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Papillary fibroelastomas have a range of clinical presentations. The surgical removal of these tumors should always be considered as best alternative to a conservative approach. Papillary fibroelastomas have a range of clinical presentations. The surgical removal of these tumors should always be considered as best alternative to a conservative approach.

IntroductionInherited cardiac conditions, including dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM), may have a monogenic cause identified through genetic testing (GT). Confirmation of pathogenic gene variants can have important implications for the patient and their relatives. The UK National Genomic Test Directory (NGTD) provides strict criteria on the indications for GT; however, the European Society of Cardiology (ESC) recommends wider GT. We reviewed the prevalence of pathogenic genotypes in patients undergoing GT who did not meet the NGTD criteria.MethodsWe conducted a retrospective analysis of patients who underwent GT with a confirmed diagnosis of HCM or DCM attending the Essex Inherited Cardiac Conditions Clinic between January 2023 and January 2025.Results257 patients were included in the analysis, with 136 patients with DCM (52.9%) and 121 patients with HCM (47.1%). The diagnostic yield of GT was 19.9% in DCM and 17.4% in HCM.14.8% of gene-positive patients with DCM and 14.3% of gene-positive patients with HCM did not meet current UK testing criteria, predominantly due to age of onset. All gene-positive patients in the DCM subgroup not meeting current NGTD criteria for testing had evidence of myocardial fibrosis.ConclusionA significant minority of patients (1 in 7) with cardiomyopathy and a pathogenic genotype did not meet current UK testing criteria; each patient has an average of 4 first-degree relatives at risk who will benefit from predictive GT. We propose the adoption of the wider ESC guidance, removing the strict age-related cut-offs and being guided more by the severity of the phenotype, particularly involving myocardial scarring.

High resolution simulations at 4.4 km and 1.5 km resolution have been performed for 12 historical tropical cyclones impacting Bangladesh. We use the European Centre for Medium-Range Weather Forecasting 5 th generation Re-Analysis (ERA5) to provide a 9-member ensemble of initial and boundary conditions for the regional configuration of the Met Office Unified Model. The simulations are compared to the original ERA5 data and the International Best Track Archive for Climate Stewardship (IBTrACS) tropical cyclone database for wind speed, gust speed and mean sea-level pressure. The 4.4 km simulations show a typical increase in peak gust speed of 41 to 118 knots relative to ERA5, and a deepening of minimum mean sea-level pressure of up to −27 hPa, relative to ERA5 and IBTrACS data. The downscaled simulations compare more favourably with IBTrACS data than the ERA5 data suggesting tropical cyclone hazards in the ERA5 deterministic output may be underestimated. The dataset is freely available from https://doi.org/10.5281/zenodo.3600201 . Measurement(s) atmospheric wind speed • wind speed of gust • temperature of air • humidity • geopotential height • water-based rainfall • snowfall • pressure of air • air pressure at sea level • wet bulb potential temperature • net down surface SW flux • surface downwelling SW flux in air Technology Type(s) computational modeling technique Factor Type(s) spatial resolution: 4.4 km & 1.5 km Sample Characteristic - Environment atmospheric weather • atmospheric boundary layer • cyclone Sample Characteristic - Location Bangladesh • South Asia Machine-accessible metadata file describing the reported data: https://doi.org/10.6084/m9.figshare.13560560

