Catastrophic antiphospholipid syndrome (CAPS) is a fulminant and life-threatening manifestation of antiphospholipid syndrome (APLS), often presenting with rapid multi-organ dysfunction and high mortality despite aggressive therapy. Its associations with hemophagocytic lymphohistiocytosis (HLH) and lupus myocarditis are not well understood. We report the case of a 43-year-old woman with systemic lupus erythematosus (SLE) and secondary APLS who developed CAPS following elective mitral valve repair. Her course was complicated by renal cortical necrosis, recurrent lupus myocarditis, refractory immune thrombocytopenia, sepsis, and HLH, requiring multiple immunosuppressive modalities including corticosteroids, intravenous immunoglobulin, plasma exchange, rituximab, cyclophosphamide, and anakinra. Despite intensive multidisciplinary management across rheumatology, cardiology, nephrology, hematology, infectious diseases, and intensive care, she suffered progressive multi-organ failure and transitioned to palliative care. This case highlights the diagnostic and therapeutic complexity of overlapping autoimmune syndromes, the risks of surgical triggers in predisposed patients, and the challenges of balancing immunosuppression against infection in the setting of CAPS. It underscores the need for dynamic multidisciplinary collaboration, early recognition of HLH and lupus myocarditis overlap, and timely integration of palliative care when maximal therapy fails.

Surgical and transcatheter mitral valve interventions are the mainstay of treatment for mitral regurgitation (MR). Their impact in patients with cancer has recently gained attention in observational studies but remains poorly characterized. We performed a systematic review and meta-analysis to evaluate outcomes of mitral valve interventions, with particular focus on M-TEER, in patients with active or prior cancer. PubMed and Scopus were systematically searched according to the PRISMA 2020 Statement. This systematic review was prospectively registered in PROSPERO (CRD420261368392). Studies reporting outcomes of transcatheter or surgical mitral valve interventions in patients with cancer were eligible. The primary outcome was all-cause mortality. Secondary outcomes included short-term mortality, procedural success, heart failure worsening or hospitalization, and reintervention. Hazard ratios (HRs) were pooled using random-effects models when appropriate. When HRs were not directly available, they were reconstructed according to established methods for time-to-event data synthesis. Eight observational studies met the inclusion criteria. Seven studies evaluated M-TEER, accounting for the vast majority of patients included in the quantitative analyses (1522 patients with cancer and 4716 controls), whereas only one study assessed surgical mitral valve intervention. In the pooled analysis of M-TEER studies, cancer was associated with a significantly higher risk of all-cause mortality during follow-up (HR 1.72, 95% CI 1.03-2.90; I2 = 74.8%). This association remained consistent across prespecified subgroup and sensitivity analyses. No significant differences were observed in 30-day mortality or procedural success between patients with and without cancer. Data on heart failure outcomes and reintervention were insufficient for quantitative synthesis. This study provides a quantitative synthesis of available evidence regarding long-term outcomes after M-TEER in patients with cancer. Patients with cancer undergoing M-TEER have higher long-term mortality than those without cancer, despite similar procedural success and short-term outcomes. These findings suggest the observed excess mortality occurs in patients with cancer but the available evidence does not establish the factors responsible for this association, which could be mediated by frailty, CV comorbidity, treatment-related comorbidity mitral regurgitation (MR) characteristics, or other residual confounders. Of note, studies comparing matched cohorts found a non-significant difference in HR for long-term mortality. Current evidence is limited by observational data and residual confounding. Prospective cardio-oncology studies are needed to improve patient selection and identify individuals most likely to benefit from transcatheter mitral valve intervention.

Computed tomography angiography (CTA) is crucial for preprocedural TAVI planning, providing the anatomical information required for prosthesis sizing and vascular access assessment. As the volume of TAVI procedure increases, improving efficiency and standardizing annotations is becoming essential in clinical practice. This study presents TAVI-TEC, a fully automated artificial intelligence-based framework integrated into a web-based DICOM viewer for routine preoperative TAVI planning. Pre-procedural CTA scans from patients undergoing TAVI with SAPIEN 3 Ultra (S3U) prostheses were processed using a fully automated pipeline. Deep learning-based segmentation of cardiovascular structures, calcification detection, centerline extraction, landmark identification, and annular plane definition was implemented to quantify key annular and aortic root measurements and color-coded maps of lumen reduction and vessel diameter for vascular access. A multilayer perceptron classifier was trained to predict prosthesis size prior to the TAVI procedure. Results revealed that TAVI-TEC enabled pre-procedural measurements in approximately 2-6 min. Strong agreement with clinician-derived measurements was observed for annular area (coefficient of concordance, CCC = 0.934; interclass correlation coefficient, ICC = 0.935; R² = 0.881) and perimeter (CCC = 0.909; ICC = 0.909; R² = 0.854). The valve-size prediction model achieved a classification accuracy of 77.1% (95% CI: 70.1-83.9%) with the clinically selected and implanted valve size, with most discordances occurring between adjacent prosthesis sizes. These findings support the technical feasibility and promising initial performance of TAVI-TEC. Prospective multicenter and external validation, including additional valve platforms, will be required before routine clinical implementation and before its effects on operator variability and clinical workflow can be established.

