Williams–Beuren syndrome (WBS) is a rare, microdeletion syndrome characterized by facial dysmorphisms, intellectual disability, a friendly personality, cardiovascular and other abnormalities. ...Cardiovascular defects (CVD) are among the most prevalent characteristics in WBS, being supravalvular aortic stenosis (SVAS) the most frequent, followed by peripheral pulmonary stenosis (PPS). A comprehensive retrospective review of medical records of 127 patients with molecular diagnosis of WBS, in a period of 20 years, was done to evaluate the incidence, the natural history of cardiovascular disease, and the need for surgical intervention, including heart transplantation (HT). A total of 94/127 patients presented with CVD. Of these 94 patients, 50% presented with SVAS and 22.3% needed heart surgery and/or cardiac catheterization including one that required HT due to severe SVAS‐related heart failure at 19 years of age. The patient died in the postoperative period due to infectious complications. Cardiovascular problems are the major cause of sudden death in patients with WBS, who have a significantly higher mortality risk associated with surgical interventions. There is a higher risk for anesthesia‐related adverse events and for major adverse cardiac events following surgery. End‐stage heart failure due to myocardial ischemia has been described in WBS patients and it is important to consider that HT can become their only viable option. To our knowledge, the case mentioned here is the first HT reported in an adolescent with WBS. HT can be a viable therapeutic option in WBS patients with adequate evaluation, planning, and a multidisciplinary team to provide the required perioperative care and follow‐up.
Autoimmune inflammatory reactions leading to rheumatic fever (RF) and rheumatic heart disease (RHD) result from untreated Streptococcus pyogenes throat infections in individuals who exhibit genetic ...susceptibility. Immune effector mechanisms have been described that lead to heart tissue damage culminating in mitral and aortic valve dysfunctions. In myxomatous valve degeneration (MXD), the mitral valve is also damaged due to non-inflammatory mechanisms. Both diseases are characterized by structural valve disarray and a previous proteomic analysis of them has disclosed a distinct profile of matrix/structural proteins differentially expressed. Given their relevance in organizing valve tissue, we quantitatively evaluated the expression of vimentin, collagen VI, lumican, and vitronectin as well as performed immunohistochemical analysis of their distribution in valve tissue lesions of patients in both diseases. We identified abundant expression of two isoforms of vimentin (45 kDa, 42 kDa) with reduced expression of the full-size protein (54 kDa) in RHD valves. We also found increased vitronectin expression, reduced collagen VI expression and similar lumican expression between RHD and MXD valves. Immunohistochemical analysis indicated disrupted patterns of these proteins in myxomatous degeneration valves and disorganized distribution in rheumatic heart disease valves that correlated with clinical manifestations such as valve regurgitation or stenosis. Confocal microscopy analysis revealed a diverse pattern of distribution of collagen VI and lumican into RHD and MXD valves. Altogether, these results demonstrated distinct patterns of altered valve expression and tissue distribution/organization of structural/matrix proteins that play important pathophysiological roles in both valve diseases.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
This study aimed to evaluate the incidence and clinical implications of myocardial injury, as determined by cardiac biomarker increase, in patients who underwent mitral bioprosthesis dysfunction ...treatment with transcatheter mitral valve replacement (TMVR) versus surgical mitral valve replacement reoperation (SMVR-REDO). Between 2014 and 2023, 310 patients with mitral bioprosthesis failure were included (90 and 220 patients for TMVR and SMVR-REDO, respectively). Multivariable analysis and propensity score matching were performed to adjust for the intergroup differences in baseline characteristics. Creatinine kinase-MB (CK-MB) and cardiac troponin I (cTn) were collected at baseline and 6 to 12, 24, 48, and 72 hours after intervention. The cardiac biomarkers values were evaluated in relation to their reference values. The outcomes were determined according to the Mitral Valve Academic Research Consortium criteria. CK-MB and cTn increased above the reference level in almost all patients after SMVR-REDO and TMVR (100% vs 94%, respectively), with the peak occurring within 6 to 12 hours. SMVR-REDO was associated with a two- to threefold higher increase in cardiac biomarkers. After 30 days, the mortality rates were 13.3% in the TMVR and 16.8% in the SMVR-REDO groups. At a median follow-up of 19 months, the mortality rates were 21.1% in the TMVR and 17.7% in the SMVR-REDO groups. Left ventricular ejection fraction, estimated glomerular filtration rate, CK-MB, and cTn were predictors of mortality. In conclusion, some degree of myocardial injury occurred systematically after the treatment of mitral bioprosthetic degeneration, especially after SMVR, and higher CK-MB and cTn levels were associated with increased cumulative late mortality, regardless of the approach.