Aims To help avoid therapeutic inertia, we developed a pragmatic treatment score (QUAD Score) for use in daily practice by healthcare professionals managing patients with a left ventricular ejection fraction (LVEF) < 50% and heart failure. We now investigate the association between achieved QUAD scores and 1 year outcomes. Methods This was a multicentre cohort study in consecutive patients with incident heart failure and LVEF <50%, who completed therapy titration between January 2021 and June 2023. The primary outcome was a composite of first hospitalization for heart failure (HHF) and all‐cause mortality at 1 year after final therapy titration, for QUAD scores that were poor (<8), good (8–14) or excellent (15–24). Results Data were analysed from 1691 participants, collected from 10 UK centres, of whom 30% were women and 82% were White. Median age, N terminal pro‐B‐type natriuretic peptide (NTproBNP) and LVEF were 70 (59–78.5) years, 1624 (536–4138) ng/L and 34 (25–38) %, respectively. At the start of therapy titration, only 97 (5%) patients were naïve to any of the four pillars of therapy. After investigator‐declared final titration, QUAD scores were excellent in 806 (48%), good in 382 (22%) and poor in 503 (30%) patients. Patients who failed eventually to achieve a good or excellent QUAD score were more often women, older and had poorer renal function and higher plasma NTproBNP (P < 0.01). The median number of days to final therapy titration was longer in those who achieved an excellent QUAD score, [174 (99–290) days,133 (80–232) days and 108 (57–193) days P < 0.01, for excellent, good and poor QUAD groups, respectively. There was wide variation in titration schedules across participating centres and overall, 33% of patients completed therapy titration within 90 days, 63% within 6 months and 88% within 1 year. The primary composite outcome at 1 year for those with poor, good and excellent QUAD scores were respectively 16.9%, 9.4% and 5.6%, (log rank P < 0.01), for mortality were 13.1%, 6.5% and 2.4% (log rank P < 0.001) and for first HHF were 7.7%, 3.9% and 3.2% (log rank P < 0.001). Conclusions The QUAD score is a simple tool that can help audit and incentivize uptake of guideline‐recommended therapy for HFrEF and prevent treatment inertia. Excellent QUAD scores are associated with better outcomes. The QUAD score was developed to promote adherence to clinical practice guidelines for patients with heart failure and a reduced left ventricular ejection fraction <50%. In newly diagnosed patients who had completed therapy titration, we found that patients with an excellent QUAD score had a significant reduction in the risk of mortality or hospitalisation for heart failure at 1 year.

IntroductionN-terminal-pro hormone of Brain Natriuretic Peptide (NT-proBNP) is a sensitive rule-out test for new-onset acute heart failure (HF) with a high negative predictive value. However, it is not specific and can be influenced by various factors including cardiac (e.g. ACS, arrhythmia) and non-cardiac (advancing age and co-morbidities such as chronic kidney disease and anaemia). These factors must be taken into account when utilising NT-proBNP as a screening tool.Due to the high demand on echocardiography services, the British Society of Echocardiography (BSE) have recommended a triage criteria for inpatient echocardiography requests, including an age-specific NT-proBNP concentration cut-off for those with suspected HF.Our aim was to evaluate the effectiveness and safety of using these BSE recommendations for the inpatient diagnosis of HF when triaging echocardiography requests, and their potential impact on the inpatient demand for echocardiography.MethodWe retrospectively analysed and compared NT-proBNP results and inpatient echocardiogram findings reported between 1st and 15th March 2024 at two centres in the East of England. Patients were divided into the three BSE NT-proBNP cut-off levels: ≥ 450 ng/l in those under 50 years of age, ≥ 900 ng/l for those between 50 and 75 years of age (inclusive), and ≥ 1800 ng/l for those over 75 years of age, for comparison.Results159 patient records were analysed to determine if their NT-proBNP concentration met the BSE criteria to trigger an inpatient echocardiogram. The mean age was 80±13.04 years. 47.8% (76) were women. 100 (62.9%) patients had an NT-proBNP concentration that met the BSE criteria. 65 (65%) of these had an inpatient echocardiogram. Of the remaining 59 (37.1%) who did not meet the criteria, 49.2% (29) had an echocardiogram.Of those who met the criteria and had an echocardiogram, 39 (60%) were diagnosed with HF. Of those who did not meet the criteria and had an echocardiogram, 9 (30.4%) were diagnosed with HF.Abstract 5-018 Table 1Results summary Number of Patients Met BSE criteria for inpatient TTE (n=100) Did not meet BSE criteria for inpatient TTE (n=59) P value Had Echo 65 (65%) 29 (49.2%) Positive HF Diagnosis 39 (60%) 9 (30.4%) 0.014 Numbers (%)A review of these nine cases found that in 7 there were valid reasons other than elevated NT-proBNP for these requests, including strong clinical suspicion of HF based on clinical presentation, new acute coronary syndrome presentation and ventricular tachycardia. Allowances in the BSE criteria mean that these patients would still have qualified for an inpatient echocardiogram despite a below-threshold NT-proBNP. In two patients with a reportedly abnormal echocardiogram, a subsequent cardiac MRI was normal. No cases of HF were, therefore, truly missed.Abstract 5-018 Table 2Diagnosis after review of the nine cases who would not have met BSE age-specific NT-proBNP criteria Number of patients Reasons 3 Known HF on treatment 2 Subsequent inpatient Cardiac MRI was normal 1 Duplicate patient 1 Acute Coronary Syndrome 1 New diagnosis of HF with clinical presentation highly suggestive of HF 1 Ventricular Tachycardia ConclusionsWith a sensitivity and specificity of 81% and 43% respectively, our data suggests that the BSE’s age-related NT-proBNP criteria for inpatient echocardiography provides a safe and viable framework for triaging inpatient requests towards a diagnosis of HF. This approach could reduce the burden on over stretched services, allowing resources to be directed more appropriately.