Background: Bicuspid aortic valve disease (BAVD) is the most frequent congenital heart disease, occurring either as an isolated lesion or in association with other congenital cardiovascular malformations, with variable patterns of progression and risk of valvular and vascular complications. Studies addressing pediatric bicuspid aortopathy are still in their infancy. This narrative review aims to provide a comprehensive perspective on the current scientific evidence regarding BAVD-associated aortopathy in pediatric patients, underscoring multiple challenges in initial diagnosis, long-term surveillance, and therapeutic decision-making, and focusing on the potential roles of circulating biomarkers and advanced multimodal imaging tools that may improve individualized risk stratification. Methods: Despite the narrative design of this review, a structured search of the current available literature was performed to identify studies addressing pediatric BAV, associated aortopathy, biomarkers, vascular remodeling, and multimodal imaging. Priority was given to pediatric cohorts, longitudinal studies, consensus documents, and contemporary guidelines. The search was conducted in the online databases PubMed/Medline and Web of Science for English-language original articles published in the last 10 years, up to May 2026. We used the following main terms: "Bicuspid Aortic Valve Disease" [MeSH], "Aorta" [MeSH], "Infant" [MeSH], "Child" [MeSH], "Adolescent" [MeSH], combined by Boolean operators with secondary keywords: "pathogenesis", "mechanism", "progression", "multimodal imaging", "echocardiography", "cardiovascular magnetic resonance imaging", "computed tomography", "circulating biomarkers". The retrieved studies were screened for eligibility using previously established inclusion and exclusion criteria. A total of 63 studies were included in the analysis for this narrative review. Results: Two main theories underpin the etiopathogenesis of aortopathy associated with the bicuspid aortic valve, positing that genetic factors predispose the aortic wall to remodeling in an abnormal hemodynamic environment. Embryological development and dysregulation of molecular and cellular structures are also intertwined during the formation and progression of the aortic valve with two semilunar cusps, resulting in consequent alterations in the aortic wall's architectural organization. Advances in molecular studies have highlighted circulating biomarkers with potential utility in predicting aortopathy, as they are involved in extracellular matrix remodeling, endothelial dysfunction, and aortic valve calcification, including matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs), transforming growth factor-β (TGF-β), and microRNAs. In addition, multimodal imaging techniques have emerged as essential tools for assessing the morphology and function of cardiovascular structures, particularly aortic biomechanical properties and hemodynamic abnormalities, at the time of initial diagnosis and during regular monitoring. Taken together, blood biomarkers and imaging parameters of aortic remodeling and flow disturbances might gain increasing prognostic value in pediatric BAVD-associated aortopathy. However, larger longitudinal studies are required for clinical validation beyond research settings. Although guidelines on the management of BAVD are available for adults, they are not entirely applicable to children, who are undergoing continuous somatic growth that affects diagnostic possibilities and therapeutic options. Conclusions: Despite growing literature in the realm of BAVD and related conditions, the management of pediatric patients remains challenging in daily clinical practice, as adult guidelines cannot be completely applied to children. Given the heterogeneity and complexity of pathogenic mechanisms, clinical presentations, natural history, and outcomes, future research is warranted to explore the progression profiles of valvular and vascular disorders associated with BAV in children and to achieve an optimal approach to pediatric bicuspid aortopathy.