The current challenge of cardiovascular surgery (CVS) is to improve the outcomes in increasingly severe patients. In this respect, continuous quality improvement (CQI) programs have had an impact on ...outcomes.
To assess the evolution of the incidence and mortality due to CVS, as well as the current outcomes of the Hospital das Clínicas Heart Institute of the University of São Paulo Medical School (InCor-HCFMUSP).
An outcome analysis of CVSs performed at the InCor, between January 1984 and June 2019. We observed the surgical volume and mortality rates in 5 time periods: 1st (1984-1989), 2nd (1990-1999), 3rd (2000-2007), 4th (2008-2015) and 5th (2016-2019). The CQI program was implemented between 2015 and 2016. The analysis included the total number of surgeries and the evolution of the most frequent procedures.
A total of 105,599 CCVs were performed, with an annual mean of 2,964 procedures and mortality of 5,63%. When comparing the 4th and the 5th periods, the average global volume of surgeries was increased from 2,943 to 3,139 (p = 0.368), bypass graft (CABG), from 638 to 597 (p = 0.214), heart valve surgery, from 372 to 465 (p = 0.201), and congenital heart disease surgery, from 530 to 615 (p = 0.125). The average global mortality went from 7.8% to 5% (p < 0.0001); in CABG surgery, from 5.8% to 3.1% (p < 0.0001); in heart valve surgery, from 14% to 7.5% (p < 0.0001) and in congenital heart disease surgery, from 12.1% to 9.6% (p < 0.0001).
In spite of a recent trend towards increased surgical volume, there was a significant decrease in operative mortality in the groups studied. After the implementation of the CQI program, the mortality rates were closer to international standards.
Objective: To assess actual data on the safety, effectiveness, and hemodynamic performance of Bovine Pericardium Organic Valvular Bioprosthesis (BVP). Methods: The BIOPRO Trial is an observational, ...retrospective, non-comparative, non-randomized, and multicenter study. We collected data from 903 patients with symptomatic, moderate, or severe valve disease who underwent BVP implants in the timeframe from 2013 to 2020 at three Brazilian institutions. Death, valve-related adverse events (AEs), functional recovery, and hemodynamic performance were evaluated at the hospital, at discharge, and six months and one year later. Primary analysis compared late (> 30 days after implant) linearized rates of valve-related AEs, such as thromboembolism, valve thrombosis, major hemorrhage, major paravalvular leak, and endocarditis, following objective performance criteria (OPC). Analysis was performed to include at least 400 valve-years for each valve position (aortic and mitral) for complete comparisons to OPC. Kaplan-Meier survival and major adverse cardiovascular and cerebrovascular event analyses were also performed. Results: This retrospective study analyzed follow-up data collected from 903 patients (834.2 late patient-years) who have undergone surgery for 455 isolated aortic valve replacement (50.4%), 382 isolated mitral valve replacement (42.3%), and 66 combined valve replacement or other intervention (7.3%). The linearized rates of valve-related AEs were < 2 × OPC. One-year survival rates were 95.1% and 92.7% for aortic and mitral valve replacement, respectively. This study demonstrated an improvement in the New York Heart Association classification from baseline and hemodynamic performance within an expected range. Conclusion: According to this analysis, BVP meets world standards for safety and clinical efficacy.
•Studies reporting the etiology of prosthetic valve endocarditis (PVE) are an unmet clinical need.•A prospective cohort study was performed along with a literature review to describe the distribution ...of the etiology of PVE.•At >120days after valve surgery, there is a decrease in the incidence of resistant microorganisms.•PVE occurring at >120days after surgery may be treated with the same empirical treatment as for late PVE.•This approach could lead to higher antibiotic efficacy and less damage to the patient’s natural flora.
To determine the annual incidence of prosthetic valve endocarditis (PVE) and to evaluate its current classification based on the epidemiological distribution of agents identified and their sensitivity profiles.