IntroductionHypertrophic cardiomyopathy (HCM) is a heart muscle disease with few targeted therapies. Patients with left ventricular outflow tract obstruction (HOCM) are at risk of sudden cardiac death and may experience symptoms of dyspnoea, fatigue, dizziness and palpitations. As a result, the burden of symptoms often has a significantly detrimental effect on activities of daily living, exercise tolerance and subsequently results in a reduction in quality of life. HOCM patients have been treated historically with limited medical therapy options (beta-blockers and/or calcium channel blockers or Disopyramide) before proceeding to high-risk invasive treatments (alcohol septal ablation or myomectomy). There is a substantial unmet need in HCM for specific treatments to reduce obstruction and improve other parameters of left ventricular function. Mavacamten, a first-in-class, small molecule, selective allosteric inhibitor of cardiac myosin ATPase, is a new treatment for HOCM, but not routinely available in the UK pending marketing authorisation expected in Q4 2022.MethodsIn anticipation of a dedicated Inherited Cardiac Conditions service for the Essex region, patients attending a general cardiology clinic with HCM were screened, to determine potential eligibility for Mavacamten. Criteria described in the double-blind, randomised multicentre Phase 3 EXPLORER-HCM study were analysed: symptomatic New York Heart Association (NYHA) class II and III; LVEF 355%; and Left Ventricular Outflow Tract (LVOT) peak gradient 350 mmHg at rest or with provocation.ResultsA total of 92 HCM patients were identified, with median follow up period 52 months, median age 56 years (range 15–86), and male sex in 64%. Twelve patients had an implantable cardioverter defibrillator (ICD, 13%), 3 patients had a dual chamber pacemaker (3%) and 1 patient underwent surgical myomectomy. Maximum wall thickness (MWT) ranged from 1.2cm to 3.3cm with median LVOT gradient 9 mmHg (IQR 5–21, maximum 135 mmHg). Thirteen patients (14%) met eligibility criteria for Mavacamten and of these 62% were on a beta-blocker and 15% were on Disopyramide. Mavacamten eligible HCM patients were of similar age to ineligible patients (median 56 years for both groups, p= 0.862), with similar wall thickness (median 1.7cm for both groups, p = 0.373) and LVEF (67% vs 66%, p = 0.471), but had significantly higher LVOT gradient (median 71 mmHg vs 7 mmHg, p <0.001) and worse symptoms (92% vs 34% NYHA class 2/3, p = 0.034). Despite this, there was similar survival (p = 0.57), with a trend towards better survival in Mavacamten eligible patients compared to Mavacamten ineligible patients under proposed criteria (Figure 1).ConclusionTreatment options for HCM are limited; the cardiac myosin inhibitor Mavacamten is an exciting new therapy, but it is currently unavailable in the UK. A small minority of our HCM patients meet the proposed criteria for Mavacamten, and within our cohort these patients do not have significantly reduced survival on current therapy. Given the mechanism of action of Mavacamten, further studies in all HCM patients, with or without obstructive physiology, are needed to expand potential licensing indications.Conflict of InterestNone to declare

