10, 15, and 20 years. Homograft size (p = 0.008) and childhood (p = 0.05) were risk factors for homograft re-operation. Pulmonary valve diameter > 24 mm (p = 0.044) and adolescence (p = 0.032) were risk factors for autograft re-operation. Our experience demonstrated excellent early and late survival. While children have preferential outcomes concerning autograft re-operation, those who received a smaller homograft had a higher right-sided re-intervention incidence than adolescents. Pulmonary diameter > 24 mm at surgery was an indicator of future autograft failure. [ABSTRACT FROM AUTHOR]

Objectives: The objective of this study was to evaluate the predictive value of main pulmonary artery (MPA) Z score, pulmonary valve annulus (PVA) prop, great aortic valve annulus (GA) ratio, PVA index (PAI), and PVA area index (PAAI) indicators in patients with Tetralogy of Fallot (TOF) and whether or not to undergo transannular patch (TAP). Methods: A retrospective analysis was performed on the clinical data of 263 patients with TOF who underwent radical operations from 2010 to 2021 at Beijing Children's Hospital. Sixteen cases were excluded, and 247 cases (male/female = 155/92) were included in this study. Based on whether TAP was selected intraoperatively, the patients were divided into the TAP group (82/247) and the non‐TAP group (165/247). The diameter of the PVA, the aortic valve annulus, and the MPA were measured by echocardiography, and the PVA Z score, MPA Z score, PVA prop, GA ratio, PAI, and PAAI indexes were calculated, and statistical analysis was carried out. Results: The PVA Z score, MPA Z score, PVA prop, GA ratio, PAI, and PAAI of the TAP group were lower than those of the non‐TAP group (p <.0001, p <.0001, p <.0001, p <.0001, p <.0001, and p <.0001). Receiver‐operating curve analysis showed that the cut‐off value of PVA Z score was −1.96 (area under the curve [AUC]: 0.822; 95% confidence interval [CI]: 0.769–0.874); the cut‐off value of MPA Z score was −1.04 (AUC: 0.778; 95% CI: 0.711–0.845); the cut‐off value of PVA prop was 0.37 (AUC: 0.812; 95% CI: 0.751–0.874); the cut‐off value of GA ratio was 0.64 (AUC: 0.812; 95% CI: 0.750–0.874); the cut‐off value of PAI is 0.78 (AUC: 0.812; 95% CI: 0.750–0.874); and the cut‐off value of PAAI is 0.4 (AUC: 0.812; 95% CI: 0.750–0.874). Pulmonary valve bicuspid malformation is one reason why predictive models fail to predict the possible avoidance of TAP. Pearson's correlation and linear regression analysis showed that PAI had the strongest correlation with PVA Z score, followed by that between PVA prop and PVA Z score, and the weakest correlation between PAAI and PVA Z score. Conclusions: PVA prop, GA ratio, PAI, and PAAI can well predict TAP selection, and the measurement is simple and convenient. Compared with PVA Z score, they are not hindered by other confounding factors, and can well replace the application value of PVA Z score in TAP prediction. The predictive efficacy of PAI and PVA prop is numerically better than the GA ratio, and PAAI, PAI, and PVA prop combined with MPA Z score can improve the predictive value of PAI and PVA prop, respectively. Although various echocardiographic parameters can be used as indicators to predict surgical approach in patients with TOF, PV morphology and tissue characteristics should also be considered. [ABSTRACT FROM AUTHOR]

We present a case of a 47-year-old female who presented with septic shock and a hyperosmolar hyperketotic state, accompanied by episodes of unresponsiveness. As part of the extensive investigations which took place, a transoesophageal echocardiogram (TOE) revealed infective endocarditis of the tricuspid, pulmonary and aortic valves. Blood cultures showed evidence of bacteraemia with Streptococcus agalactiae. After an initial improvement on intravenous antibiotics, the patient's condition deteriorated following multiple septic emboli to the lungs from the pulmonary valve vegetation, leading to urgent referral for valve replacement. A mechanical aortic, tissue pulmonary and tricuspid valve replacements were performed in a tertiary centre in the United Kingdom. The postoperative course was complicated by recurrent infections of the sternotomy wound with eventual wound dehiscence and overwhelming sepsis. [ABSTRACT FROM AUTHOR]

Background: Traditionally, the pulmonary valve annulus (PVA) z-score is used to predict the requirement of transannular patch enlargement (TAPE) of right ventricular outflow tract (RVOT) and main pulmonary artery (MPA) in repair of tetralogy of Fallot (TOF). PVA z-score is highly variable and many other parameters are being reported to be accurate in predicting need of TAPE. In this study, we analyze the role of great artery annulus ratio (pulmonary valve annulus to aortic valve annulus ratio, PVA/AVA) to be used as a predictor for TAPE. Methods: We analyzed 90 patients of TOF retrospectively who underwent repair between January 2021 and December 2021. The patients were divided as TAPE group who required TAPE of RVOT and MPA and non-TAPE group who did not have TAPE. Their baseline parameters, PVA Z-score, and cut-off great artery annulus ratio were compared. Results: Total 44 (48.9%) patients had transannular patch (TAPE) repair and 46 (51.1%) patients had non-transannular patch repair. The great artery annulus ratio and PVA z-score was lower in case of TAPE group with statistical significance. Receiver operating characteristic curve analysis showed great artery annulus ratio as more accurate predictor of TAPE. Conclusion: Great artery annulus ratio can be used as a simple and accurate predictor for transannular patch enlargement along with PVA z-score during repair of TOF. [ABSTRACT FROM AUTHOR]

