ESC Heart Failure最新文献

{"title":"Diagnostic value of computed tomography-based muscle quality and metabolic signatures in heart failure with sarcopenia.","authors":"Wataru Kawaharata, Hidemichi Kouzu, Keishi Ogura, Toshiyuki Yano, Satoshi Katano, Ryo Numazawa, Ryohei Nagaoka, Hiroki Aida, Katsuhiko Ohori, Ryo Nishikawa, Takefumi Fujito, Nobutaka Nagano, Marenao Tanaka, Masato Furuhashi","doi":"10.1002/ehf2.15371","DOIUrl":"https://doi.org/10.1002/ehf2.15371","url":null,"abstract":"<p><strong>Aims: </strong>Sarcopenia is a major comorbidity in heart failure (HF) patients, but its comprehensive evaluation remains challenging due to resource limitations. Computed tomography (CT) imaging allows opportunistic assessment of both muscle quantity and quality; however, its diagnostic utility and relationships with physical performance and underlying metabolic alterations remain unclear. We investigated the diagnostic utility of single-slice abdominal CT for sarcopenia assessment and explored its association with plasma amino acid profiles in HF patients.</p><p><strong>Methods and results: </strong>We retrospectively analysed 212 HF patients (73 ± 13 years old; 41% females) who underwent dual-energy X-ray absorptiometry (DXA), abdominal CT imaging, and amino acid profiling, of whom 186 had complete sarcopenia assessment. Sarcopenia was diagnosed based on the Asian Working Group for Sarcopenia 2019 criteria using DXA-measured appendicular skeletal muscle mass index and grip strength, with severe sarcopenia additionally requiring low Short Physical Performance Battery scores. CT-measured skeletal muscle area index (SMI) at the lumbar vertebral level combined with grip strength showed high diagnostic accuracy for sarcopenia [area under the receiver operating characteristic curve (AUC): 0.96 for males, 0.88 for females], with optimal cut-off values of 54 cm²/m² for males and 40 cm²/m² for females. SMI was similarly reduced in non-severe and severe sarcopenia patients, whereas mean muscle attenuation (MMA), an index of intramuscular lipid infiltration, was decreased exclusively in patients with severe sarcopenia. In multivariable analysis adjusted for age, sex, HF functional class and diabetes, decreased MMA remained the only CT-measured index significantly associated with low physical performance. MMA showed higher predictive value than SMI for severe sarcopenia in males (AUC 0.81 vs. 0.54, P = 0.039). Several amino acids (leucine, β-alanine and 3-methylhistidine) and the Fischer ratio were significantly associated with MMA, independent of SMI and established MMA determinants including sex, age, and body mass index.</p><p><strong>Conclusions: </strong>Single-slice abdominal CT provides accurate sarcopenia diagnosis in HF patients with reduced muscle strength, with muscle quality rather than quantity determining physical performance. Specific amino acid profiles associated with muscle quality changes suggest potential therapeutic targets for preventing sarcopenia progression.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144764785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of intra-abdominal pressure and point of care ultrasound to guide decongestive therapies in acute heart failure.","authors":"C Josa-Laorden, A Campos-Saenz de Santamaría, S Crespo-Aznarez, J Pérez-Silvestre, E Montero-Hernandez, P Llacer-Iborra, J Torres-Macho, M Méndez-Bailon, J L Morales-Rull, P Salamanca-Bautista, N Fernández-Villa, I Torres-Courchoud, M A Vázquez-Ronda, R Martínez-Gutiérrez, P Serrano-Irigoyen, N García-Lorente, J C Trullas, M Cobo-Marcos, M J Pinilla, M Sánchez-Marteles, J Rubio-Gracia","doi":"10.1002/ehf2.15380","DOIUrl":"https://doi.org/10.1002/ehf2.15380","url":null,"abstract":"<p><strong>Aims: </strong>Effective decongestion is crucial in managing acute decompensated heart failure (ADHF). Persistent congestion post-diuretic therapy correlates with adverse outcomes. This study evaluates whether a strategy guided by intra-abdominal pressure (IAP) and point-of-care ultrasound (POCUS) enhances decongestion compared to standard diuretic titration.</p><p><strong>Methods and results: </strong>ABDOPOCUS-HF is a randomized, multicentre, open-label, pragmatic clinical trial involving 168 patients hospitalized with ADHF across 14 Spanish hospitals. Inclusion criteria encompass clinical signs of congestion and elevated natriuretic peptides (NT-proBNP >1000 pg/mL or BNP > 250 pg/mL). Participants are randomized 1:1 to either standard care or an intervention arm where diuretic therapy is guided by baseline IAP measurements and POCUS assessments, including lung ultrasound, inferior vena cava diameter and VExUS score. The primary endpoint is the resolution of systemic congestion at 72 h, measured by the ADVOR score. Secondary endpoints include changes in pulmonary congestion (B-lines), intravascular congestion (VExUS and IVC), biomarkers (NT-proBNP and CA125), total diuretic dose, diuretic response, hospital length of stay and rates of cardiovascular death, rehospitalization and need for intravenous diuretics at 30 and 90 days. Safety endpoints encompass worsening renal function, electrolyte disturbances and catheter-related infections.</p><p><strong>Conclusions: </strong>The ABDOPOCUS-HF trial investigates whether integrating IAP and POCUS into decongestion strategies improves diuretic response and clinical outcomes in ADHF patients. Findings may inform future protocols for volume management in acute heart failure.