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Purpose: Splenectomy might be a risk factor for valvular heart disease (VHD) in adult Hodgkin lymphoma survivors. As this risk is still unclear for childhood cancer survivors (CCS), the aim of this study is to evaluate the association between treatments affecting splenic function (splenectomy and radiotherapy involving the spleen) and VHD in CCS., Methods: CCS were enrolled from the DCCSS-LATER cohort, consisting of 6,165 five-year CCS diagnosed between 1963 and 2002. Symptomatic VHD, defined as symptoms combined with a diagnostic test indicating VHD, was assessed from questionnaires and validated using medical records. Differences in the cumulative incidence of VHD between CCS who received treatments affecting splenic function and CCS who did not were assessed using the Gray test. Risk factors were analyzed in a multivariable Cox proportional hazards model., Results: The study population consisted of 5,286 CCS, with a median follow-up of 22 years (5-50 years), of whom 59 (1.1%) had a splenectomy and 489 (9.2%) radiotherapy involving the spleen. VHD was present in 21 CCS (0.4%). The cumulative incidence of VHD at the age of 40 years was significantly higher in CCS who received treatments affecting splenic function (2.7%, 95% confidence interval (CI) 0.4%-4.9%) compared with CCS without (0.4%, 95% CI 0.1%-0.7%) (Gray's test, p = 0.003). Splenectomy was significantly associated with VHD in a multivariable analysis (hazard ratio 8.6, 95% CI 3.1-24.1)., Conclusions and Implications: Splenectomy was associated with VHD. Future research is needed to determine if CCS who had a splenectomy as part of cancer treatment might benefit from screening for VHD., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

A 28-year-old male presented with a sudden, transient visual impairment in the right eye. Brain MRI revealed no obvious ischemic lesions, and he was diagnosed with amaurosis fugax. Physical examination revealed characteristic features of Marfan syndrome, along with a family history of tall stature and valvular heart disease. Further investigation revealed: 1) an ascending aortic aneurysm, 2) a bicuspid aortic valve with fenestration and regurgitation, and 3) a mitral valve prolapse with regurgitation. Genetic testing identified a heterozygous variant c.6905G>A of FBN1, confirming the diagnosis of Marfan syndrome. Bentall operation and mitral valve replacement were performed, resulting in a favorable outcome. Pathological examination showed extensive myxomatous degeneration with thickening of the aortic and mitral valves, suggesting a potential source of embolism.

Purpose: Radiation therapy (RT) is an essential component in the treatment of many pediatric malignancies. Thoracic RT may expose the heart to radiation dose and thereby increase the risk of late cardiac disease. This comprehensive review from the Pediatric Normal Tissue Effects in the Clinic (PENTEC) initiative focused on late cardiac disease in survivors of childhood cancer treated with RT., Methods and Materials: This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology. We identified 1496 articles; 4 were included for dose-response modeling between mean cardiac radiation dose and risk of late coronary artery disease, heart failure (HF), valvular disease, and any cardiac disease., Results: For each 10-Gy increase in corrected mean cardiac radiation dose in 1.8- to 2.0-Gy fractions, we estimated a hazard ratio of 2.01 (95% confidence interval [CI], 1.79-2.25) for coronary artery disease, of 1.87 (95% CI, 1.70-2.06) for HF, of 1.87 (95% CI, 1.78-1.96) for valvular disease, and of 1.88 (95% CI, 1.75-2.03) for any cardiac disease. From the same model, for each 100-mg/m 2 increase in cumulative anthracycline dose, the hazard ratio for the development of HF was 1.93 (95% CI, 1.58-2.36), equivalent to an increase in mean heart dose of approximately 10.5 Gy. Other nontreatment factors were inconsistently reported in the analyzed articles., Conclusions: Radiation dose to the heart increases the risk of late cardiac disease, but survivors of childhood cancer who receive a mean dose <10 Gy at standard fractionation are at low absolute risk (<∼2% approximately 30 years after exposure) of late cardiac disease in the absence of anthracycline exposure. Minimizing cardiac radiation dose is especially relevant in children receiving anthracyclines. When cardiac sparing is not possible, we recommend prioritizing target coverage. It is likely that individual cardiac substructure doses will be a better predictor of specific cardiac diseases than mean dose, and we urge the pediatric oncology community to further study these relationships., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Cardiovascular disease is an important cause of death among patients with chronic kidney disease (CKD). Valve calcification is a predictor of cardiovascular mortality and coronary artery disease., Objective: To assess heart valve disease frequency, associated factors, and progression in CKD patients., Methods: We conducted a retrospective study on 291 CKD patients at Hospital das Clínicas de Pernambuco. Inclusion criteria were age ≥ 18 with CKD and valve disease, while those on conservative management or with missing data were excluded. Clinical and laboratory variables were compared, and patients were categorized by dialysis duration (<5 years; 5-10 years; >10 years). Statistical tests, including chi-square, Fisher's exact, ANOVA, and Kruskal-Wallis, were employed as needed. Simple and multivariate binary regression models were used to analyze valve disease associations with dialysis duration. Significance was defined as p < 0.05., Results: Mitral valve disease was present in 82.5% (240) of patients, followed by aortic valve disease (65.6%; 86). Over time, 106 (36.4%) patients developed valve disease. No significant association was found between aortic, pulmonary, mitral, or tricuspid valve disease and dialysis duration. Secondary hyperparathyroidism was the sole statistically significant factor for mitral valve disease in the regression model (OR 2.59 [95% CI: 1.09-6.18]; p = 0.031)., Conclusion: CKD patients on renal replacement therapy exhibit a high frequency of valve disease, particularly mitral and aortic valve disease. However, no link was established between dialysis duration and valve disease occurrence or progression.

Mediastinal radiotherapy for childhood cancers, particularly Hodgkin disease, has numerous potential adverse effects, including coronary artery disease, pericarditis, cardiomyopathy, valvular disease, and conduction abnormalities. The prevalence of valvular stenosis is relatively low, and regurgitation is more common. Mediastinal radiotherapy-induced valvular disease develops more than 10 years after radiotherapy. Here, we present a case of a 6-year-old boy with moderate to significant mitral stenosis + moderate mitral regurgitation and mild aortic regurgitation that appeared 1.5 months after radiotherapy and showed a progressive course., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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., (Copyright © 2023 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Cardiac valve calcification (CVC), characterized by the accumulation of calcium in the heart valves, is highly prevalent among patients undergoing dialysis. This meta-analysis aimed to provide an updated summary of recent studies on the prognostic value of CVC in patients undergoing dialysis. We conducted a search of PubMed, Embase, and Web of Science to identify observational studies investigating cardiovascular or all-cause mortality associated with CVC in dialysis patients until March 2023. Hazard ratios (HRs) and the corresponding 95% confidence intervals (CIs) were calculated for the meta-analysis, and the strength and significance of the associations between CVC and mortality outcomes in dialysis patients were assessed. From 6218 initially identified studies, we included 10 critical studies with a total of 3376 dialysis patients in a further meta-analysis. Pooled analyses demonstrated a significant association between CVC and an elevated risk of all-cause and cardiovascular mortality in dialysis patients. In our study, we discovered HRs of 1.592 (95% CI 1.410-1.797) for all-cause mortality and 2.444 (95% CI 1.632-3.659) for cardiovascular mortality. Furthermore, subgroup analysis revealed elevated all-cause mortality among patients with mitral valve calcification (HR 1.572; 95% CI 1.200-2.060) compared to those with aortic valve calcification (HR 1.456; 95% CI 1.105-1.917). Similarly, patients undergoing peritoneal dialysis faced a greater risk for all-cause mortality (HR 2.094; 95% CI 1.374-3.191) than those on hemodialysis (HR 1.553; 95% CI 1.369-1.763). This highlights the possibility of CVC being an independent risk factor for dialysis patients, particularly in relation to mitral valve calcification or peritoneal dialysis., (© 2024. The Author(s).)

