Cardiovascular disease represents the leading cause of death in patients with Anderson-Fabry disease (FD), with sudden cardiac death (SCD) being one of the most frequently reported causes of mortality. However, a significant diagnostic gap persists in identifying patients at risk, as current stratification tools are inadequate for predicting lethal arrhythmic events. This narrative review resumes the existing peer-reviewed literature regarding the pathophysiology of FD arrhythmias and evaluates the diagnostic limitations of the current standard intermittent monitoring tools, such as the 24-hour Holter. Standard monitoring tools, such as the 24-hour Holter often miss paroxysmal, asymptomatic arrhythmias. Recent studies using implantable loop recorders (ILRs) show a significantly higher burden of arrhythmias than previously recognized. Myocardial fibrosis, identified as late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR), is strongly associated with malignant arrhythmias, together with the degree of left ventricular hypertrophy (LVH). Current consensus statements may underestimate the need for continuous monitoring in FD. By providing a comprehensive narrative synthesis of available observational and registry data, this paper proposes an updated, exploratory risk-stratification clinical guide for the extension of countinuous rhythm monitoring in high-risk subgroups of FD.

Early identification of large vessel occlusion (LVO) in acute ischemic stroke is critical, as the benefit of reperfusion therapies diminishes rapidly with time. Although multiple prehospital stroke severity scales exist, most require direct bedside assessment by trained personnel, with limited evidence supporting telephone-based LVO recognition. This study aimed to develop the DAFNES scale and conduct preliminary internal hospital-based validation for LVO identification in patients with suspected acute stroke. A prospective observational study enrolled 593 consecutive patients with suspected stroke admitted to the Emergency Department of a tertiary hospital (January-September 2024). Following exclusion of stroke mimics and intracerebral hemorrhages, 408 patients were analyzed. The DAFNES scale comprises six binary items: oculocephalic deviation, aphasia, facial droop, neglect, hemiparesis, and altered consciousness. Scores were assigned bedside by expert neurologists blinded to CT angiography findings. LVO status was determined by a blinded neurologist based on CT angiography results. Among 408 analyzed patients, 185 (45.3%) presented with LVO and 223 (54.7%) with non-LVO ischemic stroke. The DAFNES scale demonstrated good discriminatory performance with an area under the curve of 0.79 (95% CI 0.75-0.83). At a cutoff score of ≥ 3: sensitivity was 0.78, specificity 0.72, positive predictive value 0.70, and negative predictive value 0.80. Median DAFNES scores were significantly higher in LVO patients compared to non-LVO patients. The DAFNES scale demonstrates promising discriminatory ability for LVO detection in hospital-based cohorts. However, formal prehospital and dispatcher-based validation studies are required before clinical implementation.

Patients on hemodialysis (HD) undergoing coronary artery bypass grafting (CABG) face a high risk of perioperative complications, and the optimal timing of perioperative HD remains unclear. We evaluated the safety and feasibility of a standardized HD protocol in patients undergoing off-pump CABG (OPCAB).

We retrospectively analyzed 64 HD-dependent patients who underwent elective OPCAB at a single institution between March 2014 and December 2024. The protocol included maintaining a Monday-Wednesday-Friday HD schedule, performing routine HD on Monday morning, conducting OPCAB on Monday afternoon, and resuming HD on postoperative day (POD) 2. Clinical outcomes, unplanned dialysis, and perioperative biochemical changes were assessed.

Seven patients (10.9%) required unplanned renal replacement therapy before the first postoperative HD session for congestive heart failure (n = 3), hyperkalemia (n = 3), or respiratory failure (n = 1). None required additional dialysis after the initial postoperative HD. In-hospital mortality was 3.1%. Postoperative congestive heart failure occurred in 4.7% of patients. Body weight returned to near baseline by POD 7, and serum potassium remained within an acceptable range throughout the perioperative period.

This standardized perioperative HD protocol was feasible for maintenance HD patients undergoing elective OPCAB. However, protocol deviation in 10.9% of patients suggests that further refinement and prospective comparative studies are warranted.