For patients with heart failure, reduced left ventricular ejection fraction and iron deficiency, intravenous ferric carboxymaltose administration improves quality of life and exercise capacity in the short-term and reduces hospital admissions for heart failure up to 1 year. We aimed to evaluate the longer-term effects of intravenous ferric derisomaltose on cardiovascular events in patients with heart failure. IRONMAN was a prospective, randomised, open-label, blinded-endpoint trial done at 70 hospitals in the UK. Patients aged 18 years or older with heart failure (left ventricular ejection fraction ≤45%) and transferrin saturation less than 20% or serum ferritin less than 100 μg/L were eligible. Participants were randomly assigned (1:1) using a web-based system to intravenous ferric derisomaltose or usual care, stratified by recruitment context and trial site. The trial was open label, with masked adjudication of the outcomes. Intravenous ferric derisomaltose dose was determined by patient bodyweight and haemoglobin concentration. The primary outcome was recurrent hospital admissions for heart failure and cardiovascular death, assessed in all validly randomly assigned patients. Safety was assessed in all patients assigned to ferric derisomaltose who received at least one infusion and all patients assigned to usual care. A COVID-19 sensitivity analysis censoring follow-up on Sept 30, 2020, was prespecified. IRONMAN is registered with ClinicalTrials.gov, NCT02642562. Between Aug 25, 2016, and Oct 15, 2021, 1869 patients were screened for eligibility, of whom 1137 were randomly assigned to receive intravenous ferric derisomaltose (n=569) or usual care (n=568). Median follow-up was 2·7 years (IQR 1·8–3·6). 336 primary endpoints (22·4 per 100 patient-years) occurred in the ferric derisomaltose group and 411 (27·5 per 100 patient-years) occurred in the usual care group (rate ratio [RR] 0·82 [95% CI 0·66 to 1·02]; p=0·070). In the COVID-19 analysis, 210 primary endpoints (22·3 per 100 patient-years) occurred in the ferric derisomaltose group compared with 280 (29·3 per 100 patient-years) in the usual care group (RR 0·76 [95% CI 0·58 to 1·00]; p=0·047). No between-group differences in deaths or hospitalisations due to infections were observed. Fewer patients in the ferric derisomaltose group had cardiac serious adverse events (200 [36%]) than in the usual care group (243 [43%]; difference –7·00% [95% CI –12·69 to –1·32]; p=0·016). For a broad range of patients with heart failure, reduced left ventricular ejection fraction and iron deficiency, intravenous ferric derisomaltose administration was associated with a lower risk of hospital admissions for heart failure and cardiovascular death, further supporting the benefit of iron repletion in this population. British Heart Foundation and Pharmacosmos.