BACKGROUND Candida prosthetic valve endocarditis is a rare disease that is increasing in incidence with the rising rates of fungemia and increased use of intracardiac devices. Chronic antifungal prophylaxis is used after primary treatment to prevent recurrence, but the optimal duration of prophylaxis is currently unknown. This case report is of a woman with a history of mitral valve replacement due to Candida endocarditis presenting 2 years later with prosthetic valve and native aortic valve Candida albicans endocarditis. CASE REPORT A 32-year-old woman with a history of intravenous drug abuse, Staphylococcus and Candida endocarditis, and 2 mitral valve replacements 2 years ago on long-term oral fluconazole presented with fevers, weight loss, and dyspnea. She had stopped taking her oral antifungals prior to presentation. She was found to have vegetations on her prosthetic mitral valve and on her native aortic valve. She was started on ceftriaxone, vancomycin, and micafungin, and blood cultures grew C. albicans. She also developed a C. albicans metatarsal abscess and a splenic infarct. She underwent redo mitral valve replacement and aortic valve debridement successfully and was continued on intravenous micafungin for 8 weeks. CONCLUSIONS This case highlights the association between prosthetic valve endocarditis, intravenous drug abuse, and opportunistic fungal infections. Lifelong oral fluconazole can be considered for all patients with C. albicans prosthetic valve endocarditis, especially in the setting of the presence of other risk factors, such as intravenous drug abuse, as demonstrated in our case. Further studies are needed to determine differences in outcomes.

Background: This study examined the characteristics and outcomes of surgical aortic valve replacement (SAVR) both isolated and in combination with other cardiac surgery in Malaysia from 2015 to 2021., Methods: This was a retrospective study of 1346 patients analyzed on the basis of medical records, echocardiograms and surgical reports. The overall sample was both considered as a whole and divided into aortic stenosis (AS)/aortic regurgitation (AR)-predominant and similar-severity subgroups., Results: The most common diagnosis was severe AS (34.6%), with the 3 most common etiologies being bicuspid valve degeneration (45.3%), trileaflet valve degeneration (36.3%) and rheumatic valve disease (12.2%). The second most common diagnosis was severe AR (25.5%), with the most common etiologies being root dilatation (21.0%), infective endocarditis (IE) (16.6%) and fused prolapse (12.2%). Rheumatic valve disease was the most common mixed disease. A total of 54.5% had AS-predominant pathology (3 most common etiologies: bicuspid valve degeneration valve, degenerative trileaflet valve and rheumatic valve disease), 36.9% had AR-predominant pathology (top etiologies: root dilatation, rheumatic valve disease and IE), and 8.6% had similar severity of AS and AR. Overall, 62.9% of patients had trileaflet valve morphology, 33.3% bicuspid, 0.6% unicuspid and 0.3% quadricuspid. For AS, the majority were high-gradient severe AS (49.9%), followed by normal-flow low-gradient (LG) severe AS (10.0%), paradoxical low-flow (LF)-LG severe AS (6.4%) and classical LF-LG severe AS (6.1%). The overall in-hospital and total 1-year mortality rates were 6.4% and 14.8%, respectively. Pure severe AS had the highest mortality. For AS-predominant pathology, the etiology with the highest mortality was trileaflet valve degeneration; for AR-predominant pathology, it was dissection. The overall survival probability at 5 years was 79.5% in all patients, 75.7% in the AS-predominant subgroup, 83.3% in the AR-predominant subgroup, and 87.3% in the similar-severity subgroup., Conclusions: The 3 most common causes of AS- predominant patients undergoing SAVR is bicuspid valve degeneration, degenerative trileaflet valve and rheumatic and for AR-predominant is root dilatation, rheumatic and IE. Rheumatic valve disease is an important etiology in our SAVR patients especially in mixed aortic valve disease. Study registration IJNREC/562/2022., (© 2023. The Author(s).)

Background: Chronic valvular heart disease is a well-known, long-term complication of acute rheumatic fever (ARF), which remains a major public health problem in low- and middle-income countries. Access to surgical management remains limited. Outcomes of the minority proportion of patients that access surgery have not been described in Uganda., Objectives: To describe the volume and type of rheumatic heart disease (RHD) valvular interventions and the outcomes of operated patients in the Uganda RHD registry., Methods: We performed a retrospective cohort study of all valve surgery procedures identified in the Uganda RHD registry through December 2021., Results: Three hundred and sixty-seven surgical procedures were performed among 359 patients. More than half were among young (55.9% were ≤20 years of age), female (59.9%) patients. All patients were censored at 15 years. The median (IQR) follow up period was 43 (22,79) months. Nearly half of surgeries (46.9%) included interventions on multiple valves, and most valvular operations were replacements with mechanical prostheses (96.6%). Over 70% of the procedures were sponsored by charity organizations. The overall mortality of patients who underwent surgery was 13% (47/359), with over half of the mortalities occurring within the first year following surgery (27/47; 57.4%). Fifteen-year survival or freedom from re-operation was not significantly different between those receiving valve replacements and those receiving valve repair (log-rank p = 0.76)., Conclusions: There has been increasing access to valve surgery among Ugandan patients with RHD. Post-operative survival is similar to regionally reported rates. The growing cohort of patients living with prosthetic valves necessitates national expansion and decentralization of post-operative care services. Major reliance on charity funding of surgery is unsustainable, thus calling for locally generated and controlled support mechanisms such as a national health insurance scheme. The central illustration (Figure 1) provides a summary of our findings and recommendations., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2023 The Author(s).)

Background: Transcatheter mitral valve replacement (TMVR) using the Tendyne™ valve is regarded as one of the most studied TMVR systems. The first human experience with the procedure was reported in 2013. The present study aims to systemically revise the published literature to document the global experience with TMVR using the Tendyne™ valve., Methods: The present review was conducted in line with the PRISMA statement on systematic reviews. Database included in the search process were Scopus, Web of Science and Pubmed. Search was processed using multiple keywords combinations and was adjusted to English literature only., Results: We included 26 articles in the final analysis reporting data from 319 patients. Patients recruited by the included studies comprised 192 males (60.2%) and 127 females (39.8%). In the studied patients, mitral annular calcification (MAC) was reported in 107 patients (33.5%). Preoperatively, MR grades 1,2 and 3-4 were reported in 3,5 and 307 patients respectively. Postoperatively, MR grades 1, 2 and 4 were reported in only 12, 3 and 1 patients respectively. Technical success was achieved in 309 patients (96.9%). Follow up durations widely varied among different studies from just days before discharge to 6 years. At the end of follow up, 79 patients died (24.8%) including 52 patients (16.3%) due to cardiovascular causes., Conclusions: Management of mitral valve disease using the Tendyne system appears to be a promising minimally invasive option for many high-risk patients with accepted procedural feasibility and safety profile., (© 2023. The Author(s).)