Introduction: The choice of valve substitute for aortic valve surgery is tailored to the patient with specific indications and contraindications to consider. The use of an autologous pulmonary artery (PA) with a simultaneous homograft in the pulmonary position is called a Ross procedure. It permits somatic growth and the avoidance of lifelong anticoagulation. Concerns remain on the functionality of a pulmonary autograft in the aortic position when exposed to systemic pressure. Methods: A literature review was performed incorporating the following databases: Pub Med (1996 to present), Ovid Medline (1958 to present), and Ovid Embase (1982 to present), which was run on 1 January 2022 with the following targeted words: biomechanics of pulmonary autograft, biomechanics of Ross operation, aortic valve replacement and pulmonary autograph, aortic valve replacement and Ross procedure. To address the issues with heterogeneity, studies involving the pediatric cohort were also analyzed separately. The outcomes measured were early- and late-graft failure alongside mortality. Results: a total of 8468 patients were included based on 40 studies (7796 in pediatric cohort and young adult series and 672 in pediatric series). There was considerable experience accumulated by various institutions around the world. Late rates of biomechanical failure and mortality were low and comparable to the general population. The biomechanical properties of the PA were superior to other valve substitutes. Mathematical and finite element analysis studies have shown the potential stress-shielding effects of the PA root. Conclusion: The Ross procedure has excellent durability and longevity in clinical and biomechanical studies. The use of external reinforcements such as semi-resorbable scaffolds may further extend their longevity. [ABSTRACT FROM AUTHOR]

Background: We discuss a rare case of an adult patient with different pathologies involving the aortic and pulmonary valves in need of surgery. Case presentation: The patient had a history of congenital PV stenosis and surgical valvuloplasty. Almost 50 years later the patient underwent a complex second heart surgery due to infective endocarditis of the aortic valve and high-grade restenosis of the pulmonary valve. Replacement of the aortic and pulmonary valve, as well as reconstruction of the RVOT and closure of a persistent foramen ovale, followed. Postoperative course was uneventful and the patient was discharged home a week after surgery. Conclusion: Simultaneous surgery of pulmonary and aortic valves due to different pathologies is rare but can be performed successfully even in advanced age and can improve quality of life. [ABSTRACT FROM AUTHOR]

Endothelial cells (ECs) are critical to proper heart valve development, directly contributing to the mesenchyme of the cardiac cushions, which progressively transform into mature valves. To date, investigators have lacked sufficient markers of valve ECs to evaluate their contributions during valve morphogenesis fully. As a result, it has been unclear whether the well-characterized regional differentiation of valves correlates with any endothelial domains in the heart. Furthermore, it has been difficult to ascertain whether endothelial heterogeneity in the heart influences underlying mesenchymal zones in an angiocrine manner. To identify regionally expressed EC genes in the heart valves, we screened publicly available databases and assembled a toolkit of endothelial-enriched genes. We identified Cyp26b1 as one of many endothelial enriched genes found to be expressed in the endocardium of the developing cushions and valves. Here, we show that Cyp26b1 is required for normal heart valve development. Genetic ablation of Cyp26b1 in mouse embryos leads to abnormally thickened aortic valve leaflets, which is due in part to increased endothelial and mesenchymal cell proliferation in the remodeling valves. In addition, Cyp26b1 mutant hearts display ventricular septal defects (VSDs) in a portion of null embryos. We show that loss of Cyp26b1 results in upregulation of retinoic acid (RA) target genes, supporting the observation that Cyp26b1 has RA-dependent roles. Together, this work identifies a novel role for Cyp26b1 in heart valve morphogenesis and points to a role of RA in this process. Understanding the spatiotemporal expression dynamics of cardiac EC genes will pave the way for investigation of both normal and dysfunctional heart valve development. [Display omitted] • A mouse heart valve gene expression atlas was generated with publicly available online gene expression databases. • The endothelium of developing mouse heart valves is regionally heterogeneous. • Cyp26b1 is expressed in the endocardial/endothelial lining of developing heart valves. • Loss of Cyp26b1 leads to significant enlargement of aortic valves and to ventricular septal defects. • Retinoic acid targets are upregulated in Cyp26b1 −/− heart valves, indicating dysregulation of RA metabolism. [ABSTRACT FROM AUTHOR]

Surgical simulation is becoming increasingly important in training cardiac surgeons. However, there are currently no training simulators capable of testing the quality of simulated heart valve procedures under dynamic physiologic conditions. Here we describe a dynamic ventricular simulator, consisting of a 3D printed valve suspension chamber and a model 1423 Harvard apparatus pulsatile pump, which can provide close to physiologic hemodynamic perfusion of porcine aortic roots attached to the valve chamber for education and training in cardiac surgery. The simulator was validated by using it to test aortic valve leaflet repairs (n = 6) and aortic valve replacements (n = 3) that were performed by two trainees. Procedural success could be evaluated by direct visualization of the opening and closing valve, hemodynamic measurements and echocardiography. We conclude that, unlike other methods of simulation, this novel ventricular simulator is able to test the functional efficacy of aortic procedures under dynamic physiologic conditions using clinically relevant echocardiographic and hemodynamic outcomes. While validated for valve surgery, other potential applications include ascending aortic interventions, coronary re-implantation or catheter-based valve replacements. [ABSTRACT FROM AUTHOR]