Background: Valvular heart disease frequently accompanies acute heart failure (AHF), yet most studies address single valve lesions, and the prognostic weight of concurrent multivalvular involvement is less well defined. We examined the prevalence of single- and multi-valve disease and its relationship with one-year mortality. Methods: We analyzed 530 consecutive patients enrolled in a prospective single-center AHF registry between February 2023 and June 2025 and followed for 12 months. Aortic, mitral and tricuspid disease was graded according to European Society of Cardiology/European Association of Cardiovascular Imaging (ESC/EACVI) criteria, a valve being considered involved when it carried at least moderate stenosis and/or regurgitation; multivalvular disease was defined as involvement of two or more valves. The primary endpoint was one-year all-cause mortality. Cox regression was adjusted for admission log N-terminal pro-B-type natriuretic peptide (NT-proBNP), frailty (Clinical Frailty Scale ≥ 5) and age. In-hospital acute kidney injury was examined descriptively but was not entered into the models, because it is ascertained after admission. Results: Overall, 71.3% of patients had at least one significantly diseased valve-mitral in 44.3%, tricuspid in 43.8% and aortic in 27.5%-and 34.9% had multivalvular involvement. One-year mortality rose stepwise with the number of valves involved: 19.7%, 24.9%, 36.3% and 54.0% for zero, one, two and three valves, respectively (log-rank p < 0.001), corresponding to a crude hazard ratio (HR) of 1.51 (95% confidence interval [CI] 1.28-1.78) per additional valve. Multivalvular versus single or no valve involvement carried a crude HR of 2.07 (1.51-2.84) and remained associated with mortality after adjustment (HR 1.48, 95% CI 1.06-2.07, p = 0.020), as did each additional valve (HR 1.22, 1.03-1.45, p = 0.023). Adjustment for clinically manifest right heart failure (HF) attenuated the association with mortality (HR 1.15, 95% CI 0.96-1.37), whereas the association with the triple composite remained significant (HR 1.19, 95% CI 1.03-1.36). Propensity-score adjustment excluding right HF retained the association with mortality (HR 1.43, 95% CI 1.02-2.00), whereas inclusion of right HF attenuated it (HR 1.33, 0.94-1.87). The association was not detectable in the 304 patients without clinically manifest right HF (HR 1.06, 0.80-1.41, p = 0.69). In a post-discharge sensitivity analysis, the adjusted associations were directionally similar but did not reach statistical significance. Conclusions: In hospitalized AHF, multivalvular involvement was common, followed a predominantly mitral-tricuspid pattern, and identified an older, frailer, more congested phenotype with substantial right-sided and chronic renal involvement. Valve burden showed a graded association with one-year mortality that persisted after adjustment for baseline prognostic characteristics, but was attenuated once right-sided involvement was taken into account, whether by direct adjustment or by propensity methods; the association was retained when right HF was omitted from the propensity model. Because right-sided variables lie downstream of significant tricuspid disease, this attenuation is what adjustment for an intermediate is expected to produce and does not establish absence of prognostic value. Cumulative valve burden is therefore best interpreted as a powerful phenotypic marker of a more advanced HF phenotype with prominent right-heart involvement, rather than as an independent causal determinant of mortality.

Objectives: Conventionally, the gold standard for aortic valve disease has been surgical aortic valve replacement (SAVR). Advances in transcatheter aortic valve replacement (TAVR) have since produced marked improvements in both outcomes and procedural volume. We sought to characterize this evolution by comparing the earliest and most recent 100 procedures of each modality at a single high-volume institution. Methods: We retrospectively compared four cohorts of 100 consecutive isolated procedures: 100 TAVRs from 2012 (the earliest period of complete and verifiable registry capture) and 100 first-time SAVRs from a contemporaneous period (2011), each versus the 100 most recent procedures of the same modality (TAVR 2024; SAVR 2024-2025). The SAVR cohorts were limited to first-time, isolated replacement; the TAVR cohorts to native-valve procedures. The primary endpoint was the 30-day composite of death or stroke. Secondary endpoints included new permanent pacemaker implantation, paravalvular leak, vascular access route, anesthetic technique, and length of stay. Results: For TAVR, the 30-day composite of death or stroke fell from 10% to 1% (p = 0.010), with fewer in-hospital deaths (6% to 0%; p = 0.029) and less new dialysis (4% to 0%; p = 0.059). A paravalvular leak of at least mild severity fell from 27% to 3% (p < 0.001), with none as moderate or greater in either era. New pacemaker implantation decreased from 15% to 6% (p = 0.056). Within the same comparison, general anesthesia decreased from 100% to 23%, non-transfemoral access from 29% to 2%, median ICU stay from 43 to 0 h, and hospital stay from 6 to 1 day (all p < 0.001). SAVR outcomes remained similar (composite 2% to 0%, p = 0.497; pacemaker 1% to 0%; and paravalvular leak 0% to 4%), while prolonged ventilation (11% to 1%; p = 0.007) and hospital stay (8 to 5 days; p < 0.001) improved. The SAVR population became younger (74 to 64 years; p < 0.001), and isolated first-time surgery was markedly less frequent, requiring 18.9 versus 5.7 months to accrue 100 consecutive cases. Conclusions: In this two-era institutional comparison, recent-era TAVR outcomes were markedly better, while SAVR outcomes remained excellent in both eras within a recent population that was younger and in whom eligible isolated first-time surgery was less frequent. Because the eras differed substantially in patient risk profile, these unadjusted within-modality comparisons describe a real-world redistribution of aortic stenosis care between two increasingly complementary treatments rather than isolating the effect of any single procedural or technological factor.