Purpose of Review: To synthesize and critically assess recent clinical and research advancements in pediatric bicuspid aortic valve (BAV) and its associated aortopathy., Recent Findings: In pediatric patients with BAV, progressive aortic dilation (i.e. bicuspid aortopathy) is commonly present and associated with increased risk for aortic aneurysm, dissection, and surgery in adulthood. Ongoing research explores the cause, incidence, and progression of bicuspid aortopathy to promote earlier diagnosis and improve preventive management. Recent findings include: high familial incidence and need for improved familial screening; safety of recreational physical activity in most affected children; potential for medical management to slow aortic growth; feasibility of pediatric registries to evaluate longitudinal outcomes; and potential genetic and hemodynamic biomarkers for disease risk stratification., Summary: Pediatric bicuspid aortopathy is an important area for investigation and preventive management to improve long-term cardiovascular outcomes. Recent literature promotes familial screening, recreational exercise, medical prophylaxis, registry-based longitudinal evaluation, and continued scientific inquiry., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Bicuspid aortic valve (BAV) is present in approximately 0.5%-2% of the general population, causing significant aortic stenosis (AS) in 12%-37% of affected individuals. Transcatheter aortic valve replacement (TAVR) is being considered the treatment of choice in patients with symptomatic AS across all risk spectra., Aim: Aim Our study aims to compare TAVR outcomes in patients with BAV versus tricuspid aortic valves (TAV)., Methods: A comprehensive literature search was performed in PubMed, Web of Science, and Cochrane trials. Studies were included if they included BAV and TAV patients undergoing TAVR with quantitative data available for at least one of our predefined outcomes. Meta-analysis was performed by the random-effects model using Stata software., Results: Fifty studies of 203,288 patients were included. BAV patients had increased 30-day all-cause mortality (odds ratio [OR] = 1.23 [1.00-1.50], p = 0.05), in-hospital stroke (OR = 1.39 [1.01-1.93], p = 0.05), in-hospital and 30-day PPI (OR = 1.13 [1.00-1.27], p = 0.04; OR = 1.16 [1.04-1.13], p = 0.01) and in-hospital, 30-day and 1-year aortic regurgitation (AR) (OR = 1.48 [1.19-1.83], p < 0.01; OR = 1.79 [1.26-2.52], p < 0.01; OR = 1.64 [1.03-2.60], p = 0.04). Subgroup analysis on new-generation valves showed a reduced 1-year all-cause mortality (OR = 0.86 [CI = 0.75-0.98], p = 0.03), despite higher in-hospital and 30-day PPI (OR = 0.1.21 [1.04-1.41], p = 0.01; OR = 1.17 [1.05-1.31], p = 0.01) and in-hospital AR (OR = 1.62 [1.14-2.31], p = 0.01) in the BAV group. The quality of included studies was moderate-to-high, and only three analyses presented high heterogeneity., Conclusion: TAVR is associated with comparable outcomes in patients with BAV and TAV. Careful selection of BAV cases by preprocedural assessment of valve anatomy and burden of calcification, pre- and post-procedural dilation, and implementing newer generations of valves may improve the safety and efficacy of TAVR in BAV patients., (© 2023 Wiley Periodicals LLC.)

Valvular heart disease is one of the common heart diseases in clinical practice, characterized by valve stenosis and/or incomplete closure. At present, drug therapy, surgery, and emerging percutaneous intervention therapy are the main treating methods for heart valve disease. Although the research and development of percutaneous intervention therapy devices is relatively mature, there are still problems such as postoperative mechanical hemolysis. The occurrence of mechanical hemolysis is associated with factors such as excessive shear stress experienced by red blood cells, direct interaction between red blood cells and the heart and valve surfaces, and thrombus formation. Furthermore, the presence of postoperative infection and other hemolytic diseases can also affect the occurrence of mechanical hemolysis. Although most patients are asymptomatic when hemolysis occurs, there are still critical cases. This type of hemolysis can accelerate the deterioration of the condition, and even endanger life in severe cases. Therefore, elucidating the background, pathogenesis, epidemiology, and related clinical research progress of mechanical hemolysis after percutaneous intervention therapy for valvular heart disease is of great significance for guiding the standardized diagnosis and treatment of the disease.

In patients receiving hemodialysis, infective endocarditis (IE) may present in a similar way to other causes of bacteremia, which may delay early diagnosis and can lead to worse outcomes. In this study, we aimed to identify the risk factors for IE in hemodialysis patients with bacteremia. This study was conducted on all patients diagnosed with IE and receiving hemodialysis between 2005 and 2018 in Salford Royal Hospital. Patients with IE were propensity score matched with similar hemodialysis patients with episodes of bacteremia between 2011 and 2015 (non-IE bacteremic (NIEB)). Logistic regression analysis was used to predict the risk factors associated with infective endocarditis. There were 35 cases of IE, and these were propensity matched with 70 NIEB cases. The median age of the patients was 65 years with a predominance of males (60%). The IE group had higher peak C-reactive protein compared to the NIEB group (median, 253 mg/L vs. 152, p = 0.001). Patients with IE had a longer duration of prior dialysis catheter use than NIEB patients (150 vs. 28.5 days: p = 0.004). IE patients had a much higher 30-day mortality rate (37.1% vs. 17.1%, p = 0.023). Logistic regression analysis showed previous valvular heart disease (OR: 29.7; p < 0.001), and a higher baseline C-reactive protein (OR: 1.01; p = 0.001) as significant predictors for infective endocarditis. Bacteremia in patients receiving hemodialysis through a catheter access should be actively investigated with a high index of suspicion for infective endocarditis, particularly in those with known valvular heart disease and a higher baseline C-reactive protein.

Background: The "UFO procedure" was initially developed as a surgical option to enlarge the aortic annulus in patients requiring valve replacement. This technique can be employed to treat extensive endocarditis located in the intervalvular fibrous body (IVFB). One of the indications for a "UFO procedure" is massive aortic and mitral valve calcification. It is a challenging surgical procedure with a high risk of intraoperative complications. We present a 76-year-old male patient with massive aortic and mitral valve calcification involving the left atrium, the left ventricle and the left ventricular outflow tract. Both valves exhibited severe stenosis and moderate to severe regurgitation. The left ventricle was hypertrophic and the left ventricular ejection fraction was > 55%. The patient was prediagnosed with persistent atrial fibrillation. The risk of death following heart surgery (EuroSCORE II) was calculated as 9.21%. We successfully performed a so-called "UFO procedure" including replacement of both valves without annular decalcification to avoid atrioventricular dehiscence. We enlarged the IVFB and replaced the non-coronary sinus of Valsalva with doubled bovine pericardium. The left ventricular outflow tract was decalcified. The patient was transferred to a local hospital on the 13th postoperative day., Conclusion: Successful surgical treatment to this extent was demonstrated for the first time. Due to the high perioperative mortality, the surgical treatment of patients with this constellation would be refused in most cases. In our patient, the preoperative imaging showed extreme calcification of both valves and the surrounding myocardium. Excellent preoperative planning and a highly experienced surgical team is necessary., (© 2023. The Author(s).)

Objectives: This study seeks to estimate the potential societal economic impact of treating patients with heart valve disease (HVD) in China with surgical tissue valve replacement versus mechanical valves., Methods: This societal economic cost-benefit evaluation is based on an individual simulation model for subgroups of patients with HVD that is also aggregated to a macrosocietal model. The individual simulation model was developed to estimate the likely economic impact of surgical aortic valve replacement (SAVR) with tissue versus mechanical valves for different subgroups among all eligible patients with HVD over their remaining lifetimes. Clinical inputs were informed by health claims database analysis, expert clinical opinion, and published literature. Epidemiological inputs and demographic inputs were sourced from the published literature and the China Statistical Yearbook 2020. Health gains were valued at 3 times the average national income., Results: Projected total lifetime economic gains were greater for patients receiving tissue valves. Costs were reported in 2021 US dollars. The average lifetime net economic gain for tissue valve patients was $51 736 (20.0% more than for mechanical valve patients). Increasing the use of tissue valves to 50% among all eligible patients with HVD would provide aggregate long-term economic gains of $167 billion during their remaining lifetimes. The economic gains from greater tissue valve use were due to avoiding anticoagulation monitoring costs, improved quality of life, and greater post-SAVR labor force participation., Conclusion: Increased use of tissue valves versus mechanical values in SAVR procedures in China would be likely to generate a substantial societal economic gain., (Copyright © 2023 International Society for Health Economics and Outcomes Research. Published by Elsevier Inc. All rights reserved.)