The management of heart failure with a reduced ejection fraction is a true success story of modern medicine. Evidence from randomised clinical trials provides the basis for an extensive catalogue of disease-modifying drug treatments that improve both symptoms and survival. These treatments have undergone rigorous scrutiny by licensing and guideline development bodies to make them eligible for clinical use. With an increasing number of drug therapies however, it has become a complex management challenge to ensure patients receive these treatments in a timely fashion and at recommended doses. The tragedy is that, for a condition with many life-prolonging drug therapies, there remains a potentially avoidable mortality risk associated with delayed treatment. Heart failure therapeutic agents have conventionally been administered to patients in the chronological order they were tested in clinical trials, in line with the aggregate benefit observed when added to existing background treatment. We review the evidence for simultaneous expedited initiation of these disease-modifying drug therapies and how these strategies may focus the heart failure clinician on a time-defined smart goal of drug titration, while catering for patient individuality. We highlight the need for adequate staffing levels, especially heart failure nurse specialists and pharmacists, in a structure to provide the capacity to deliver this care. Finally, we propose a heart failure clinic titration schedule and novel practical treatment score which, if applied at each heart failure patient contact, could tackle treatment inertia by a constant assessment of attainment of optimal medical therapy.

Dilated cardiomyopathy (DCM) is a heterogenous group of disorders characterised by left ventricular dilatation and dysfunction, in the absence of factors affecting loading conditions such as hypertension or valvular disease, or significant coronary artery disease. The prevalence of idiopathic DCM is estimated between 1:250 and 1:500 individuals. Determining the aetiology of DCM can be challenging, particularly when evaluating an individual and index case with no classical history or investigations pointing towards an obvious acquired cause, or no clinical clues in the family history to suggest a genetic cause. We present a family affected by DCM associated with Filamin C variant, causing sudden cardiac death at a young age and heart failure due to severe left ventricular impairment and myocardial scarring. We review the diagnosis and treatment of DCM, its genetic associations and potential acquired causes. Thorough assessment is mandatory to risk stratify and identify patients who may benefit from primary prevention implantable cardioverter defibrillator therapy according to international guidelines. Genetic testing has some limitations, and is positive in only 20%–35% of DCM, but should be considered in specific cases to identify families who may benefit from cascade screening after appropriate counselling. The management of often complex familial cardiomyopathy requires specialist input for every case, and the appropriate infrastructure to coordinate investigations.

BackgroundIntravenous iron therapy with ferric derisomaltose (FDI) has been shown to improve outcomes in patients with heart failure with reduced ejection fraction (HFrEF) and iron deficiency. However, its effects across different age groups remain unclear. This analysis of the Effectiveness of Intravenous Iron Treatment versus Standard Care in Patients with Heart Failure and Iron Deficiency (IRONMAN) trial explored the efficacy and safety of FDI across age groups.MethodsThe IRONMAN trial was a prospective, open-label, blinded end point randomised controlled trial enrolling patients with HFrEF and iron deficiency. This prespecified analysis stratified the population into four quarters by age group: <67 years, 67–73 years, 74–79 years, >79 years. The primary outcome was a composite of recurrent heart failure hospitalisations and cardiovascular death. Secondary outcomes included changes in haemoglobin and quality of life. Clinical outcomes comparing FDI versus usual care in each age subgroup were analysed by the method of Lin et al for recurrent events and Cox proportional hazards model for time to first event. Interactions between age and treatment effects were explored.ResultsAmong 1137 randomised patients (median age 73 years), the primary outcome rate ratio (FDI vs usual care) was 0.87 (95% CI 0.61 to 1.23) in patients <67 years, 0.93 (95% CI 0.66 to 1.32) in those aged 67–73 years, 0.88 (95% CI 0.59 to 1.33) in those aged 74–79 years and 0.66 (95% CI 0.45 to 0.96) in those aged >79 years (p-interaction=0.38). Improvements in haemoglobin and quality of life scores at 4 months did not differ statistically across age groups (p-interaction=0.92 and 0.64, respectively). Older patients were more symptomatic at baseline, with higher N-terminal-pro B-type natriuretic peptide levels and poorer renal function, but safety outcomes did not differ across age groups.ConclusionsWe found no evidence that the effects of FDI on heart failure hospitalisations, cardiovascular death, haemoglobin and quality of life differed by age. These findings support its use in patients with HFrEF and iron deficiency, including older adults.Trial registration number NCT02642562.