This paper provides a review of engineering applications and computational methods used to analyze the dynamics of heart valve closures in healthy and diseased states. Computational methods are a cost-effective tool that can be used to evaluate the flow parameters of heart valves. Valve repair and replacement have long-term stability and biocompatibility issues, highlighting the need for a more robust method for resolving valvular disease. For example, while fluid–structure interaction analyses are still scarcely utilized to study aortic valves, computational fluid dynamics is used to assess the effect of different aortic valve morphologies on velocity profiles, flow patterns, helicity, wall shear stress, and oscillatory shear index in the thoracic aorta. It has been analyzed that computational flow dynamic analyses can be integrated with other methods to create a superior, more compatible method of understanding risk and compatibility. [ABSTRACT FROM AUTHOR]

Background: Pulmonary regurgitation is imminent after transannular patch (TAP). We analyze the long-term performance of untreated autologous pericardium (UAP) as valve substitute at pulmonary position in patients requiring TAP.Material and Methods: This cross-sectional study include patients operated between 2007 and 2012 (n = 92). A sample of 19 patients was selected for this study which had a follow-up of more than 3 years. This includes patients with no TAP (n = 4) and with TAP and valve substitute, a monocusp (n = 11) or a tricuspid valve (n = 4) at neopulmonary annulus. Patients underwent echocardiography for assessment of right ventricle function and 18 fluoro-deoxyglucose PET CT scan for measurements of valve substitute at neopulmonary annulus. The target to blood ratio (TBR) of uptake of glucose by monocusp was measured at the cooptation edge of the neopulmonary valve.Results: The median age of the patients is 14 (9 - 37). RV function is preserved (TAPSE 18.9 (10.6 - 22.8)) at a mean follow-up of 4 years (3-9). The measurements of monocusp shows a shrinkage in height of the cusp by 35.5% (70% - 1.0%) and length by 7% (-44% - +104%). There was less shrinkage observed in patients below 15 years of age. The TBR of monocusp was 0.945 (0.17 - 3.35) with a strong correlation between the TBR values of aortic valve leaflet and monocusp leaflet of same patient.Conclusion: The UAP is functional and successful as a valve substitute at neo pulmonary annulus at long-term follow-up. It has resisted calcification and has shown uptake of glucose in physiological limits. [ABSTRACT FROM AUTHOR]

Objective: Most of the available information on the natural history of aortic stenosis (AS) and pulmonary stenosis (PS) in children is based on studies carried out over the past 35-40 years using cardiac catheterization. This study aimed to reveal the natural history of congenital isolated mild valvular AS and PS in children using serial Doppler echocardiographic examinations. Materials and Methods: A total of 125 children, 50 with mild AS and 75 with mild PS, who underwent Doppler echocardiography were included in this study. The prognoses of mild AS and PS were compared according to age, gender, and valvular gradient at the time of initial diagnosis. Results: The mean age of patients was 26.1±37.6 months at diagnosis. The mean follow-up duration was 27.65±21.60 (1- 120) months. There was a significant decrease in the final gradient of the PS group compared to the baseline (23.58±6.97 vs. 19.88±11.21 mmHg, p=0.001). In the AS group, there was an increase in the final gradient, which was more pronounced in patients ≤1-year-old (22.42±6.12 vs. 27.74±14.12 mmHg, p=0.002). Four percent of patients in the PS group and 12% of patients in the AS group progressed to moderate to severe stenosis. All patients who progressed in the PS group were ≤1-year-old and male. Conclusion: The results showed that mild PS had a better prognosis than mild AS and that the risk of progression in AS was higher. Careful follow-up should be performed in mild PS cases ≤1-yearold, especially in boys, since progression may be detected, even if infrequently. Mild AS should also be followed closely, as the disease may show progressive characteristics in all age groups. [ABSTRACT FROM AUTHOR]

Surgical treatment of infective endocarditis remains a challenge, with concerns of optimal prosthesis selection and risks of recurrent infection remaining paramount. The pulmonary autograft has unique features which may make it the ideal aortic valve substitute, especially in infectious endocarditis. We describe strategic considerations and technical details in performing a Ross procedure in a young patient with acute aortic valve endocarditis. [ABSTRACT FROM AUTHOR]

Purpose: Cardiac effects after breast cancer radiation therapy potentially affect more patients as survival improves. The heart's heterogeneous radiation exposure and composition of functional structures call for establishing individual relationships between structure dose and specific late effects. However, valid dosimetry requires reliable contouring which is challenging for small volumes based on older, lower-quality computed tomography imaging. We developed a heart atlas for robust heart contouring in retrospective epidemiologic studies.Methods and Materials: The atlas defined the complete heart and geometric surrogate volumes for six cardiac structures: aortic valve, pulmonary valve, all deeper structures combined, myocardium, left anterior myocardium, and right anterior myocardium. We collected treatment planning records from 16 patients from 4 hospitals including dose calculations for 3D conformal tangential field radiation therapy for left-sided breast cancer. Six observers each contoured all patients. We assessed spatial contouring agreement and corresponding dosimetric variability.Results: Contouring agreement for the complete heart was high with a mean Jaccard similarity coefficient (JSC) of 89%, a volume coefficient of variation (CV) of 5.2%, and a mean dose CV of 4.2%. The left (right) anterior myocardium had acceptable agreement with 63% (58%) JSC, 9.8% (11.5%) volume CV, and 11.9% (8.0%) mean dose CV. Dosimetric agreement for the deep structures and aortic valve was good despite higher spatial variation. Low spatial agreement for the pulmonary valve translated to poor dosimetric agreement.Conclusions: For the purpose of retrospective dosimetry based on older imaging, geometric surrogate volumes for cardiac organs at risk can yield better contouring agreement than anatomical definitions, but retain limitations for small structures like the pulmonary valve. [ABSTRACT FROM AUTHOR]