Background and Purpose: Valvular Heart Disease (VHD) is a known complication of childhood cancer after radiotherapy treatment. However, the dose-volume-effect relationships have not been fully explored., Materials and Methods: We obtained individual heart Dose Volume Histograms (DVH) for survivors of the French Childhood Cancer Survivors Study (FCCSS) who had received radiotherapy. We calculated the Mean Dose to the Heart (MHD) in Gy, as well as the heart DVH parameters (V d Gy , which represents the percentage of heart volume receiving at least d Gy), fixing the thresholds to 0.1 Gy, 5 Gy, 20 Gy, and 40 Gy. We analyzed them furtherly in the subpopulation of the cohort that was treated with a dose lower than 5 Gy (V 0.1Gy | V5Gy=0% ), 20 Gy (V 5Gy | V20Gy=0% ), and 40 Gy (V 20Gy | V40Gy=0% ), respectively. We investigated their role in the occurrence of a VHD in this population-based observational cohort study using the Cox proportional hazard model, adjusting for age at cancer diagnosis and chemotherapy exposure., Results: Median follow-up was 30.6 years. Eighty-one patients out of the 7462 (1 %) with complete data experienced a severe VHD (grade ≥ 3). The risk of VHD increased along with the MHD, and it was associated with high doses to the heart (V 40Gy  < 50 %, hazard ratio (HR) = 7.96, 95 % CI: 4.26-14.88 and V 20Gy | V40Gy=0% >50 %, HR = 5.03, 95 % CI: [2.35-10.76]). Doses 5-20 Gy to more than 50 % (V 5Gy | V20Gy=0% >50 %) of the heart induced a marginally non-significant estimated risk. We also observed a remarkable risk increase with attained age., Conclusions: Our results provide new insight into the VHD risk that may impact current treatments and long-term follow-up of childhood cancer survivors., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Valvular heart disease is becoming an increasingly prevalent with population ageing. We sought to define the current prevalence of valvular heart disease in Australia., Methods: The TasELF and VicELF studies prospectively recruited 962 asymptomatic participants ≥65 years, with at least one cardiovascular risk factor, from the Tasmanian and Victorian communities. People were excluded if they had a previous diagnosis of heart failure, or a life expectancy <1 year. All underwent baseline echocardiography. Those with moderate or severe valvular disease were identified. The current prevalence of clinically significant valve disease was applied to the Australian Bureau of Statistics population projections., Results: Echocardiograms were interpretable in 943 participants (98%). Clinically significant valve disease was present in 5% of the population, and mitral regurgitation was the most common overall valvular lesion, present in 36% of the population. The projected numbers of people with clinically significant valvular disease is expected to increase significantly across all age groups by the year 2060., Conclusions: Clinically significant yet asymptomatic valvular disease was prevalent in a large community cohort of participants with at least one risk factor. The total burden of valvular heart disease is expected to increase dramatically over the coming decades., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Indications for transcatheter aortic valve implantation (TAVI) have expanded in many countries to include patients with aortic stenosis who are at low surgical risk, and a similar expansion to this cohort is anticipated elsewhere in the world, together with an increase in the proportion of patients with bicuspid aortic valve (BAV) morphology as the age of the patients being treated decreases. To date, patients with BAV have been excluded from major randomized trials of TAVI owing to anatomical considerations. As a consequence, BAV has been a relative contraindication to the use of TAVI in international guidelines. Although clinical experience and observational data are accumulating, BAV presents numerous anatomical challenges for successful TAVI, despite advances in device design. Furthermore, in those with BAV, substantial geographical variation exists in patient characteristics, clinical approach and procedural strategy. Therefore, in this Roadmap article, we summarize the existing evidence and provide consensus recommendations from an international group of experts on the application of TAVI in patients with BAV in advance of the anticipated growth in the use of this procedure in this challenging cohort of patients., (© 2022. Springer Nature Limited.)

Background: Cardiovascular calcification includes cardiac valve calcification (CVC) and vascular calcification. We aimed to analyze risk factors for CVC, and construct a predictive model in maintenance peritoneal dialysis (MPD) patients., Methods: We retrospectively analyzed MPD patients who began peritoneal dialysis between January 2014 and September 2021. Patients were randomly assigned to the derivation cohort and validation cohort in a 7:3 ratio. The patients in the derivation cohort were divided into the CVC group and non-CVC group. Logistic regression was used to analyze risk factors, then the rms package in R language was used to construct a nomogram model to predict CVC., Results: 1,035 MPD patients were included, with the age of 50.0 ± 14.2 years and 632 males (61.1%). Their median follow-up time was 25 (12, 46) months. The new-onset CVC occurred in 128 patients (12.4%). In the derivation cohort, multivariate logistic regression indicated old age, female, high systolic blood pressure (SBP), high calcium-phosphorus product (Ca × P), high Charlson comorbidity index (CCI) and long dialysis time were independent risk factors for CVC ( p  < 0.05). We constructed a nomogram model for predicting CVC in the derivation cohort, with a C index of 0.845 (95% CI 0.803-0.886). This model was validated with a C index of 0.845 (95%CI 0.781-0.909) in the validation cohort., Conclusion: We constructed a nomogram model for CVC in MPD patients, using independent risk factors including age, sex, SBP, Ca × P, CCI and dialysis time. This model achieved high efficiency in CVC prediction.

Background: Cardiac valve calcification (CVC) is an important risk factor for cardiovascular complications. However, limited data are available concerning the prevalence, clinical features and risk factors for CVC in end-stage kidney disease (ESKD) patients. In this study, we aimed to assess these parameters in Chinese ESKD patients receiving combination therapy with hemodialysis and hemodiafiltration., Methods: We conducted a cross-sectional study on 293 ESKD patients undergoing combination therapy of hemodialysis and hemodiafiltration at the First Affiliated Hospital of Chongqing Medical University from October 2014 to December 2015. CVC was evaluated via echocardiography., Results: ESKD patients with CVC had a higher prevalence of diabetes mellitus, aortic and/or coronary artery calcification, arrhythmia, heart failure and coronary heart disease; increased systolic, diastolic and pulse pressure; longer duration of hemodialysis and hypertension; reduced hemoglobin, albumin and high-density lipoprotein cholesterol levels; and increased serum calcium and calcium-phosphorus product levels compared with those without CVC. Logistic regression analysis showed that increased dialysis duration ( p  = 0.006, OR = 2.25), serum calcium levels ( p  = 0.046, OR = 2.04) and pulse pressure ( p  < 0.001, OR = 3.22), the presence of diabetes ( p  = 0.037, OR = 1.81) and decreased serum albumin levels ( p  = 0.047, OR = 0.54) were risk factors for CVC. The correlation analysis indicated a significantly increased CVCs prevalence with an increase prevalence of heart failure, aortic and coronary artery calcification., Conclusions: CVC represents a common complication and a danger signal for cardiovascular events in ESKD patients undergoing combination therapy of hemodialysis and hemodiafiltration. The presence of diabetes, increased pulse pressure, long dialysis duration, hypoalbuminemia and high serum calcium levels were independent risk factors for CVC.

