Fishing and environment drive spatial heterogeneity in celtic sea fish community size structure

The Large Fish Indicator (LFI) is a univariate size-based indicator of fish community state that has been selected to support the OSPAR fish community Ecological Quality Objective (EcoQO). To operate this EcoQO, a survey-based LFI for each OSPAR region needs to be developed. However, fish communities in these regions are spatially heterogeneous, and there is evidence of within-region spatial variation in the LFI that could confound an overall indicator series. For Celtic Sea trawl-survey sites, spline correlograms indicate positive spatial autocorrelation at a similar range (similar to 40 km) for the LFI and for fishing effort (h year(-1)) from vessel monitoring systems. Statistical models reveal a strong negative effect on annual LFI by site of fishing effort within a radius of 40 km. There was a weak effect of fishing within 20 km and no effect at 10 km. LFI also varied significantly with substratum and with local fish community composition identified by a resemblance matrix derived from the survey data. Finally, there was a weak effect of survey year on LFI. Spatial stratification of LFI calculations may be necessary when developing size-based indicators for OSPAR or Marine Strategy Framework Directive regions

Fishing and environment drive spatial heterogeneity in celtic sea fish community size structure

The Large Fish Indicator (LFI) is a univariate size-based indicator of fish community state that has been selected to support the OSPAR fish community Ecological Quality Objective (EcoQO). To operate this EcoQO, a survey-based LFI for each OSPAR region needs to be developed. However, fish communities in these regions are spatially heterogeneous, and there is evidence of within-region spatial variation in the LFI that could confound an overall indicator series. For Celtic Sea trawl-survey sites, spline correlograms indicate positive spatial autocorrelation at a similar range (similar to 40 km) for the LFI and for fishing effort (h year(-1)) from vessel monitoring systems. Statistical models reveal a strong negative effect on annual LFI by site of fishing effort within a radius of 40 km. There was a weak effect of fishing within 20 km and no effect at 10 km. LFI also varied significantly with substratum and with local fish community composition identified by a resemblance matrix derived from the survey data. Finally, there was a weak effect of survey year on LFI. Spatial stratification of LFI calculations may be necessary when developing size-based indicators for OSPAR or Marine Strategy Framework Directive regions

A novel risk model for predicting potentially life-threatening arrhythmias in non-ischemic dilated cardiomyopathy (DCM-SVA risk)

BACKGROUND: Non-ischemic dilated cardiomyopathy (DCM) can be complicated by sustained ventricular arrhythmias (SVA) and sudden cardiac death (SCD). By now, left-ventricular ejection fraction (LV-EF) is the main guideline criterion for primary prophylactic ICD implantation, potentially leading either to overtreatment or failed detection of patients at risk without severely impaired LV-EF. The aim of the European multi-center study DETECTIN-HF was to establish a clinical risk calculator for individualized risk stratification of DCM patients. METHODS: 1393 patients (68% male, mean age 50.7 ± 14.3y) from four European countries were included. The outcome was occurrence of first potentially life-threatening ventricular arrhythmia. The model was developed using Cox proportional hazards, and internally validated using cross validation. The model included seven independent and easily accessible clinical parameters sex, history of non-sustained ventricular tachycardia, history of syncope, family history of cardiomyopathy, QRS duration, LV-EF, and history of atrial fibrillation. The model was also expanded to account for presence of LGE as the eight8h parameter for cases with available cMRI and scar information. RESULTS: During a mean follow-up period of 57.0 months, 193 (13.8%) patients experienced an arrhythmic event. The calibration slope of the developed model was 00.97 (95% CI 0.90-1.03) and the C-index was 0.72 (95% CI 0.71-0.73). Compared to current guidelines, the model was able to protect the same number of patients (5-year risk ≥8.5%) with 15% fewer ICD implantations. CONCLUSIONS: This DCM-SVA risk model could improve decision making in primary prevention of SCD in non-ischemic DCM using easily accessible clinical information and will likely reduce overtreatment

Association of Left Ventricular Systolic Dysfunction Among Carriers of Truncating Variants in Filamin C With Frequent Ventricular Arrhythmia and End-stage Heart Failure

