Tom Verbelen

Background: Pulmonary arterial hypertension (PAH) is a rare, progressive pulmonary vascular disorder characterised by pulmonary arteriole remodelling leading to increased pulmonary vascular resistance (PVR). Key features of PAH are vascular pruning and plexiform lesion formation. While these features have been described histologically, a detailed quantitative analysis is lacking. In this study, we used micro-computed tomography (µCT) to provide three-dimensional quantification of pulmonary vascular alterations. Methods: We performed µCT imaging on explanted lungs from seven patients with end-stage idiopathic (n=4) or heritable (n=3) PAH and five healthy controls. Arterial lumen diameter, the number of acinar vessels and plexiform lesion distribution were quantified and correlated with clinical and haemodynamic data. Results: We observed a 50% reduction in the diameter of the acinar arterial lumen and a 61% decrease in the number of intra-acinar arterioles, confirming vascular pruning. Arterial narrowing correlated with PVR and mean pulmonary artery pressure. Heritable PAH showed a higher number of plexiform lesions than idiopathic PAH, with more distal, acinar-level localisation. Conclusions: This study provides quantitative evidence of arterial narrowing and objective loss of small intra-acinar arteries in end-stage PAH, supporting their central role in elevated PVR. Pulmonary vascular rarefaction may represent an initiating process of vascular remodelling rather than a secondary phenomenon. Heritable PAH was characterised by a higher number and more distal distribution of plexiform lesions than idiopathic PAH, which may contribute to its more severe phenotype.

Objectives: Pulmonary artery sarcoma (PAS) is a rare and aggressive malignancy often mimicking pulmonary embolism, leading to diagnostic delays and poor outcomes. This study aims to describe the clinical characteristics, diagnostic pathways, treatment modalities, and outcomes of PAS patients in a multicentre cohort. Methods: This retrospective cohort study included patients from 7 institutions between 1994 and 2024. Clinical, radiological, histological, and treatment data were collected via a standardized REDCap-based platform. All patients underwent either multimodal treatment or single therapy approach, including systemic therapy, surgery, and/or radiotherapy. Kaplan–Meier survival analysis and log-rank testing were performed to assess overall survival (OS) and progression-free survival (PFS). Results: 84 patients from 7 international centres were analysed. The mean age was 54.4 years, and 43.8% of patients were female. The most common presenting symptoms were dyspnoea (81.2%) and chest pain (50.0%). Pulmonary embolism was the most frequent initial misdiagnosis (49.1%). Histologically, PAS was diagnosed in 46 cases. Tumours were bilateral in 58.1% of patients with surgery only. Most commonly, endarterectomy was performed in 59.5%. After a median follow-up time of 39 months, 19 were excluded as they either had no treatment information or received monotherapy only. Additionally, 11 patients were excluded due to missing date of diagnosis and 17 due to missing overall follow-up date. There was moderate evidence that OS differed between treatment groups (75th quantile: 34 months for multimodal [95% CI, 26 to not reached] vs 11 months for surgery only [95% CI, 7-21]; log-rank P = .022). There was no evidence that PFS differed between treatment groups (median 19 months for multimodal [95% CI, 14-39] vs 16 months surgery only [95% CI, 11-21]; log-rank P = .69). Conclusions: This study represents the largest reported PAS cohort to date and underscores the diagnostic challenges and poor prognosis associated with this disease. Our findings offer evidence that multimodal treatment provides significant prognostic benefits. However, further prospective studies are warranted to establish standardized diagnostic and therapeutic protocols.

Cardiovascular disease remains the leading global cause of morbidity and mortality. Although advances in prevention, diagnostics, and disease-modifying therapies have prolonged survival, many individuals now live longer with high symptom burden, functional decline, and complex decisional needs. Palliative care (PC) for adults with advanced cardiovascular disease can improve quality of life, support caregivers, and align treatments with patient values and goals. Core elements include symptom management, effective communication, shared decision-making, advance care planning, and integration of psychosocial and spiritual support across the disease trajectory and different care settings. Current evidence demonstrates that early PC intervention can improve symptom control, enhance quality of life, reduce psychological distress, and decrease high-intensity yet low-value care near the end of life. Nevertheless, outside of heart failure populations, gaps in widespread PC implementation across populations supported with cardiac devices as well as across diverse cultural and health systems remain. This state-of-the-art review (i) synthesizes conceptual foundations, referral triggers, and delivery models for PC in cardiovascular medicine; (ii) reviews disease-specific considerations across heart failure, valvular disease, pulmonary hypertension, arrhythmias, and congenital heart disease; (iii) outlines ethical and legal issues including advance directives and device deactivation; (iv) provides practical guidance for symptom management and communication frameworks; and (v) proposes a pragmatic algorithm to support clinical integration of PC into cardiovascular medicine.