Background: Transcatheter aortic valve implantation (TAVI) has increasingly been utilized in patients with bicuspid aortic valve (BAV) related aortic stenosis (AS) with insufficient large-scale data on its safety., Methods: The Nationwide Inpatient Sample and Nationwide Readmission Database (2011-2018) were queried to identify patients undergoing TAVI for BAV versus trileaflet aortic valve (TAV) associated AS. The in-hospital, 30- and 180-day odds of outcomes were assessed using a propensity-matched analysis (PSM) to calculate adjusted odds ratios (aOR) with its 95% confidence interval (CI)., Results: A total of 216,723 TAVI (TAV: 214,050 and BAV: 2,673) crude and 5,347 matched population (TAV: 2,674 and BAV: 2,673) was included in the final analysis. At index admission, the adjusted odds of in-hospital mortality (aOR: 1.57, 95% CI: 0.67-3.66), stroke (aOR: 0.77, 95% CI: 0.38-1.57), cardiac tamponade (aOR: 0.75, 95% CI: 0.17-3.36), vascular complications (aOR: 0.33, 95% CI: 0.09-1.22), cardiogenic shock (aOR: 1.77, 95% CI: 0.93-3.38), paravalvular leak (aOR: 0.55, 95% CI: 0.26-1.14), need for mechanical circulatory support device, and permanent pacemaker implantation (PPM) (aOR: 1.02, 95% CI: 0.69-1.52) were not significantly different between TAVI for BAV versus TAV. At 30- and 180-day follow-up duration, the risk of stroke and major postprocedural complications remained similar, except that TAVI in BAV had a higher incidence of PPM implantation compared with TAV. The yearly trend showed an increase in the utilization of TAVI for both TAV and BAV and a steady decline in the overall annual rate of in-hospital complications., Conclusion: TAVI utilization in patients with BAV has increased over the recent years. The relative odds of in-hospital mortality, and all other major complications, were similar between patients undergoing TAVI for BAV- and TAV-related AS., (© 2022 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Introduction: The totally thoracoscopic approach for mitral valve (MV) disease is a minimally invasive method. We investigated the procedure's feasibility, safety and effectiveness when it was performed by an experienced operator., Methods: We retrospectively analysed 96 consecutive patients with MV disease treated between March 2016 and November 2019 by minimally invasive procedures. The procedures were performed on a femoral artery-vein bypass through two ports, including a main operation port and a thoracoscopic port. The clinical data of patients were collected, including preoperative cardiac function, operative data, postoperative complications, and follow-up., Results: A total of 96 patients (57 male patients; average age, 49.7±14.5 years; left ventricular ejection fraction, 65.6±7.7%) were enrolled in this study. No intraoperative conversion incision or death occurred. The cardiopulmonary bypass and aortic cross-clamp times were 163.8±50.6 minutes and 119.7±38.9 minutes, respectively. Postoperative chest tube drainage in the first 24 hours was 232.8±108.1 ml. The ventilation time and length of intensive care unit stay were 13.2±6.2 hours and 2.9±2.2 days, respectively. One patient died of disseminated intravascular coagulation and prosthesis thrombosis 3 days after the operation, fearing anticoagulant-related hemorrhage. The overall success rate of valve repair during 1-year follow-up was 97.9%., Conclusion: The totally thoracoscopic procedure on mitral valves by an experienced surgeon is technically feasible, safe, effective and worthy of widespread adoption in clinical practice.

Purpose of Review: This review provides the summary of the appropriate use of these modalities when caring for patients with Kawasaki disease at diagnosis and for long-term management., Recent Findings: Kawasaki disease is an inflammatory syndrome of unknown etiology that can result in coronary artery dilations or aneurysms if left untreated in 25% of the patients and 3-5% in treated patients. In addition to coronary artery aneurysms, patients can have ventricular dysfunction, valvular regurgitation, aortic root dilation, and pericardial effusion due to inflammation of the myocardium. Noninvasive imaging modalities are important to these assessments. Echocardiography is the first-line noninvasive evaluation of coronaries and function. Cardiac magnetic resonance imaging is useful for functional assessment in long-term follow-up. Distal coronaries, thromboses, and stenoses are best evaluated by cardiac computed tomography. Future research should demonstrate the effectiveness of advanced functional imaging in patients with Kawasaki disease and decreased radiation from cardiac computed tomography., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

A calcified amorphous tumor( CAT) is a non-neoplastic cardiac tumor, which has been reported to be associated with hemodialysis dependent end-stage renal disease. We report a case of CAT attached to mitral annular calcification (MAC) in the posterior leaflet annulus of the mitral valve in a 56-year-old man who had been receiving peritoneal dialysis for three years. The CAT grew to 10 mm long in a half year. Peritoneal dialysis dependent end-stage renal disease is associated with MAC. Additionally, MAC-related CAT has been reported growing rapidly. We should perform periodic echocardiography not only for hemodialysis patients but also for peritoneal dialysis patients. When CAT is diagnosed, operation should be performed early to prevent embolism such as cerebral infarction.

Introduction: Hodgkin lymphoma (HL) is a highly curable hematological malignancy. Consolidation radiation therapy techniques have made significant progresses to improve organ-at-risk sparing in order to reduce late radiation-induced toxicity. Recent technical breakthroughs notably include intensity modulated proton therapy (IMPT), which has demonstrated a major dosimetric benefit at the cardiac level for mediastinal HL patients. However, its implementation in clinical practice is still challenging, notably due to the limited access to proton therapy facilities. In this context, the purpose of this study was to estimate the benefit of IMPT for HL proton therapy for diverse cardiac adverse events and to propose a general frame for mediastinal HL patient selection strategy for IMPT based on cardiotoxicity reduction, patient clinical factors, and IMPT treatment availability., Material and Methods: This retrospective dosimetric study included 30 mediastinal HL patients treated with VMAT. IMPT plans were generated on the initial simulation scans. Dose to the heart, to the left ventricle and to the valves were retrieved to calculate the relative risk (RR) of ischemic heart disease (IHD), congestive heart failure (CHF) and valvular disease (VD). Composite relative risk reduction (cRRR) of late cardiotoxicity, between VMAT and IMPT, were calculated as the weighted mean of relative risk reduction for IHD, CHF and VD, calculated across a wide range of cardiovascular risk factor combinations. The proportion of mediastinal HL patients who could benefit from IMPT was estimated in European countries, based on the country population and on the number of active gantries, to propose country-specific cRRR thresholds for patient selection., Results: Compared with VMAT, IMPT significantly reduced average mean doses to the heart (2.36 Gy vs 0.99 Gy, p  < 0.01), to the left ventricle (0.67 Gy vs 0.03, p  < 0.01) and to the valves (1.29 Gy vs. 0.06, p  < 0.01). For a HL patient without cardiovascular risk factor other than anthracycline-based chemotherapy, the relative risks of late cardiovascular complications were significantly lower after IMPT compared with VMAT for ischemic heart disease (1.07 vs 1.17, p  < 0.01), for congestive heart failure (2.84 vs. 3.00, p  < 0.01), and for valvular disease (1.01 vs. 1.06, p  < 0.01). The median cRRR of cardiovascular adverse events with IMPT was 4.8%, ranging between 0.1% and 30.5%, depending on the extent of radiation fields and on the considered cardiovascular risk factors. The estimated proportion of HL patients currently treatable with IMPT in European countries with proton therapy facilities ranged between 8.0% and 100% depending on the country, corresponding to cRRR thresholds ranging from 24.0% to 0.0%., Conclusion: While a statistically significant clinical benefit is theoretically expected for ischemic heart disease, cardiac heart failure and valvular disease for mediastinal HL patients with IMPT, the overall cardiotoxicity risk reduction is notable only for a minority of patients. In the context of limited IMPT availability, this study proposed a general model-based selection approach for mediastinal HL patient based on calculated cardiotoxicity reduction, taking into consideration patient clinical characteristics and IMPT facility availability.

Acquired diseases of the aortic and mitral valves are the most common cause of morbidity and mortality among Valvular heart diseases. Aortic stenosis (AS) is increasing in incidence in the United States (4,43 US), driven largely by an aging demographic. Aortic valve replacement is the only effective treatment of AS and has a dramatic mortality benefit. Mitral valve regurgitation (MR) is the most common form of valvular heart disease (VHD) in the US, whereby MR is most often the result of mitral valve prolapse; rheumatic heart disease (RHD) is a more common etiology of MR in underdeveloped countries. interventions for MR in the US are increasing., (Published by Elsevier Inc.)