Objective: To investigate prenatal changes in cardiac biometric and flow parameters in fetuses with bicuspid aortic valve (BAV) diagnosed neonatally compared with controls with normal cardiac anatomy. Methods: This analysis was conducted as part of the Copenhagen Baby Heart Study, a multicenter cohort study of 25 556 neonates that underwent second‐trimester anomaly scan at 18 + 0 to 22 + 6 weeks' gestation and neonatal echocardiography within 4 weeks after birth, in Copenhagen University Hospital Herlev, Hvidovre Hospital and Rigshospitalet in greater Copenhagen, between April 2016 and October 2018. From February 2017 (Rigshospitalet) and September 2017 (Herlev and Hvidovre hospitals), the protocol for second‐trimester screening of the heart was extended to include evaluation of the four‐chamber view, with assessment of flow across the atrioventricular valves, sagittal view of the aortic arch and midumbilical artery and ductus venosus pulsatility indices. All images were evaluated by two investigators, and cardiac biometric and flow parameters were measured and compared between cases with BAV and controls. All cases with neonatal BAV were assessed by a specialist. Maternal characteristics and first‐ and second‐trimester biomarkers were also compared between the two groups. Results: Fifty‐five infants with BAV and 8316 controls with normal cardiac anatomy were identified during the study period and assessed using the extended prenatal cardiac imaging protocol. There were three times as many mothers who smoked before pregnancy in the group with BAV as in the control group (9.1% vs 2.7%; P = 0.003). All other baseline characteristics were similar between the two groups. Fetuses with BAV, compared with controls, had a significantly larger diameter of the aorta at the level of the aortic valve (3.1 mm vs 3.0 mm (mean difference, 0.12 mm (95% CI, 0.03–0.21 mm))) and the pulmonary artery at the level of the pulmonary valve (4.1 mm vs 3.9 mm (mean difference, 0.15 mm (95% CI, 0.03–0.28 mm))). Following conversion of the diameter measurements of the aorta and pulmonary artery to Z‐scores and Bonferroni correction, the differences between the two groups were no longer statistically significant. Pregnancy‐associated plasma protein‐A (PAPP‐A) multiples of the median (MoM) was significantly lower in the BAV group than in the control group (0.85 vs 1.03; P = 0.04). Conclusions: Our findings suggest that fetuses with BAV may have a larger aortic diameter at the level of the aortic valve, measured in the left‐ventricular‐outflow‐tract view, and a larger pulmonary artery diameter at the level of the pulmonary valve, measured in the three‐vessel view, at 20 weeks' gestation. Moreover, we found an association of maternal smoking and low PAPP‐A MoM with BAV. © 2021 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Infective endocarditis remains a medical challenge among urgent cases of cardiac disease. Multi-valvular endocarditis is uncommon and simultaneous right and left-sided valvular involvement, particularly affecting the pulmonary valve, is scarcely reported. A rare case of a patient with subacute myelodysplastic syndrome, who presented with endocarditis involving both aortic and pulmonary valves, complicated with new-onset heart failure, is described. The patient presented prompt recovery of both right and left ventricular function after combined aortic and pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Ventricular tachycardia and premature ventricular complexes (PVCs) arising from right ventricular outflow tract (RVOT) are the most common type of ventricular arrhythmias (VAs) in patients without structural heart disease. Radiofrequency ablation is now the gold standard of treatment in this setting due to high efficacy rates and optimal safety profile. During the last few years, the pulmonary valve (PV) and the pulmonary artery (PA) have attracted much attention as reliable sites of origin of RVOT‐type arrhythmias. In the mean while intracardiac echocardiogram (ICE) has undoubtedly improved our understanding of the cardiac anatomy. Aim of this paper is to provide an illustrated step‐by‐step guide on how to use ICE with the CARTOSOUND module to visualize and reconstruct 3D shell of the RV, the PV, as well of other contiguous anatomical structures (i.e., the aortic valve and coronary arteries) to perform aware and safe ablation in this region. [ABSTRACT FROM AUTHOR]

Pathogenic heterozygous variants in the NOTCH1 gene are known to be associated with both left and right‐sided congenital cardiac anomalies with strikingly incomplete penetrance and variable phenotypic expressivity. De novo NOTCH1 whole gene deletion has been reported rarely in the literature and its association with cardiac defects is less well established. Here, we report four cases of NOTCH1 gene deletion from two families associated with a spectrum of congenital heart defects from bicuspid aortic valve to complex cardiac anomalies. This is the first description of a familial NOTCH1 deletion, showing apparently high penetrance, which may be unique to this mechanism of disease. Immunohistochemical staining of cardiac tissue demonstrated reduced levels of NOTCH1 expression in both the left and right ventricular outflow tracts. These cases suggest that haploinsufficiency caused by NOTCH1 gene deletion is associated with both mild and severe cardiac defects, similar to those caused by pathogenic variants in the gene, but with apparently higher, if not complete, penetrance. [ABSTRACT FROM AUTHOR]