Consecutive cases of PVE occurring within the first year of valve surgery during the period 1997–2014 were included in this prospective cohort study. Incidence, demographic, clinical, microbiological, and in-hospital mortality data of these PVE patients were recorded.
One hundred and seventy-two cases of PVE were included, and the global annual incidence of PVE was 1.7%. Most PVE cases occurred within 120days after surgery (76.7%). After this period, there was a reduction in resistant microorganisms (64.4% vs. 32.3%, respectively; p=0.007) and an increase in the incidence of Streptococcus spp (1.9% vs. 23.5%; p=0.007). A literature review revealed 646 cases of PVE with an identified etiology, of which 264 (41%) were caused by coagulase-negative staphylococci and 43 (7%) by Streptococcus spp. This is in agreement with the current study findings.
Most PVE cases occurred within 120days after valve surgery, and the same etiological agents were identified in this period. The current cut-off level of 365days for the classification of early-onset PVE should be revisited.
•950 patients who experienced thoracic surgical-site infection were studied (131 mediastinitis, 819 superficial/deep infections).•Staphylococcus aureus was identified in 43% of mediastinitis, and the ...overall mortality was 42%.•A diagnostic score for mediastinitis was found. Groups with high (31%) and low (5%) risk of mediastinitis were identified.
The aim of this study was to elaborate on and validate a score for the early diagnosis of mediastinitis after cardiothoracic surgery.
Between 2007 and 2017, patients who experienced thoracic surgical-site infection after cardiothoracic surgery were enrolled. Laboratory, clinical, and chest CT findings were retrospectively analyzed. Patients were followed up until hospital discharge or intra-hospital death. Univariate and multivariate regression analyses were performed.
950 surgical-site infections were found and analyzed (131 mediastinitis, 819 superficial/deep infections). Of the 131 mediastinitis episodes, 88% required surgical thoracic debridement,Staphylococcus aureus was identified in 43%, and overall mortality was 42%. The following variables were related to mediastinitis diagnosis: sternal diastasis (OR=2.5; 95% confidence interval 95%CI: 1.2–5.3; P=0.012), bilateral pleural effusion (OR=1.9; 95%CI: 1.0–3.6; P=0.04), leukocyte count ≥14,000cells/mm3 (OR=2.5; 95%CI: 1.3–4.7; P=0.006), male sex (OR=2; 95%CI: 1.11–4; P=0.022), and positive blood culture (OR=3.0; 95%CI: 1.6–5.6; P=0.001). The score predicted with reasonable accuracy mediastinitis in the derivation cohort (AUC-ROC, 0.7476) and the validation cohort (AUC-ROC, 0.7149). Groups with high (31%) and low (5%) risk of mediastinitis were identified.
An early diagnostic score in patients with surgical-site infection after cardiothoracic surgery identified groups with a low and high risk for mediastinitis.
Infective endocarditis continues to be a significant concern and may be undergoing an epidemiological transition.
Were studied 1804 consecutive episodes of infective endocarditis between 1978 and ...2022. The mean age was 48 ( ± 19), and 1162 (64%) patients were male. Temporal trends in demographic data, comorbidities, predisposing conditions, microorganisms, complications and in-hospital death have been studied over the decades (1978–1988, 1989–1999, 2000–2010 and 2011–2022). The outcomes and clinical characteristics were modeled using nonlinear cubic spline functions.
Valve surgery was performed in 50% of the patients and overall in-hospital mortality was 30%. From the first to the fourth decade studied, the average age of patients increased from 29 to 57 years (p < 0.001), with significant declines in the occurrence of rheumatic valvular heart disease (15% to 6%; p < 0.001) and streptococcal infections (46% to 33%; p < 0.001). Healthcare-associated infections have increased (9% to 21%; p < 0.001), as have prosthetic valve endocarditis (26% to 53%; p < 0.001), coagulase-negative staphylococcal infections (4% to 11%; p < 0.001), and related-complications (heart failure, embolic events, and perivalvular abscess; p < 0.001). These changes were associated with a decline in adjusted in-hospital mortality from 34% to 25% (p = 0.019).
In the 44 years studied, there was an increase in the mean age of patients, healthcare-related, prosthetic valve, coagulase-negative staphylococci/MRSA infections, and related complications. Notably, these epidemiological changes were associated with a decline in the adjusted in-hospital mortality.
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