Aims: Transcatheter mitral valve implantation (TMVI) is a new treatment option for patients with symptomatic mitral valve (MV) disease. Real-world data have not yet been reported. This study aimed to assess procedural and 30-day outcomes of TMVI in a real-world patient cohort., Method and Results: All consecutive patients undergoing implantation of a transapically delivered self-expanding valve at 26 European centres from January 2020 to April 2021 were included in this retrospective observational registry. Among 108 surgical high-risk patients included (43% female, mean age 75 ± 7 years, mean STS-PROM 7.2 ± 5.3%), 25% was treated for an off-label indication (e.g. previous MV intervention or surgery, mitral stenosis, mitral annular calcification). Patients were highly symptomatic (New York Heart Association [NYHA] functional class III/IV in 86%) and mitral regurgitation (MR) was graded 3+/4+ in 95% (38% primary, 37% secondary, and 25% mixed aetiology). Technical success rate was 96%, and MR reduction to ≤1+ was achieved in all patients with successful implantation. There were two procedural deaths and 30-day all-cause mortality was 12%. At early clinical follow-up, MR reduction was sustained and there were significant reductions of pulmonary pressure (systolic pulmonary artery pressure 52 vs. 42 mmHg, p < 0.001), and tricuspid regurgitation severity (p = 0.013). Heart failure symptoms improved significantly (73% in NYHA class I/II, p < 0.001). Procedural success rate according to MVARC criteria was 80% and was not different in patients treated for an off-label indication (74% vs. 81% for off- vs. on-label, p = 0.41)., Conclusion: In a real-world patient population, TMVI has a high technical and procedural success rate with efficient and durable MR reduction and symptomatic improvement., (© 2022 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Severe cardiac failure from mechanical mitral valve thrombosis due to poor warfarin control is a major cause of emergency redo mitral valve replacement (MVR) in South Africa. This study aimed to review the outcomes of redo MVR in patients presenting with mitral valve failure to a tertiary South African centre., Methods: Retrospective chart review of patients undergoing redo MVR over a 10-year period (2005-2014). Patient demographics, aetiology of valve dysfunction, preoperative clinical assessment and outcomes were analysed., Results: Sixty-four patients had 80 mitral valve procedures. The M:F ratio was 1:2.8 and the median age was 18 (IQR 14-28.5) and 25 (IQR 18-40) at initial surgery and at redo surgery, respectively. Median interval between original and redo MVRs was 47.5 (IQR 7.5-124) months. Rheumatic valve disease was the original pathology in 58 patients (90.6%). Fifty-two patients underwent a single redo MVR and 12 patients had multiple redo MVRs. Fifty-eight (72.5%) were emergency redo procedures. Prosthetic valve thrombosis was present in 73.8%. Ten patients (15.6%) developed postoperative complications. The median hospital stay and ICU stay were 19 (IQR 12-27.5) days and 4 (IQR 3-7) days, respectively. Two patients died in the postoperative period (3.1%). The mean patient follow-up was 42 months. Three patients died during follow-up., Conclusion: The majority of redo MVR procedures were undertaken as an emergency with valve thrombosis being the most common aetiology. The mortality rate was 3.1% and postoperative complication rate was 15.6%., (Copyright© Authors.)

Background: Rheumatic heart valve disease (RHVD) is a leading cause of cardiovascular death in low- and middle-income countries and affects predominantly women. The underlying mechanisms of chronic valvular damage remain unexplored and regulators of sex predisposition are unknown., Methods: Proteomics analysis of human heart valves (nondiseased aortic valves, nondiseased mitral valves [NDMVs], valves from patients with rheumatic aortic valve disease, and valves from patients with rheumatic mitral valve disease; n=30) followed by system biology analysis identified ProTα (prothymosin alpha) as a protein associated with RHVD. Histology, multiparameter flow cytometry, and enzyme-linked immunosorbent assay confirmed the expression of ProTα. In vitro experiments using peripheral mononuclear cells and valvular interstitial cells were performed using multiparameter flow cytometry and quantitative polymerase chain reaction. In silico analysis of the RHVD and Streptococcus pyogenes proteomes were used to identify mimic epitopes., Results: A comparison of NDMV and nondiseased aortic valve proteomes established the baseline differences between nondiseased aortic and mitral valves. Thirteen unique proteins were enriched in NDMVs. Comparison of NDMVs versus valves from patients with rheumatic mitral valve disease and nondiseased aortic valves versus valves from patients with rheumatic aortic valve disease identified 213 proteins enriched in rheumatic valves. The expression of the 13 NDMV-enriched proteins was evaluated across the 213 proteins enriched in diseased valves, resulting in the discovery of ProTα common to valves from patients with rheumatic mitral valve disease and valves from patients with rheumatic aortic valve disease. ProTα plasma levels were significantly higher in patients with RHVD than in healthy individuals. Immunoreactive ProTα colocalized with CD8 + T cells in RHVD. Expression of ProTα and estrogen receptor alpha correlated strongly in circulating CD8 + T cells from patients with RHVD. Recombinant ProTα induced expression of the lytic proteins perforin and granzyme B by CD8 + T cells as well as higher estrogen receptor alpha expression. In addition, recombinant ProTα increased human leukocyte antigen class I levels in valvular interstitial cells. Treatment of CD8 + T cells with specific estrogen receptor alpha antagonist reduced the cytotoxic potential promoted by ProTα. In silico analysis of RHVD and S pyogenes proteomes revealed molecular mimicry between human type 1 collagen epitope and bacterial collagen-like protein, which induced CD8 + T-cell activation in vitro., Conclusions: ProTα-dependent CD8 + T-cell cytotoxicity was associated with estrogen receptor alpha activity, implicating ProTα as a potential regulator of sex predisposition in RHVD. ProTα facilitated recognition of type 1 collagen mimic epitopes by CD8 + T cells, suggesting mechanisms provoking autoimmunity.

Purpose: Studies on cardiac structural and functional abnormalities in primary hyperparathyroidism (PHPT) have yielded conflicting and inconsistent results. In this prospective case-control study, we sought to compare cardiac structure and function in symptomatic PHPT patients and controls., Methods: One hundred consecutive symptomatic PHPT patients and 113 matched controls underwent echocardiographic evaluation by the same operator., Results: Left ventricular mass index (LVMI) was significantly higher in patients as compared to controls, (median of 90.95 g/m 2 vs 86.5 g/m 2 , p = 0.041). Patients had significantly lower early trans-mitral diastolic flow (E velocity) as compared to controls (57.13 ± 14.88 vs 64.76 ± 15.45 cm/s, p < 0.001). Patients also had significantly lower early to late mitral annular velocity (E/A) as compared to controls (0.98 ± 0.37 vs 1.10 ± 0.34, p 0.013). Patients had higher frequency of aortic valve calcification (29% vs 2.65%, p < 0.001), mitral annular calcification (23% vs. 4.42%, p < 0.001), myocardial and septal calcifications (25% vs none, p < 0.001) as compared to controls. Serum PTH, calcium and uric acid significantly correlated with calcifications. Serum calcium showed a negative correlation with E/A ratio., Conclusions: Symptomatic patients with PHPT have substantial cardiac structural and functional abnormalities. These abnormalities include elevated LVMI, diastolic dysfunction, and aortic valve, mitral annular, septal and myocardial calcifications. We strongly suggest and conclude that the evaluation of PHPT patients should not only include traditional end organs like bones and kidneys but also the cardiovascular system in the form of echocardiography to detect subclinical cardiac dysfunction so that the cardiovascular health of such patients can be optimized., (© 2021. Italian Society of Endocrinology (SIE).)

Background: Systemic sclerosis (SSc) is a chronic autoimmune disease that is characterized by vasculopathy and fibrosis of the skin and visceral organs. Heart valve diseases are poorly described and generally not considered typical of SSc. We aimed to describe valvular abnormalities in a multicenter cohort of SSc patients and to investigate their correlation with SSc features., Methods: We recruited 118 consecutive SSc patients (male/female, 14/104; mean age, 55.2 ± 12.1 years) in 3 rheumatology centers in Sicily, Italy, from January to October 2019., Results: Mitral and tricuspid valve insufficiency was found in 85% and 91% of patients, respectively; regurgitations were generally mild and never severe. Mitral stenosis was rare (2%), and tricuspid stenosis was not observed. Sclerosis and calcification were present in 30% of mitral valves and in only 4% of tricuspid valves. The aortic valve was affected in 25% of cases, and it generally presented as regurgitation or sclerosis, whereas stenosis was rare (3%). Finally, 11% of SSc patients showed regurgitation of the pulmonary valve. No specific associations between SSc features and valve alterations were found., Conclusions: Valvular diseases are frequently observed in SSc patients, with a predominant pattern of valvular regurgitations. Therefore, echocardiography should be routinely performed during SSc patient follow-up, considering the potential influence of additional cardiac involvement in the prognosis of these patients., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Mitral valve diseases are relatively rare in the paediatric population; however, they can cause considerable mortality and morbidity worldwide. Acquired causes are a major contributor to cardiovascular disease burden in the paediatric population. Diseases can be detected before birth, at birth, or when the child is older and presents with symptoms of advanced heart failure. Definitive management consists of surgical intervention, with mitral valve replacement being the gold standard.Conclusion: Repair has been gaining popularity; however, its outcomes require further study. Percutaneous mitral balloon valvuloplasty is an emerging technique which holds promise as a bridge to surgical treatment. The effect of these interventions on quality of life must be emphasised and requires further study. What is Known: • The epidemiology of mitral valve disease in the paediatric population - predominant causes include rheumatic disease and congenital defects.  • Mitral valve repair and replacement are the standard treatment methods for paediatric mitral valve disease.  What is New: • Emergence of percutaneous mitral valve interventions and their potential as bridge-to-surgery or definitive treatment in high-risk surgical candidates.  • Recent evidence comparing mitral valve repair and replacement in the paediatric population demonstrates increasing popularity of repair techniques., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