Perventricular pulmonary valve implantation (PPVI) of a xenograft valve can be a less invasive technique that avoids cardiopulmonary bypass in patients who require pulmonary valve replacement. We compared the hemodynamics, durability, and histologic changes between two different xenogenic valves (pericardial vs. aortic valve porcine xenografts) implanted into the pulmonary valve position using a PPVI technique and evaluated the safety and efficacy of PPVI as a preclinical study. In 18 sheep, pericardial (group porcine pericardial [PP], n = 9) or aortic valve (group porcine aortic valve [PAV], n = 9) xenogenic porcine valves manufactured as a stented valve were implanted using a PPVI technique. The porcine tissues were decellularized, alpha‐galactosidase treated, fixed with glutaraldehyde after space‐filler treatment, and detoxified to improve durability. Hemodynamic and immunohistochemical studies were performed after the implantation; radiologic and histologic studies were performed after a terminal procedure. All stented valves were positioned properly after the implantation, and echocardiography and cardiac catheterization demonstrated good hemodynamic state and function of the valves. All the anti‐α‐Gal IgM and IgG titers were below 0.3 optical density. Computed tomography of extracted valves demonstrated no significant differences in the degree of calcification between the two groups (P =.927). Microscopic findings revealed a minimal amount of calcification and no significant infiltration of macrophage or T‐cell in both groups, regardless of the implantation duration. The PPVI is a feasible technique. Both stented valves made of PP and PAV showed no significant differences in hemodynamic profile, midterm durability, and degree of degenerative dystrophic calcification. [ABSTRACT FROM AUTHOR]

Background. The pulmonary autograft is currently the best valve substitute in terms of longevity and performance. However, there is no agreement about the optimal method of insertion (sub-coronary position or freestanding root). Objectives. We sought to examine the clinical status, detailed imaging and morphometric changes in an explanted pulmonary autograft 22 years after sub-coronary implantation. Methods. A 30-year-old female underwent pulmonary autograft replacement of a severely stenotic valve at the age of 7 years, after presenting to us with signs of moderate to severe heart failure. She underwent clinical examination, detailed imaging including echocardiographic and CT examination with computerised image analysis. The explanted valve was examined by morphometry. Results. Clinical examination showed signs of heart failure (NYHA III). Trans-thoracic and trans-oesophageal 2D echo showed severe malfunction of both the aortic and pulmonary valves associated with dilatation and hypertrophy of both the right and left ventricles. Surgical correction was performed by replacing both the pulmonary and aortic valves with Medtronic 27mm Freestyle valves. The pulmonary autograft showed degeneration of the trilamellar layering of the leaflets, loss and disorganisation of GAGs, increased collagen with fibrotic overgrowth, and markers of fibrosis, inflammation, and calcification. Post-operative imaging showed good correction of the haemodynamic lesions. Conclusion. The pulmonary autograft implanted into the sub-coronary position presented with adverse remodelling, which was detrimental to the functionality and longevity of the valve. [ABSTRACT FROM AUTHOR]

• Lambl excrescences (LEs) are common in healthy hearts, but less common than previously reported. • There is a strong correlation between LE prevalence and age, supporting the idea that LEs may stem from age-related degeneration of valve cusps. • Pulmonary valve LEs are much more uncommon than those arising from the aortic valve. Lambl excrescences (LEs) were initially described in the mid-1800s during autopsies of human hearts, and their significance and biology have been debated ever since. LEs are typically found on aortic and pulmonary valve (semilunar) cusps. There is debate concerning whether LEs are a significant cause of thromboembolic events, or whether they are harmless growths. However, there have not been many reports discussing LEs, and fewer still have examined the prevalence and characteristics of LEs in healthy human hearts. Those who have examined LE prevalence have reported a very high incidence of LEs (85–90%). Herein, we examine LE prevalence and characteristics (size, location, number) in 403 healthy human hearts across all age groups. We find that the prevalence of LEs in healthy hearts is far lower than previously reported. [ABSTRACT FROM AUTHOR]

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions. [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide Aortic stenosis or regurgitation that requires operations in children often results from unicuspid valve morphology. In all paediatric patients with this anomaly, we have performed unicuspid valve repair by bicuspidization, creating a new commissure via adding patch material. This study reviewed our experience with this procedure. METHODS All patients with a unicuspid aortic valve who underwent bicuspidization at ≤18 years of age between 2003 and 2018 were evaluated. Autologous pericardium had initially been used for cusp augmentation. Since 2014, decellularized xenogeneic tissue or expanded polytetrafluoroethylene membrane has been applied. RESULTS There were 60 consecutive patients. The median operative age was 13 (1–18) years. Thirty patients had prior surgical or catheter valvuloplasties. Aortic regurgitation, aortic stenosis and their combination were present in 22, 11 and 27 patients, respectively. Autologous pericardium decellularized tissue and expanded polytetrafluoroethylene were used in 45, 11 and 4 patients, respectively, without intraoperative conversion to valve replacement. The overall survival was 96% at 10 years with 2 late deaths. Twenty patients underwent aortic valve reoperation due to patch degeneration (n  = 11), suture dehiscence of the patch (n  = 3), subaortic stenosis (n  = 3) or other reasons (n  = 3). Freedom from aortic valve reoperation was 73% and 50% at 5 and 10 years, respectively. Fifteen patients (25%) required valve replacement with pulmonary autograft (n  = 14) or prosthesis (n  = 1) 5.2 (0.2–13) years after bicuspidization at the age of 19 (10–32) years. CONCLUSIONS Bicuspidization is a safe and reproducible alternative to valve replacement with acceptable freedom from reoperation. It can serve as a bridge to valve replacement providing superior outcomes in adults. [ABSTRACT FROM AUTHOR]