Importance: Truncating variants in the gene encoding filamin C (FLNCtv) are associated with arrhythmogenic and dilated cardiomyopathies with a reportedly high risk of ventricular arrhythmia. / Objective To determine the frequency of and risk factors associated with adverse events among FLNCtv carriers compared with individuals carrying TTN truncating variants (TTNtv). / Design, Setting, and Participants: This cohort study recruited 167 consecutive FLNCtv carriers and a control cohort of 244 patients with TTNtv matched for left ventricular ejection fraction (LVEF) from 19 European cardiomyopathy referral units between 1990 and 2018. Data analyses were conducted between June and October, 2020. / Main Outcomes and Measures: The primary end point was a composite of malignant ventricular arrhythmia (MVA) (sudden cardiac death, aborted sudden cardiac death, appropriate implantable cardioverter-defibrillator shock, and sustained ventricular tachycardia) and end-stage heart failure (heart transplant or mortality associated with end-stage heart failure). The secondary end point comprised MVA events only. / Results: In total, 167 patients with FLNCtv were studied (55 probands [33%]; 89 men [53%]; mean [SD] age at baseline evaluation, 43 [18] years). For a median follow-up of 20 months (interquartile range, 7-60 months), 29 patients (17.4%) reached the primary end point (19 patients with MVA and 10 patients with end-stage heart failure). Eight (44%) arrhythmic events occurred among individuals with baseline mild to moderate left ventricular systolic dysfunction (LVSD) (LVEF = 36%-49%). Univariable risk factors associated with the primary end point included proband status, LVEF decrement per 10%, ventricular ectopy (≥500 in 24 hours) and myocardial fibrosis detected on cardiac magnetic resonance imaging. The LVEF decrement (hazard ratio [HR] per 10%, 1.83 [95% CI, 1.30-2.57]; P < .001) and proband status (HR, 3.18 [95% CI, 1.12-9.04]; P = .03) remained independent risk factors on multivariable analysis (excluding myocardial fibrosis and ventricular ectopy owing to case censoring). There was no difference in freedom from MVA between FLNCtv carriers with mild to moderate or severe (LVEF ≤35%) LVSD (HR, 1.29 [95% CI, 0.45-3.72]; P = .64). Carriers of FLNCtv with impaired LVEF at baseline evaluation (n = 69) had reduced freedom from MVA compared with 244 TTNtv carriers with similar baseline LVEF (for mild to moderate LVSD: HR, 16.41 [95% CI, 3.45-78.11]; P < .001; for severe LVSD: HR, 2.47 [95% CI, 1.04-5.87]; P = .03). / Conclusions and Relevance: The high frequency of MVA among patients with FLNCtv with mild to moderate LVSD suggests that higher LVEF values than those currently recommended should be considered for prophylactic implantable cardioverter-defibrillator therapy in FLNCtv carriers

Clinical features and natural history of PRKAG2 Variant Cardiac Glycogenosis

BACKGROUND PRKAG2 gene variants cause a syndrome characterized by cardiomyopathy, conduction disease, and ventricular pre-excitation. Only a small number of cases have been reported to date, and the natural history of the disease is poorly understood. OBJECTIVES The aim of this study was to describe phenotype and natural history of PRKAG2 variants in a large multicenter European cohort. METHODS Clinical, electrocardiographic, and echocardiographic data from 90 subjects with PRKAG2 variants (53% men; median age 33 years; interquartile range [IQR]: 15 to 50 years) recruited from 27 centers were retrospectively studied. RESULTS At first evaluation, 93% of patients were in New York Heart Association functional class I or II. Maximum left ventricular wall thickness was 18 +/- 8 mm, and left ventricular ejection fraction was 61 +/- 12%. Left ventricular hypertrophy (LVH) was present in 60 subjects (67%) at baseline. Thirty patients (33%) had ventricular pre-excitation or had undergone accessory pathway ablation; 17 (19%) had pacemakers (median age at implantation 36 years; IQR: 27 to 46 years), and 16 (18%) had atrial fibrillation (median age 43 years; IQR: 31 to 54 years). After a median follow-up period of 6 years (IQR: 2.3 to 13.9 years), 71% of subjects had LVH, 29% had AF, 21% required de novo pacemakers (median age at implantation 37 years; IQR: 29 to 48 years), 14% required admission for heart failure, 8% experienced sudden cardiac death or equivalent, 4% required heart transplantation, and 13% died. CONCLUSIONS PRKAG2 syndrome is a progressive cardiomyopathy characterized by high rates of atrial fibrillation, conduction disease, advanced heart failure, and life-threatening arrhythmias. Classical features of pre-excitation and severe LVH are not uniformly present, and diagnosis should be considered in patients with LVH who develop atrial fibrillation or require permanent pacemakers at a young age. (c) 2020 the American College of Cardiology Foundation. Published by Elsevier. All rights reserved.Instituto de Salud Carlos IIIInstituto de Salud Carlos IIIEuropean Commission [PI17/01941, AC16/0014, PI17/01690, PI18/01582, PT17/0015/0043]ERA-CVD Joint Transnational Call 2016 (GENPROVIC)DETECTIN-HF project (ERA-CVD framework)Wellcome TrustWellcome TrustEuropean Commission [107469/Z/15/, HICF-R6-373]National Institute for Health Research (NIHR) Royal Brompton Cardiovascular Biomedical Research UnitNIHR Imperial Biomedical Research CentreDepartment of Health, United Kingdom [HICF-R6-373]British Heart FoundationBritish Heart Foundation [SP/10/10/28431]Obra Social La Caixa FoundationLa Caixa Foundation [100010434]Fundacio Privada Daniel Bravo AndreuInstituto de Salud Carlos III - Plan Estatal de I.D.I. 2013-2016, European Regional Development Fund ("A Way of Making Europe")Spanish Ministry of Economy and Competitiveness - Plan Estatal de I.D.I. 2013-2016, European Regional Development Fund ("A Way of Making Europe")Medical Research Council Clinical Academic Research Partnership AwardUCL Hospitals NIHR Biomedical Research CentreFondazione per la Ricerca Ospedale MaggioreNIHR Great Ormond Street Hospital Biomedical Research Centreinfo:eu-repo/semantics/publishedVersio