In the recent years, there has been significant transformation in the management of valvular heart disease (VHD), as a result of new minimally invasive technologies, such as the transcatheter aortic valve implantation (TAVI). Conventionally, mechanical heart valves require anticoagulation with warfarin to prevent thrombogenic events. Lately, there has been an uptrend in the usage of direct-acting oral anticoagulants (DOACs) in both mechanical and bioprosthetic heart valves. In clinical practice, there has shown to be notable heterogeneity in the antithrombotic regimen for patients. Recommendations from clinical guidelines and emerging data on DOAC use in these settings will be critically reviewed here. Future large, randomized-controlled trials are warranted to delineate the role of DOACs in patients receiving a bioprosthetic valve/TAVI or mechanical heart valve, with and without a baseline indication for anticoagulation or antiplatelets. Until clinical trial data from well-designed studies are available, providers must remain vigilant about DOAC use in patients with VHD, especially in patients with a bioprosthetic or mechanical heart valve.

Background: Carcinoid heart disease (CHD) is a rare complication of hormonally active neuroendocrine tumors that often requires surgical intervention. Data on cardiac implantable electronic device (CIED) implantation in patients with CHD are limited., Objective: The purpose of this study was to evaluate the experience of CIED implantation in patients with CHD., Methods: Patients with a diagnosis of CHD and a CIED procedure from January 1, 1995, through June 1, 2020, were identified using a Mayo Clinic proprietary data retrieval tool. Retrospective review was performed to extract relevant data, which included indications for implant, procedural details, complications, and mortality., Results: A total of 27 patients (55.6% male; mean age at device implant 65.6 ± 8.8 years) with cumulative follow-up of 75 patient-years (median 1.1 years; interquartile range 0.4-4.6 years) were included for analysis. The majority of implanted devices were dual-chamber permanent pacemakers (63%). Among all CHD patients who underwent any cardiac surgery, the incidence of CIED implantation was 12%. The most common indication for implantation was high-grade heart block (66.7%). Device implant complication rates were modest (14.8%). No patient suffered carcinoid crisis during implantation, and there was no periimplant mortality. Median time from implant to death was 2.5 years, with 1-year mortality of 15%., Conclusion: CHD is a morbid condition, and surgical valve intervention carries associated risks, particularly a high requirement for postoperative pacing needs. Our data suggest that CIED implantation can be performed relatively safely. Clinicians must be aware of the relevant carcinoid physiology and take appropriate precautions to mitigate risks., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Purpose: Acromegaly is a rare disease and is associated with increased cardiovascular (CV) morbidity and mortality, especially in patients with uncontrolled disease. We aimed to analyze the prevalence and severity of cardiomyopathy and valvular heart disease in a large cohort of patients with a confirmed acromegaly diagnosis, at baseline and after treatment., Methods: We retrospectively reviewed an institutional approved database; 190 patients with confirmed acromegaly and follow-up data available (years 2006-2018). Patients with at least one baseline echocardiogram, were included. Demographic, disease control and echocardiogram variables were collected for analysis., Results: Of the 190 patients 110 (58%) had a baseline echocardiogram and 43 (39.1%) had at least one follow-up echocardiogram after surgical, medical or multimodal treatment. Baseline left ventricular hypertrophy (LVH) prevalence was 17.8% (64.7% concentric; 35.3% eccentric), diastolic and systolic dysfunction, and overt cardiomyopathy with heart failure were 15.8, 7.9, and 3.0%, respectively. Concentric remodeling of the left ventricle (LV) was noted in 31.4% of patients without LVH. Valve defects were found in 87.3% of patients (14.6% with significant valvular heart disease)., Conclusion: Early diagnosis of acromegaly and disease control should be attempted to prevent LVH/LV dysfunction and development of valvular heart disease. Concentric LV remodeling develops prior to obvious LV hypertrophy in almost a third of patients with acromegaly, which is a novel finding. Similar to other epidemiological studies, we found a high prevalence of LVH/LV dysfunction. Although possible, reversal of systolic and diastolic dysfunction is sporadic after treatment of acromegaly., (© 2021. Italian Society of Endocrinology (SIE).)

Objectives: Rheumatic heart disease is endemic in sub-Saharan Africa. There is a paucity of data on the outcomes of valvular surgery for rheumatic heart disease in the developing world. The objective of this study was to evaluate the outcomes of aortic and mitral valve surgery for rheumatic heart disease in Ethiopia., Methods: Between 2009 and 2017, 240 consecutive patients with rheumatic heart disease underwent aortic and/or mitral surgery at the Cardiac Center of Ethiopia in Addis Ababa. These surgeries were performed in the context of 22 international humanitarian missions. Median follow-up was 2.3 (interquartile range, 0.5-4.0) years and 96% complete. Outcomes were compared between patients who underwent mechanical valve implantation (n = 90, 38%), bioprosthetic valve implantation (n = 58, 24%), and valve repair (n = 92, 38%)., Results: Mean age of patients was 19 ± 8 years, and 136 patients (57%) were female. Operative mortality occurred in 5 patients (2.1%) and was not significantly different between the groups. Eleven additional patients (5%) died at follow-up, and 55 patients (23%) had at least 1 major adverse valve-related event. Propensity score-adjusted Cox regression analysis demonstrated higher rates of death in the bioprosthetic group compared with the mechanical group (hazard ratio, 8.82; 95% confidence interval, 1.64-47.39; P = .011). Survival was not significantly different between the repair and mechanical groups (hazard ratio, 1.09; 95% confidence interval, 0.17-7.16; P = .93). Likewise, rates of major adverse valve-related event were higher in the bioprosthetic group compared with the mechanical group (hazard ratio, 2.71; 995% confidence interval, 1.13-6.49; P = .025), but not significantly different between the repair and mechanical groups (hazard ratio, 1.98; 95% confidence interval, 0.89-4.39; P = .092)., Conclusions: Left-sided valve surgery for rheumatic heart disease in sub-Saharan Africa is associated with acceptable perioperative outcomes, but a high incidence of major adverse valve-related event at follow-up. The use of bioprosthetic valves is associated with poor outcomes in this patient population., (Copyright © 2020. Published by Elsevier Inc.)

Background: Valve thrombosis is a potentially lethal complication of mechanical cardiac valves. We examined the clinical characteristics as well as the early outcomes of patients undergoing emergency surgery for left-sided mechanical valve thrombosis., Methods: Between January 2012 and May 2020, 104 consecutive patients were offered an emergency redo surgery for acute mechanical valve thrombosis. Ninety-seven of these patients were included in the current study., Results: The mean age was 34.2 ± 10.3 years. Most of the patients were females (61 patients), and 27 patients (27.8%) were pregnant. The mitral valve was the site of thrombosis in 81 patients. Inadequate anticoagulation was found in 60.8% of patients. The overall early mortality was 32.9% (32 patients) with an operative mortality of 25.7%. Outcomes in the pregnant subgroup tended to be worst with a maternal mortality in the range of 37%, and with fetal and neonatal survival as low as 33.3%., Conclusion: The overall mortality in cases of mechanical valve thrombosis warranting surgery remains high. Since inadequate anticoagulation seems to be one of the major precipitating factors, the current study highlights the need for improvements in anticoagulation practices. The use of tissue valves should also be contemplated more seriously in some younger patients, especially females expressing the desire for future pregnancies.