The Ross procedure has been considered in children as an optimal surgical procedure due to potential growth of the aortic annulus, lack of anticoagulation requirement, very low morbidity rate and excellent survival. Five-hundred-thirty-six (366 male, mean age 29.4 ± 11.1 years) underwent Ross procedure between 1990 and 2016 and had complete clinical and echocardiographic follow-up. Mean follow-up was 16.3 ± 4.9 years. Patients were divided in 2 groups according to age at surgery. Group 1 consisted of 320 (60%) patients less than 18 years old (223 male, mean age at surgery of 9.5 ± 5.6 years). Group 2 consisted of 216 (40%) patients older than 18 years of age (143 male, mean age at surgery of 26.3 ± 8.2 years). One-hundred-thirty (24%) patients had a redo procedure or surgery. Freedom from all re-operation and or percutaneous reintervention on either the aortic and pulmonary valves was 99% after 1 year, 94% after 5 years, 89% after 10 years, 83% after 15 years and 78% after 20 years. Freedom from redo surgery for AV 99% after 1 year, 94% after 5 years, 90% after 10 years, 81% after 15 years and 80% after 20 years. Freedom from redo surgery for PV was 100% after 1 year, 95% after 5 years, 89% after 10 years, 78% after 15 years and 76% after 20 years. The ideal candidate for Ross operation is a patient with congenital aetiology and an aortic root diameter ≤ 15 mm/m2. A pulmonary fresh preserved homograft seems to perform better on the long term. • The use of the Ross procedure represents <1% of all aortic valve replacements. • Freedom from all re-operation was 89% after 10 years and 83% after 15 years. • Freedom from redo surgery for AV was 90% after 10 years and 81% after 15 years. • Rheumatic valves and aortic root diameter > 15 mm/m2 have worse outcome. • A pulmonary fresh preserved homograft seems to perform better on the long term. [ABSTRACT FROM AUTHOR]

Background: Bicuspid aortic valve is the most common congenital valvular heart defect in the general population. BAV is associated with significant morbidity due to valve failure, formation of thoracic aortic aneurysm, and increased risk of infective endocarditis and aortic dissection. Loss of function mutations in NOTCH1 (OMIM 190198) has previously been associated with congenital heart disease involving the aortic valve, left ventricle outflow tract, and mitral valve that segregates in affected pedigrees as an autosomal dominant trait with variable expressivity. Methods: We performed whole‐exome sequencing in four members of a three‐generational family (three affected and one unaffected subject) with clinical phenotypes including aortic valve stenosis, thoracic aortic aneurysm, and ventricular septal defect. Results: We identified 16 potentially damaging genetic variants (one stop variant, one splice variant, and 14 missense variants) cosegregating with the phenotype. Of these variants, the nonsense mutation (p.Tyr291*) in NOTCH1 was the most deleterious variant identified and the most likely variant causing the disease. Conclusion: Inactivating NOTCH1 mutations are a rare cause of familial heart disease involving predominantly left ventricular outflow tract lesions and characterized by the heterogeneity of clinical phenotype. [ABSTRACT FROM AUTHOR]

The human heart valves are complex anatomical structures consisting of leaflets with many supporting structures. With advancing age, the microstructure of the components of the valves can change. Knowledge and understanding of the anatomical relationships between the different components of the heart valve structures and their relationship with age is crucial for the development and progression of treatment of valvular disease. The purpose of this study was to determine histological changes of the components of the heart valves and their relationship with age. Fifty hearts taken from cadavers were included to examine the histology of the tricuspid, mitral, pulmonary, and aortic valves. All specimens were stained with Elastic Van Gieson, and picrosirius red to enable the evaluation of elastic and collagen fibers, respectively. There was a gradual increase in elastic and collagen fibers with advancing age, particularly over 40 years, in all valve types. In the case of tricuspid and mitral valves increases in collagen and elastic fibers were observed starting in the fifth decade. Elastic fiber fragmentation was observed in specimens over 50 years. In the case of the pulmonary and the aortic valves, collagen fibers were denser and more irregular in the sixth to seventh decades when compared to younger ages while elastic fibers were significantly increased in the sixth decade. In addition, an increase in fat deposition had an association with aging. These findings provide additional basic knowledge in age-related morphological changes of the heart valves and will increase understanding concerning valvular heart diseases and treatment options. [ABSTRACT FROM AUTHOR]

Pulmonary artery stenosis is a frequent complication after arterial switch operation. This case study presented a complex pulmonary artery stenosis initially treated with bilateral pulmonary artery stenting. The patient was found to have aortic valve endocarditis and recurrent pulmonary artery stenosis. The aortic valve was replaced and the pulmonary valve was repaired, followed by interposition of a Y-shaped graft from the sinotubular junction to both pulmonary arteries. The patient had an uneventful recovery and was discharged home asymptomatic after endocarditis antibacterial treatment. [ABSTRACT FROM AUTHOR]

Vascular and valvular calcification are commonly encountered in clinical medicine and a greater understanding of their significance and pathophysiology remain a subject of immense importance. In the coronary arteries, vascular calcification burden correlates with the severity of luminal stenosis and atherosclerotic plaque burden. While in progressive lesions, the presence of coronary calcification is not binary but rather depends on the type of calcification. Racial and gender differences, and comorbidities like diabetes mellitus and chronic kidney disease, all affect the presence and severity of calcification. The peripheral arteries of the lower extremities are affected by both medial calcification and intimal calcification, and the former barely contributes to luminal stenosis. The character of atherosclerosis differs between above-knee and below-knee lesions. Valvular calcification generally occurs on the aortic valve leaflets, and pathologic findings range from minimal fibrocalcific changes in early disease to end-stage lesions characterized by fibrotic thickening and nodular calcification. Valvular calcification is similar to atherosclerotic changes, in terms of lipid deposition, inflammation, osteogenic differentiation of valvular interstitial cells, and oxidative stress. However, the mechanisms of vascular and valvular calcification are still not well understood. A deeper understanding of vascular and valvular calcification is needed in order to develop effective anti-calcification therapies and to improve outcomes in these patients. [ABSTRACT FROM AUTHOR]

