Pseudoxanthoma elasticum (PXE) is a rare, genetic, metabolic disease with an estimated prevalence of between 1 per 25,000 and 56,000. Its main hallmarks are characteristic skin lesions, development of choroidal neovascularization, and early-onset arterial calcification accompanied by a severe reduction in quality-of-life. Underlying the pathology are recessively transmitted pathogenic variants of the ABCC6 gene, which results in a deficiency of ABCC6 protein. This results in reduced levels of peripheral pyrophosphate, a strong inhibitor of peripheral calcification, but also dysregulation of blood lipids. Although various treatment options have emerged during the last 20 years, many are either already outdated or not yet ready to be applied generally. Clinical physicians often are left stranded while patients suffer from the consequences of outdated therapies, or feel unrecognized by their attending doctors who may feel uncertain about using new therapeutic approaches or not even know about them. In this review, we summarize the broad spectrum of treatment options for PXE, focusing on currently available clinical options, the latest research and development, and future perspectives.

Major vascular complications (VCs) of ilio–femoral arterial access after percutaneous coronary interventions are infrequent, but are associated with increased mortality and morbidity. Routine endovascular repair of VCs is becoming the treatment of choice, especially for patients who cannot tolerate vascular surgery due to advanced cardiovascular disease or are in a bailout situation. Here, we review the different types of vascular access site complications associated with percutaneous coronary interventions (PCIs) and assess the safety and efficacy of endovascular treatment. Data were retrospectively analysed from patients who experienced VCs after transfemoral PCIs, from December 2014 to May 2018. During this period, out of 2833 patients who underwent femoral coronary interventions, 53 (1.9%) experienced major VCs. In total, 40/53 (75.5%) cases with major VCs led to unplanned endovascular repair and 13/53 (24.5%) cases required surgical repair. VCs included 17 (32.1%) retroperitoneal bleeding events (BARC-2, 3a,b), 20 (37.7%) intimal dissections, and 16 (30.2%) femoral pseudoaneurysms. Overall, 32 (60.4%) patients received a covered stent, two (3.8%) received a nitinol stent, five (9.4%) patients with dissections were treated with prolonged balloon angioplasty alone, and one patient with femoral pseudoaneurysm underwent thrombin injection with simultaneous balloon occlusion. The mean hospital stay for patients after endovascular treatment was 11.06 ± 5.2 days, while for patients after surgical repair it was 17 ± 8.2 days. Endovascularly treated patients were transfused with red blood cells (13/40 32.5% vs. 2/13 15.4%) significantly more often than patients treated surgically, although surgically treated patients received more red blood cell concentrates per unit than endovascularly treated patients (1 ± 0.47 vs. 2 ± 0.93). During the one-year follow-up, no intermittent claudication was reported, and no patient required secondary endovascular or surgical repair. For patients who cannot tolerate vascular surgery due to advanced cardiovascular disease or are in a bailout situation, endovascular management of VCs following PCIs seems to be a feasible and safe treatment option, and represents an alternative to surgical repair in life-threatening situations. Endovascular treatment was associated with significantly fewer red blood cell concentrates per patient and fewer days in hospital than surgical treatment.

Despite advancements in surgical and endovascular interventions and improved screening protocols, abdominal aortic aneurysm (AAA) remains a progressive vascular condition associated with significant morbidity and mortality owing to rupture. However, the mechanisms underlying the progression of AAA are poorly understood. AAA progression is driven by complex biological mechanisms, including endothelial dysfunction, chronic inflammation, extracellular matrix (ECM) degradation, proteolytic activity, and vessel wall remodeling. Some studies highlight proteases like matrix metalloproteinases in ECM remodeling, while others focus on miRNAs regulating inflammation; risk factors such as smoking and hypertension further increase vascular wall stress and aneurysm growth. The lack of detailed understanding limits the development of targeted therapies and individual risk assessments. Risk-prediction models are promising; however these models require further external validation to ensure reliability and clinical applicability. Personalized approaches integrating biomechanics and advanced imaging may improve rupture risk assessment. However, trials of antibiotics and renin–angiotensin system inhibitors have shown limited benefit. Observational studies have suggested potential benefits of metformin and statins. Preclinical studies have proposed that targeting inflammatory pathways such as the NOD-like receptor P3 inflammasome is a novel therapeutic strategy to mitigate aneurysm progression. Furthermore, innovative nanoparticle-based drug delivery systems have been explored to deliver matrix metalloproteinase inhibitors directly to the aneurysm site to prevent aneurysm expansion while minimizing systemic side effects. Integrative research is urgently needed to clarify AAA progression, improve outcomes, and enable personalized detection of high-risk subthreshold AAAs while avoiding overtreatment of low-risk cases. This review consolidates current knowledge on AAA pathophysiology, epidemiology, and treatment challenges. Graphical Abstract

Stroke remains a clinical risk in the long-term follow-up of patients after transcatheter aortic valve implantation (TAVI). We aimed to investigate the association between existing carotid artery stenosis (CS) and the risk of stroke after TAVI. The study participants were consecutive patients who underwent TAVI between 2008 and 2020 and were screened for CS at baseline and at the 30-day follow-up. Hemodynamically relevant CS was defined as a reduction of the carotid artery caliber by >50% in relation to the distal segment. The primary endpoint of this study was ischemic stroke or transient ischemic attack (TIA) within 30 days of TAVI. Stroke-related death was also assessed. Of the 1,537 patients included, 220 (14.3%) patients had CS ≥50%. Within 30 days following TAVI, 41 patients (2.7%) experienced an ischemic stroke or TIA, and seven had stroke-related death. The median time from TAVI to stroke/TIA was 3.0 days. Patients with CS ≥50% had a 2.56-fold increased risk of stroke/TIA after TAVI (95%CI: 1.29–5.10, P = .006). The association was consistent in the multivariable model after adjusting for sex, atrial fibrillation, and estimated glomerular filtration rate. Investigations are needed to mitigate the risk of stroke in patients with CS ≥50%.

The involvement of myeloperoxidase in the production of dysfunctional high-density lipoproteins and oxidised biomolecules leads to oxidative stress in the blood vessel endothelium. This prospective cohort study aimed to examine the prognostic value of myeloperoxidase in patients with peripheral artery disease in relation to major adverse cardiac events (MACEs), target lesion revascularisation, and major adverse limb events (MALEs) and its association with multi-bed vascular disease, which is defined as any combination of the following: peripheral artery disease and coronary artery disease. Myeloperoxidase levels were measured in patients with peripheral artery disease and coronary artery disease during angiography. A total of 94 patients were analysed and followed up regarding their MACEs, target lesion revascularisation, and MALEs from August 2016 until February 2019. Among patients with peripheral artery disease, the rates of MACE and mortality were higher in patients with high myeloperoxidase levels than in those with low myeloperoxidase levels; the myeloperoxidase levels were 3.68 times higher in these patients (p < 0.0001). Patients with peripheral artery disease and coronary artery disease (multi-bed vascular disease) had higher myeloperoxidase levels than those with only peripheral artery disease and only coronary artery disease (one-bed vascular disease). Peripheral artery disease patients with higher myeloperoxidase levels had significantly higher rates of limb ischaemia, requiring further revascularisation than those with low myeloperoxidase levels. High myeloperoxidase levels suggest poor outcomes and are associated with MACE and limb ischaemia. Our findings indicated that myeloperoxidase levels could become a prognostic marker and may be used in conjunction with other methods for risk stratification in patients with peripheral artery disease and multi-bed vascular disease.