Valve disease and particularly calcific aortic valve disease (CAVD) and diabetes (DM) are progressive diseases constituting a global health burden for all aging societies (Progress in Cardiovascular Diseases. 2014;56(6):565: Circulation Research. 2021;128(9):1344). Compared to non-diabetic individuals (The Lancet. 2008;371(9626):1800: The American Journal of Cardiology. 1983;51(3):403: Journal of the American College of Cardiology. 2017;69(12):1523), the diabetic patients have a significantly greater propensity for cardiovascular disorders and faster degeneration of implanted bioprosthetic aortic valves. Previously, using an original experimental model, the diabetic-hyperlipemic hamsters, we have shown that the earliest alterations induced by these conditions occur at the level of the aortic valves and, with time these changes lead to calcifications and CAVD. However, there are no pharmacological treatments available to reverse or retard the progression of aortic valve disease in diabetes, despite the significant advances in the field. Therefore, it is critical to uncover the mechanisms of valve disease progression, find biomarkers for diagnosis and new targets for therapies. This review aims at presenting an update on the basic research in CAVD in the context of diabetes. We provide an insight into the accumulated data including our results on diabetes-induced progressive cell and molecular alterations in the aortic valve, new potential biomarkers to assess the evolution and therapy of the disease, advancement in targeted nanotherapies, tissue engineering and the potential use of circulating endothelial progenitor cells in CAVD., (© 2021 The Authors. Journal of Cellular and Molecular Medicine published by Foundation for Cellular and Molecular Medicine and John Wiley & Sons Ltd.)

Mitral valve disease (MVD) is an important and most frequently acquired heart disease found in dogs. MVD is classified into different stages according to its severity. There is a challenge in differentiation between asymptomatic and symptomatic stages of the MVD. Moreover, pulmonary hypertension (PH) is a common complication in dogs affected by MVD. In clinical practice, there are also some limitations to identify PH. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) is a technique that can characterize specific patterns of peptide mass called peptide barcodes from various samples. Besides, in combination with liquid chromatography-tandem mass spectrometry (LC-MS/MS), potential peptide sequences associated with specific conditions could be identified. The present study aimed to use MALDI-TOF coupled with LC-MS/MS to characterize specific peptide barcodes and potential peptide candidates in serum samples from healthy dogs, dogs with MVD stage B (MVD B, asymptomatic stage), MVD stage C (MVD C, symptomatic stage), MVD stage B with PH (MVD B PH), and MVD stage C with PH (MVD C PH). Discrete clusters of the 5 sample groups were identified by 3D plot analysis. Peptide barcodes also revealed differences in peptide patterns among the 5 groups. Six amino acid sequences of peptide candidates at 1,225.60, 1,363.85, 1,688.71, 1789.52, 2020.21, and 2156.42 Da were identified as part of the proteins CLCN1, CLUL1, EDNRA, PTEN, SLC39A7, and CLN6, respectively. The network interactions between these discovered proteins and common cardiovascular drugs were also investigated. These results demonstrate that MALDI-TOF MS has promise as an optional technique for diagnosing dogs affected by asymptomatic and symptomatic stages of MVD with and without PH. Further studies are required to identify peptide barcodes in dogs with other diseases to create peptide barcode databases in veterinary medicine before using this method as a novel diagnostic tool in the future., Competing Interests: The authors have declared that no competing interests exist.

Background: Limited surgical options are available for patients with extensive mitral annular calcification. Several reports have shown the feasibility of surgical mitral valve replacement (MVR) using a transcatheter aortic valve in the mitral position (MVR-TAVR). This study reviewed 30-day and 1-year outcomes of a minimally invasive approach for MVR-TAVR., Methods: Between 2017 and 2019, 16 patients underwent MVR-TAVR under direct vision. Primary end points included overall survival, technical success, and effectiveness. Secondary end points included valve and cardiac hemodynamics postoperatively and during follow up., Results: The 16 patients (69% female) were 53 to 88 years of age (average, 77 ± 9 years). Comorbidities on presentation were as follows: 31% (5 of 16) were reoperative procedures; 13% (3 of 16) of patients had right ventricular dysfunction, 31% (5 of 16) had severe pulmonary hypertension, and 20% (3 of 16) had chronic kidney disease. Isolated MVR-TAVR was performed on 69% (11 of 16) of patients, with a cross-clamp (58%; 7 of 12) or on a fibrillating heart (42%; 5 of 12), whereas concomitant MVR-TAVR was performed in 31% (5 of 16) of patients with a cross-clamp. At follow-up, the estimated 30-day mortality rate was 12.5%, and the 1-year mortality rate was 36.2%. A moderate postoperative paravalvular leak (PVL) was evident immediately in 1 patient, and a severe PVL was noted after 30-day follow-up in another. In addition, 1 patient had mild left ventricular outflow tract obstruction, and another had moderate mitral valve stenosis. At 1-year follow-up, there were no changes in the mild PVL and moderate stenosis identified postoperatively., Conclusions: Minithoracotomy MVR-TAVR is an acceptable alternative to conventional and transcatheter MVR in patients with mitral valve disease and extensive mitral annular calcification., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Murine animal models are an established standard in translational research and provides a potential platform for studying heart valve disease. To date, studies on heart valve disease using murine models have been hindered by a lack of appropriate methodologies due to their small scale. In the present study, we developed a multi-scale, imaging-based approach to extract the functional structure and geometry for the murine heart valve. We chose the pulmonary valve (PV) to study, due to its importance in congenital heart valve disease. Excised pulmonary outflow tracts from eleven 1-year old C57BL/6J mice were fixed at 10, 20, and 30 mmHg to simulate physiological loading. Micro-computed tomography was used to reconstruct the 3D organ-level PV geometry, which was then spatially correlated with serial en-face scanning electron microscopy imaging to quantify local collagen fiber distributions. From the acquired volume renderings, we obtained the geometric descriptors of the murine PV under increasing transvalvular pressures, which demonstrated remarkable consistency. Results to date suggest that the preferred collagen orientation was predominantly in the circumferential direction, as in larger mammalian valves. The present study represents a first step in establishing organ-level murine models for the study of heart valve disease.

Valvular heart diseases have long been considered to be similar in men and women and across races/ethnicities. Recently, studies have demonstrated major differences between sexes. Unfortunately, studies on valvular heart diseases, as on other cardiovascular diseases, are mostly performed in Caucasian men or in cohorts with a vast majority of Caucasian men. Therefore, our knowledge on valvular diseases in women and non-Caucasians remains limited. Nevertheless, aortic stenosis has been shown to be almost as prevalent in women as in men, and less prevalent in African Americans. Men appear to have a more calcified aortic valve lesion, and women tend to have a more fibrosed one. Primary mitral regurgitation is more frequent in women who have more rheumatic and Barlow etiologies, whereas men have more fibroelastic deficiency and posterior leaflet prolapse/flail. Left ventricular remodelling due to valvular heart diseases is sex related in terms of geometry and probably also in composition of the tissue. Outcomes seem to be worse in women after surgical interventions and better than or equivalent to men after transcatheter ones. Regarding other valvular heart diseases, very few studies are available: Aortic regurgitation is more frequent in men, isolated tricuspid regurgitation more frequent in women. Rheumatic valve diseases are more frequent in women and are mostly represented by mitral and aortic stenoses. Many other sex/gender- and race/ethnic-specific studies are still needed in epidemiology, pathophysiology, presentation, management, and outcomes. This review aims to report the available data on sex differences and race specificities in valvular heart diseases, with a primary focus on aortic stenosis and mitral regurgitation., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)