We describe successful placement of the Inspiris Resilia aortic valve in the pulmonary position. This valve has advantages for immediate benefit and future percutaneous interventions, making it a promising prosthesis for adult congenital patients. [ABSTRACT FROM AUTHOR]

Heart valve diseases are common disorders with five million annual diagnoses being made in the United States alone. All heart valve disorders alter cardiac hemodynamic performance; therefore, treatments aim to restore normal flow. This paper reviews the state-of-the-art clinical and engineering advancements in heart valve treatments with a focus on hemodynamics. We review engineering studies and clinical literature on the experience with devices for aortic valve treatment, as well as the latest advancements in mitral valve treatments and the pulmonic and tricuspid valves on the right side of the heart. Upcoming innovations will potentially revolutionize treatment of heart valve disorders. These advancements, and more gradual enhancements in the procedural techniques and imaging modalities, could improve the quality of life of patients suffering from valvular disease who currently cannot be treated. [ABSTRACT FROM AUTHOR]

Purpose. The aim of the work was to create an appropriate substrate for organ transplantation using bioactive tissue-based scaffold populated by cells of the graft recipient. The purpose of the modeling was to investigate the mechanical effects of wave loading of aortic and pulmonary tissue material. Methods. The biological properties of tissues of aortic and pulmonary valves were modified by the process of decellularization. The host cells were removed by various physical methods with focus on minimal degradation of the extracellular matrix. Thus, the decellularization process was controlled by histological methods. The tissue decellularization process was simulated by finite element modeling. Results. The mechanical results represented by displacement at the center of the sample were coherent and the heterogeneity of the distribution of the caves on the surface of the samples was confirmed, both by experiment and in the simulation by the alternate occurrence of local minima and maxima. The latter results from the uneven removal of cells from the effect of the wave causing decellularization were also predicted by the numerical model. Laser radiation had a destructive effect on the components of the extracellular matrix (e.g., collagen and elastic fibers), mainly depending on the fluence and number of pulses in a single exposure. Conclusions. The differences between the valve tissue materials were shown, and the impact of the process of decellularization on the properties of the tissues analyzed. It should be emphasized that due to low absorption and high scattering, laser radiation can deeply penetrate the tissue, which allows effective decellularization process in the entire volume of irradiated tissue. [ABSTRACT FROM AUTHOR]

Artificial heart valves, used to replace diseased human heart valves, are life-saving medical devices. Currently, at the device development stage, new artificial valves are primarily assessed through time-consuming and expensive benchtop tests or animal implantation studies. Computational stress analysis using the finite element (FE) method presents an attractive alternative to physical testing. However, FE computational analysis requires a complex process of numeric modeling and simulation, as well as in-depth engineering expertise. In this proof of concept study, our objective was to develop machine learning (ML) techniques that can estimate the stress and deformation of a transcatheter aortic valve (TAV) from a given set of TAV leaflet design parameters. Two deep neural networks were developed and compared: the autoencoder-based ML-models and the direct ML-models. The ML-models were evaluated through Monte Carlo cross validation. From the results, both proposed deep neural networks could accurately estimate the deformed geometry of the TAV leaflets and the associated stress distributions within a second, with the direct ML-models (ML-model-d) having slightly larger errors. In conclusion, although this is a proof-of-concept study, the proposed ML approaches have demonstrated great potential to serve as a fast and reliable tool for future TAV design. [ABSTRACT FROM AUTHOR]

Neural crest‐derived melanocytes have been recorded in several parts of the mammalian heart but not in the pulmonary valve. We report here the presence of melanin‐containing cells in the leaflets (cusps) of both the aortic and pulmonary valves. A total of 158 C57BL/6J x Balb/cByJ hybrid mice exhibiting four coat colours, namely black, white, agouti and non‐agouti brown, were examined. We sought for any relationship between the presence of melanocytes in the valves and the coat colour of the animals. The pigmentation levels of the leaflets were accomplished using a scale of five pigment intensities. White mice lacked pigment in the heart. In 10.5% of the remaining animals, there were melanocytes in the pulmonary valve leaflets. Thus, this is the first study to report the presence of such cells in the pulmonary valve of mammals. Melanocytes occurred in the leaflets of the aortic valves of 87.2% of mice. The incidence of melanocytes and the pigmentation level of the leaflets did not statistically differ according to the coat colours of the animals. This disagrees with previous observations, indicating that the amount of melanocytes in the heart reflects that of the skin. The incidence and distribution of melanocytes in aortic and pulmonary valves are consistent with the notion that the formation of the arterial valves is mediated by specific subpopulations of neural crest cells. We hypothesize that melanocytes, even not producing melanin, may be more frequent in the heart than previously thought, exerting presumably an immunological function. [ABSTRACT FROM AUTHOR]