Delirium is a common yet underrecognized neuropsychiatric syndrome in cardiovascular medicine associated with prolonged hospitalization, increased mortality, and long-term cognitive decline. Patients undergoing interventional or surgical cardiovascular procedures—such as transcatheter aortic valve replacement, surgical aortic valve replacement, coronary artery bypass grafting, or percutaneous coronary interventions—may be particularly vulnerable to its development. Delirium incidence varies widely across cardiovascular procedures, influenced by patient characteristics, procedural invasiveness, and diagnostic methodology. Risk factors include advanced age, baseline cognitive impairment, cerebrovascular disease, extended operative times, perioperative complications, and systemic inflammation. Diagnostic tools such as the Confusion Assessment Method (CAM) and the Confusion Assessment Method for the Intensive Care Unit (CAM-ICU) score are established but underutilized in the diagnosis of delirium. While preventive strategies emphasizing non-pharmacological, multicomponent approaches—such as early mobilization, cognitive stimulation, and sleep hygiene—are supported by strong evidence, preventive use of pharmacologic agents remains controversial. Pharmacologic treatment is reserved for select cases; dexmedetomidine shows benefits in intensive care unit settings, while antipsychotics like quetiapine and risperidone may be used cautiously. Overall, delirium poses a significant clinical challenge in cardiovascular medicine and requires a proactive, interdisciplinary approach. Systematic risk assessment and multimodal preventive strategies should be the standard of care, while pharmacologic treatment should be symptom- and context-specific. Further high-quality studies are needed to inform evidence-based guidelines tailored to cardiovascular populations. The present state-of-the-art review summarizes the current literature on the epidemiology, mechanisms, clinical manifestations, diagnosis, prevention, and treatment of delirium in cardiovascular medicine. By integrating findings and interdisciplinary expert discussions from interventional cardiology, cardiac surgery, and psychiatry, it aims to define the unique vulnerability of this patient population, highlight critical knowledge gaps, and lay the foundation for developing targeted, evidence-based management strategies.

Right ventricular (RV) function assessment using catheter‐derived pressure‐volume (PV) loops is used in translational research, providing detailed insights into cardiac mechanics. Its practical implementation requires methodological accuracy to ensure rigor, reproducibility and transparency. However, current practices for PV loop acquisition are set at individual laboratories, resulting in overall practice remaining largely unknown. This study aimed to explore current practices in RV PV loop acquisition in large animal research. We therefore conducted a scoping review registered on the Open Science Framework and guided by PRISMA Extension for Scoping Reviews, with comprehensive searches in PubMed, Embase, Scopus, and Web of Science (December 2024). Eligible studies included large animal models published after 2014 using conductance or admittance catheters. Out of 5133 identified records, 62 met inclusion criteria. The review revealed underreporting and substantial variability in methodology, particularly in catheter placement, calibration, and ventilatory mode during PV loop recording. Catheter insertion sites included RV inflow (42%) and outflow tracts (19.4%); fluoroscopy was used in 51.6%. Calibration was not reported in one‐third of studies. Loop recording during apnea were reported in 23%, mechanical ventilation in 26%, and was not specified in 51%. Overall, RV PV loop protocols show substantial heterogeneity, highlighting the need for standardized methodological reporting, to improve transparency, research reproducibility and translational significance. Expert consensus is warranted to establish methodological guidelines.