This retrospective chart review presents a case of bilateral noninfectious retinitis exhibiting cotton wool spot-like lesions, associated with combined retinal vasculitis. A 26-year-old woman with no known systemic illness presented with bilateral combined retinal vasculitis, associated with multiple cotton wool-like lesions along the vessels. Swept-source optical coherence tomography (SS-OCT) revealed a prominent middle limiting membrane (MLM) sign with cystoid macular edema. Fundus fluorescein angiography (FFA) showed blocked hypofluorescence and optic disc leakage, but notably, no vascular leakage. Serological investigations and neuroimaging were unremarkable, except for a positive antinuclear antibody (ANA). Following initiation of oral corticosteroids, the cotton wool spot-like lesions began to resolve within 2 days, accompanied by an increase in hemorrhagic components, which subsequently resolved over 3 weeks. After complete resolution, optic disc pallor and retinal thinning were observed. This rare case of bilateral combined retinal vasculitis with distinctive cotton wool spot-like lesions along the vascular arcades showed a rapid response to corticosteroid therapy and remained clinically quiescent over long-term follow-up.

To compare the efficacy and safety of expectant management versus acetaminophen for early treatment of hemodynamically significant patent ductus arteriosus (hsPDA) in very preterm infants.

A prospective randomized controlled trial was conducted from April 2024 to November 2025, enrolling 99 very preterm infants aged 4 to 7 days diagnosed with hsPDA. Participants were randomly assigned to either the expectant management group (n=54), which received no drug intervention, or the acetaminophen group (n=45), which received oral acetaminophen at 15 mg/kg every 6 hours for 3 days. Rates of hsPDA persistence at 3-4 days post-enrollment (7-11 days of age), 14-17 days of age, and at discharge or 36 weeks of corrected gestational age were compared between the two groups. Additionally, total duration of respiratory support, serum levels of alanine aminotransferase and cystatin C, and incidences of oliguria, upper gastrointestinal bleeding, intraventricular hemorrhage, moderate to severe bronchopulmonary dysplasia (BPD) were compared.

The acetaminophen group showed lower rates of hsPDA persistence at 3-4 days post-enrollment and at 14-17 days of age compared to the expectant management group (both P0.05). No significant differences were found between groups at discharge or 36 weeks of corrected gestational age in hsPDA persistence, total respiratory support duration, or incidences of oliguria, upper gastrointestinal bleeding, intraventricular hemorrhage, and moderate to severe BPD (all P0.05). The acetaminophen group exhibited higher serum alanine aminotransferase and cystatin C levels at 3-4 days post-enrollment, and lower hourly urine output within 4 days post-enrollment compared with the expectant management group (all P0.05). Smaller gestational age and assisted reproductive technology were independent risk factors for persistent hsPDA at 14-17 days of age (OR=8.569 and 7.092, respectively; P0.05), while acetaminophen use was an independent protective factor (OR=0.126, P0.05).

Early oral acetaminophen treatment in very preterm infants with hsPDA aged 4-7 days reduces the rate of persistent hsPDA at 14-17 days and demonstrates a favorable safety profile compared with expectant management, but does not affect hsPDA persistence at discharge or 36 weeks of corrected gestational age or the rate of moderate to severe BPD.

To examine whether epidural analgesia in labour is associated with neonatal neurological morbidity, other neonatal morbidity, neonatal sepsis, low Apgar score, neonatal mortality, and childhood cerebral palsy.

National population based cohort study.

All NHS hospitals in Scotland, using Scottish NHS administrative linked data.

495 695 women in labour with a singleton pregnancy between 24+0 and 42+6 weeks' gestation delivering vaginally or via unplanned caesarean birth between 1 January 2007 and 31 December 2019.

The primary outcome was neonatal neurological morbidity defined using ICD-10 codes as one or more of hypoxic ischaemic encephalopathy, neonatal seizures, intraventricular haemorrhage, intraventricular infarction, periventricular leukomalacia, meningitis, encephalitis, kernicterus, hypotonia, birth asphyxia, or other cerebral diagnosis occurring within 28 days of birth. Secondary outcomes were other neonatal morbidity (one or more of acidosis at birth (cord artery pH <7.10), traumatic birth injury, brachial plexus injury, necrotising enterocolitis, respiratory distress syndrome, respiratory failure, pneumothorax, hypoglycaemia, or hypothermia), neonatal sepsis, Apgar score <4 at five minutes following birth, neonatal mortality, and cerebral palsy diagnosed at any point during childhood.