Understanding cell geometric and mechanical properties is crucial to understanding how cells sense and respond to their local environment. Moreover, changes to cell mechanical properties under varied micro-environmental conditions can both influence and indicate fundamental changes to cell behavior. Atomic Force Microscopy (AFM) is a well established, powerful tool to capture geometric and mechanical properties of cells. We have previously demonstrated substantial functional and behavioral differences between aortic and pulmonary valve interstitial cells (VIC) using AFM and subsequent models of VIC mechanical response. In the present work, we extend these studies by demonstrating that to best interpret the spatially distributed AFM data, the use of spatial statistics is required. Spatial statistics includes formal techniques to analyze spatially distributed data, and has been used successfully in the analysis of geographic data. Thus, spatially mapped AFM studies of cell geometry and mechanics are analogous to more traditional forms of geospatial data. We are able to compare the spatial autocorrelation of stiffness in aortic and pulmonary valve interstitial cells, and more accurately capture cell geometry from height recordings. Specifically, we showed that pulmonary valve interstitial cells display higher levels of spatial autocorrelation of stiffness than aortic valve interstitial cells. This suggests that aortic VICs form different stress fiber structures than their pulmonary counterparts, in addition to being more highly expressed and stiffer on average. Thus, the addition of spatial statistics can contribute to our fundamental understanding of the differences between cell types. Moving forward, we anticipate that this work will be meaningful to enhance direct analysis of experimental data and for constructing high fidelity computational of VICs and other cell models. [ABSTRACT FROM AUTHOR]

The European Blood Alliance (EBA) Tissue and Cells annual benchmarking exercise identified that in 2014, the heart valve (HV) discard rate in tissue establishments (TEs) run by EBA members was between 19 and 65%. Given this significant discard rate, a decision was taken to carry out a worldwide data-gathering exercise to assess the processing methodology in different TEs. In collaboration with the Foundation of European Tissue Banks, a questionnaire asking for the details on HV processing was sent to TEs worldwide. Nineteen questionnaires were received back from 15 European TEs and 4 non-European TEs. The data provided confirmed a significant discard rate of HVs with 43–50% of aortic valves and 20–32% of pulmonary valves being discarded in 2015. The causes of HV discard varied, with microbiology contamination, anatomical and medical reasons being the main causes. This data-gathering exercise highlighted significant variations in practice in different TEs including how donor suitability is assessed, critical timings for heart retrieval and processing, heart rinsing, HV decontamination protocols and methods of microbiological testing. To reduce the discard rates, there are several aspects of HV banking that could be validated and standardised. Here, we report the findings of this data-gathering exercise. We consider this a first step that will help lead to standardising HV banking. [ABSTRACT FROM AUTHOR]

• Semilunar valve fenestrations are common in healthy hearts, but less common than reported in those with cardiovascular disease. • Size and prevalence of semilunar valve fenestrations have no demonstrable relationship with age or sex. • Fenestrations are slightly more common and larger in aortic valves when compared with pulmonary valves. Fenestrations in semilunar valves of human hearts have been incidentally described during autopsies since the 1800s, and were thought to be a degenerative process of the valve cusps. Due to the nature of autopsy, prior literature has primarily examined these fenestrations in pathologic hearts, and has implicated them in leading to valve insufficiency, regurgitation, and cusp rupture. More recent studies have predicted an increase in fenestration prevalence in the rapidly aging United States and have warned of a potential increase in fenestration-related valvular pathology. Herein, we analyze fenestration prevalence in 403 healthy human hearts and report findings that differ from these prior reports, and emphasize that fenestrations may not necessarily portend significant valvular dysfunction. [ABSTRACT FROM AUTHOR]

Simultaneous pulmonary and aortic endocarditis is extremely rare, and there is no consensus on its surgical management. Here, we report a case of infective endocarditis of pulmonary and aortic valves complicated by severe pulmonary regurgitation due to complete damage of valve cusps. We performed pulmonary valve reconstruction using autologous pericardium using Ozaki's technique, with excellent outcomes. [ABSTRACT FROM AUTHOR]

Aims: More than mild paravalvular aortic regurgitation (pAR) negatively impacts prognosis after transcatheter aortic valve implantation (TAVI). “Newer generation” transcatheter heart valves (THVs) including Direct Flow Medical, Medtronic Evolut R, Boston Lotus, and Edwards SAPIEN 3 valve system promise to improve outcome by reducing the rate of TAVI-related issues such as pAR. Aim was to evaluate and compare the hemodynamic performance with AR index of “early” vs. “newer generation” THVs and its impact on outcome. Methods and results: In 805 patients undergoing TAVI, the degree of pAR was assessed using imaging modalities (angiography, echocardiography) and hemodynamic measurements (aortic regurgitation index, ARI ratio). Severity of pAR and outcome were assessed according to the VARC-2 criteria. 805 patients underwent TAVI with use of the CoreValve (n = 400), SAPIEN XT (n = 48), Direct Flow Medical (n = 38), Evolut R (n = 114), Lotus (n = 104), or SAPIEN 3 (n = 101) prosthesis. TTE post TAVI revealed that a total of 7.3% of the patients showed moderate/severe pAR. The occurrence of greater than mild pAR occurred less frequently in patients treated with “newer generation” THVs (p<0.001): CoreValve (11.3%), SAPIEN XT (12.5%), Direct Flow Medical (5.3%), Evolut R (5.3%), Lotus (0.0%), and SAPIEN 3 (0.0%). The AR index was significantly higher (p<0.001) in patients receiving “newer generation” prostheses compared to those in whom “earlier generation” THVs were used. However, the ARI was only predictive of cumulative all-cause mortality at 1 and 3 years in “early generation”, but not in “newer generation” THVs. In the overall cohort, 30-day and 1-year mortality was 4.8% and 20.1%, respectively. In patients treated with “newer generation” devices, the respective mortality rates remained substantially below those of patients treated with “earlier generation” THVs (30-day mortality: 2.5% vs. 6.7%, p< 0.001; 1-year mortality: 11.2% vs. 27.2%, p<0.001). Conclusion: TAVI with use of “newer generation” THVs showed significantly reduced pAR and improved outcomes compared to “early generation” devices that could at least in part be explained by more favorable hemodynamics [ABSTRACT FROM AUTHOR]