Background and Aims: Fulminant myocarditis (FM) is a complex clinical syndrome characterized by acute myocardial inflammation and cardiogenic shock. Evidence on long-term outcomes, mortality risk factors, and targeted treatment options remains limited. Methods: This retrospective analysis included consecutive adult patients admitted for FM between January 2012 and November 2022 at 26 European tertiary centres. According to endomyocardial biopsy (EMB) findings, patients were categorized as lymphocytic (LM), eosinophilic (EM), necrotizing eosinophilic (NEM), and giant-cell myocarditis (GCM) for the main analyses. Patients with inconclusive histopathology and those who did not undergo EMB were analysed separately. Primary outcomes were in-hospital and 1-year mortality. Multivariable logistic regression models were employed as generalized linear models to analyse the association of prespecified risk factors with mortality. Estimated associations are reported as odds ratios, generalized marginal effects, and adjusted prediction plots. Results: A total of 271 patients [median age 43 years (interquartile range: 33–55 years), 58% male] were included in the analysis, of whom 146 fulfilled histopathologic criteria of LM (n = 99), EM (n = 18), NEM (n = 3), or GCM (n = 26). In the remaining 125 cases, EMB was inconclusive (n = 48) or not performed (n = 77). While there were no significant differences in cardiac arrest rate, admission Simplified Acute Physiology Score (SAPS) II, ventricular function, initial pH, and liver and kidney function parameters between histopathologic subgroups, the use of immunosuppressive medications and need for organ replacement varied. In the overall population, in-hospital and 1-year mortality were 31% and 34%, respectively. The highest 1-year mortality rate was observed among patients with GCM (LM: 25%, EM: 28%, NEM: 33%, GCM: 54%, P = .04). Patients who survived to discharge had an excellent functional outcome and long-term recovery of ventricular function. In total, 17 patients (6%) received a heart transplant during follow-up. Older age, lower pH, and histopathologic diagnosis of GCM were independently associated with in-hospital and 1-year mortality. Conclusions: In-hospital mortality among patients with FM remains high. Age, pH, and histopathological findings may serve as prognostic markers to identify high-risk patients and to guide treatment strategies. Long-term outcomes are excellent among those who survive to discharge.

Objectives: To assess tricuspid regurgitation evolution after pulmonary endarterectomy and to identify predictive factors for residual postoperative tricuspid regurgitation. Methods: Perioperative and 6-month follow-up echocardiography and right heart catheterization data were retrospectively retrieved from all chronic thromboembolic pulmonary hypertension patients who underwent pulmonary endarterectomy between January 2009 and December 2023 in University Hospitals Leuven, Leuven and St. Antonius Hospital, Nieuwegein. Patients with vs without decreased tricuspid regurgitation and with vs without moderate/severe residual postoperative tricuspid regurgitation were compared. Results: Moderate/severe preoperative tricuspid regurgitation was present in 150/162 (93%) patients. It decreased with at least 1 degree 6 months postoperatively in 70/150 (47%) patients. In these patients, reductions in mean pulmonary artery pressure (−23 [−26; −21] vs −13 [−16; −11] mm Hg, P < .001), pulmonary vascular resistance (−6.73 [−7.61; −5.85] vs −3.28 [−4.05; −2.52] Wood units, P < .001), and N-terminal pro brain natriuretic peptide (ratio post-/preoperative measurement: 0.227 [0.168; 0.307] vs 0.968 [0.741; 1.265], P < .001) were larger than in patients without this decrease. Moderate/severe residual tricuspid regurgitation was seen in 26/162 (16%) patients. Postoperative mean pulmonary artery pressure (34 [30; 37] vs 24 [22; 25] mm Hg, P < .001) and pulmonary vascular resistance (5.54 [4.77; 6.31] vs 2.99 [2.65; 3.33] Wood units, P < .001) were higher in these patients vs those without moderate/severe residual tricuspid regurgitation. Arterial hypertension, older age, atrial fibrillation, shorter 6-min walking distance, and higher tricuspid regurgitation degree were potential preoperative risk factors for residual tricuspid regurgitation. Conclusions: In our study population, tricuspid regurgitation improvement after pulmonary endarterectomy was associated with improved pulmonary haemodynamics. Concomitant tricuspid valve annuloplasty might be considered in patients with atrial fibrillation and a dilated tricuspid valve annulus, but in the absence of risk factors for persistent pulmonary hypertension.