Of the 495 695 women, 114 897 (23.2%) had epidural analgesia in labour. Neonatal neurological morbidity occurred in 434 babies (0.9 per 1000 births, 95% confidence interval (CI) 0.8 to 1.0). No association was found between epidural analgesia in labour and neonatal neurological morbidity (adjusted relative risk 0.87, 95% CI 0.68 to 1.12), other severe neonatal morbidity (1.17, 0.90 to 1.51), neonatal sepsis (1.11, 0.90 to 1.37), Apgar score <4 at five minutes (0.97, 0.87 to 1.09), neonatal mortality at 28 days (0.81, 0.62 to 1.06), or cerebral palsy in childhood (0.80, 0.60 to 1.06). Findings were consistent across subgroups including women considered to have high risk pregnancies, preterm births, and across different modes of birth.

Epidural analgesia during labour was not associated with clinically significant risks of harm to newborn babies or children, including risks of neonatal morbidity, death, or cerebral palsy. These findings have important policy implications, and support widening availability and equitable access to epidural analgesia as a safe component of intrapartum care.

Elevated resting heart rate (RHR) predicts mortality in middle-aged and older adults, primarily through cardiovascular disease (CVD). Whether RHR predicts mortality in young adults, which causes of death are involved and whether the association replicates are unclear. In young US adults, who fall below the starting age of current cardiovascular risk tools, we examined RHR and all-cause and cause-specific mortality across two cohorts.

We analysed 3291 adults aged 20-49 years from National Health and Nutrition Examination Survey (NHANES) 1999-2004 (primary cohort) linked to mortality through 2019 (median follow-up 17.8 years; 120 deaths) with replication in 8941 adults from NHANES III (1988-1994; 1317 deaths; median follow-up 27.6 years; temporal replication). RHR and heart rate reserve (HRR) were modelled per 10 beats per minute increment using Cox regression adjusted for demographic, lifestyle and comorbidity covariates.

Each 10 beats per minute higher RHR was associated with higher all-cause mortality (primary cohort: HR, 1.26; 95% CI 1.07 to 1.50; validation: HR, 1.17; 95% CI 1.12 to 1.22). In the primary cohort most deaths (99 of 120) were non-CVD; the CVD-specific analysis was underpowered (21 deaths; HR, 1.15; 95% CI 0.77 to 1.71). In validation, RHR predicted non-CVD (HR, 1.17; 95% CI 1.11 to 1.23) and CVD (HR, 1.18; 95% CI 1.08 to 1.29) mortality about equally. The association strengthened with age, emerging by the early-to-mid 30 s in both cohorts, before the starting age of current risk tools. HRR, measured under submaximal conditions, was not associated with any outcome.

In young US adults, elevated resting heart rate, a free and routinely measured vital sign, predicts long-term mortality across two cohorts, marking broad survival vulnerability rather than cardiovascular risk alone. Detectable in the early-to-mid 30 s, below the starting age of current cardiovascular risk tools, resting heart rate may flag at-risk young adults before these tools apply. Submaximal HRR carried no prognostic value.

Avacopan, an oral C5a receptor antagonist, is approved for treatment of severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in combination with rituximab or cyclophosphamide. This study evaluated its real-world efficacy, safety and glucocorticoid-sparing effects.

This retrospective single-centre study included 35 patients with new-onset or relapsing AAV treated with avacopan plus standard induction therapy (rituximab, cyclophosphamide or both). Outcomes were compared with 70 matched controls receiving standard therapy. Primary endpoints were remission (Birmingham Vasculitis Activity Score (BVAS)=0, prednisolone ≤5 mg/day) at 6 and 12 months. Secondary endpoints included relapse, renal function, glucocorticoid exposure and adverse events.

Baseline characteristics were generally comparable, although median estimated glomerular filtration rate (eGFR) was lower in the avacopan group (24 vs 49 mL/min). Induction therapy included rituximab (28%), cyclophosphamide (11%) or combination therapy (61%), reflecting severe disease. Remission rates were similar at 6 months (69% vs 68%) but tended to be higher with avacopan at 12 months (86% vs 68%; p=0.11). Relapses occurred less frequently with avacopan (16% vs 51%, p=0.003), including after treatment discontinuation. Avacopan was associated with accelerated glucocorticoid tapering (≤5 mg/day; 102 vs 164 days, p<0.001), lower cumulative doses (3266 vs 4288 mg, p=0.008) and greater renal function improvement (ΔeGFR+18 vs +7 mL/min after 1 year). Nine patients (26%) discontinued avacopan due to adverse events.

In a real-world practice, avacopan was effective with an acceptable safety profile, enabling steroid sparing, improved renal recovery and reduced relapse rates, including in patients receiving combined induction therapy. Benefits persisted beyond 12 months, but prospective confirmation of these presumed long-term benefits is needed.

To evaluate the diagnostic pathway of patients affected by large vessel vasculitis (LVV) and to determine factors associated with the time to diagnosis including referring and previously consulted medical specialties.

This retrospective single-centre study enrolled patients diagnosed with giant cell arteritis (GCA) and Takayasu arteritis (TAK) between January 2014 and May 2025. Clinical, laboratory and referral data were retrieved from medical records. Time to diagnosis was defined as the interval between the onset of first LVV-related symptoms and the confirmed diagnosis. Factors associated with diagnostic delay were analysed by multivariate Cox regression. Temporal differences were visualised using Kaplan-Meier curves.

In total, 501 patients were included (GCA n=488; TAK n=13). Compared with GCA (89 days (IQR 31-184)), the median time to diagnosis for TAK (245 days (IQR 31-1308)) was longer. Presentation with typical cranial symptoms was associated with a shorter diagnostic interval (HR 1.27, p=0.022), involvement of the aorta and its major branches was linked to prolonged delay (HR 0.64, p<0.001). Prior medical consultations by rheumatologists were associated with longer diagnostic delay (HR 0.73, p=0.004), whereas referrals by ophthalmologists (HR 2.40, p=0.001) and cardiologists (HR 3.81, p=0.008) were associated with earlier diagnosis.

Diagnostic delay varies by subtype and clinical presentation. Time to diagnosis was longer in TAK compared with GCA, whereas typical cranial symptoms facilitate earlier diagnosis. Increasing awareness is crucial to minimise diagnostic delay and prevent organ damage.

Psoriatic arthritis (PsA) is a chronic inflammatory disease characterised by joint involvement, significant impact on quality of life and frequently linked with obesity, metabolic syndrome and cardiovascular comorbidities. Despite advances in PsA management, obesity remains a critical factor influencing disease severity, treatment response and patient outcomes. We examine the interplay between PsA and obesity, the current clinical guidelines, gaps in clinical practice, research on shared inflammatory pathways, treatment implications and the potential of weight-loss interventions to modify disease progression. This comprehensive narrative review uses a non-systematic search of PubMed, Embase and Scopus databases and analyses literature on PsA, obesity, cytokine signalling and therapeutic outcomes, with priority to clinical trials, meta-analyses and large cohort studies. Obesity exacerbates PsA through systemic inflammation, adipokine dysregulation and mechanical joint stress, contributing to worse clinical outcomes, increased comorbidity and poorer response to biologics. Weight management improves disease activity and therapeutic efficacy, although its effects on PsA pathophysiology remain underexplored. Our review highlights that obesity should be recognised as a risk factor and comorbidity in PsA for the integral management of patients. Further research is needed to elucidate the cytokine and molecular changes induced by weight management, generate more clinical data and optimise personalised management strategies. While clinical guidelines recommend a coordinated care approach to optimising the management of PsA in patients with obesity, emerging clinical data support further focus on the role of targeting inflammation, obesity and metabolic dysfunction to improve long-term PsA outcomes and quality of life, mitigate complications and provide better cardiovascular and overall health to patients.