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144752667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patient-specific modifiers of survival benefit in cardiac resynchronization therapy - A multicentre interaction analysis.","authors":"Bert Vandenberk, Marius Brusselmans, Gabor Voros, Pieter Martens, Sebastian Ingelaere, Pascal Betschart, Jens-Uwe Voigt, Matthias Dupont, Alexander Breitenstein, Jan Steffel, Rik Willems, Frank Ruschitzka, Wilfried Mullens, Sander Trenson, Stephan Winnik","doi":"10.1002/ehf2.15367","DOIUrl":"https://doi.org/10.1002/ehf2.15367","url":null,"abstract":"<p><strong>Background: </strong>Cardiac resynchronization therapy (CRT) is a key intervention for patients with heart failure. The choice between a CRT with defibrillator therapy (CRT-D) and a CRT with pacemaker (CRT-P) is influenced by individual clinical characteristics. This study explores the interaction between these clinical variables and the benefit of CRT-D versus CRT-P on all-cause mortality.</p><p><strong>Methods: </strong>All patients who underwent CRT implantation in three European centres were included in a multicentre, retrospective registry. The impact of clinical variables on all-cause mortality was analysed using interaction tests within multivariable Cox proportional hazard models. Significant interactions were explored to assess how patient characteristics modify the effect of CRT-D compared with CRT-P.</p><p><strong>Results: </strong>A total of 2271 patients with CRT implantation were included. CRT-D was associated with a 35% reduction in all-cause mortality compared with CRT-P [hazard ratio (HR) 0.65; 95% confidence interval (CI) 0.53-0.80]. Significant interactions were observed for left bundle branch block (LBBB) morphology (P = 0.028), left ventricular ejection fraction (LVEF, P = 0.025) and renal function (P = 0.019). The survival benefit of CRT-D was pronounced in patients with LBBB (HR 0.57; 95% CI 0.44-0.73) but was not significant in those without LBBB (HR 0.81; 95% CI 0.59-1.10). For LVEF at implant, CRT-D provided benefit between 17.9% and 37.6%. Similarly, CRT-D improved outcomes in patients with an estimated glomerular filtration rate >31.8 mL/min but not in those with more advanced renal impairment. No interaction was observed with age at implant (P = 0.286).</p><p><strong>Conclusions: </strong>This study provides insights into the benefits of CRT-D over CRT-P, identifying LBBB morphology, LVEF and renal function as key covariates associated with implantable cardioverter-defibrillator (ICD) therapy's benefit.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144741704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intense optimization of oral therapy rapidly restores respiratory function in worsening heart failure patients.","authors":"Hajer Yaakoubi, Lotfi Boukadida, Marwa Toumia, Anaïs Caillard, Houda Ben Soltane, Rahma Jaballah, Imen Trabelsi, Randa Dhaoui, Asma Zorgati, Hamdi Boubaker, Meriem Khrouf, Zied Mezgar, Houda Ben Salah, Alexandre Mebazaa, Benjamin Deniau, Semir Nouira, Riadh Boukef","doi":"10.1002/ehf2.15373","DOIUrl":"https://doi.org/10.1002/ehf2.15373","url":null,"abstract":"<p><strong>Aims: </strong>Breathlessness is the primary symptom of decompensated heart failure (HF), but the prevalence and evolution of persistent respiratory distress post-hospitalization remain unclear.</p><p><strong>Methods and results: </strong>The Longitudinal Observational Study for mIddle Term Follow-up Patients Admitted for Acute Dyspnea in TunIsia (SIDI) study is a prospective and multicentre study on HF patients with preserved ejection fraction admitted for acute dyspnoea. Patients were followed for 6 months. The primary endpoint was to assess the incidence of persistent respiratory distress and factors linked to respiratory recovery at Day 90. The secondary endpoint was rehospitalization and/or death at Day 90 and 180. Among 231 patients, 140 had SpO₂ and respiratory rate data at Day 90. Persistent respiratory distress was observed in 97 (69%). Multivariable analysis found no association between respiratory recovery and sex, diabetes, hypertension, kidney disease or NT-proBNP levels. However, intensive follow-up (n = 54) with oral neuroendocrine inhibition significantly improved respiratory parameters at Day 90 (adjusted HR: 5.70 [95% CI 2.13-18.28], P = 0.0001) compared with standard follow-up (n = 86) and reduced rehospitalization and/or death at Day 90 and 180.</p><p><strong>Conclusions: </strong>Persistent respiratory distress is frequent in the months following HF hospitalization. Optimized guideline-directed therapy and close follow-up improve respiratory recovery. SpO₂ and respiratory rate monitoring should be integrated into HF management.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Age-adjusted natriuretic peptide thresholds for a diagnosis of heart failure in the community: Diagnostic accuracy study.","authors":"Clare J Taylor, Kathryn S Taylor, Nicholas R Jones, Jose M Ordóñez-Mena, Antoni Bayes-Genis, F D Richard Hobbs","doi":"10.1002/ehf2.15383","DOIUrl":"https://doi.org/10.1002/ehf2.15383","url":null,"abstract":"<p><strong>Background: </strong>European Society of Cardiology (ESC) chronic heart failure (HF) guidelines recommend a single N-terminal pro-B-type natriuretic peptide (NT-proBNP) threshold of ≥125 pg/mL for specialist referral in symptomatic patients; however, natriuretic peptide levels increase with age.</p><p><strong>Objectives: </strong>We aimed to assess NT-proBNP test performance at age-adjusted thresholds recently proposed by the ESC Heart Failure Association (HFA).</p><p><strong>Methods: </strong>Diagnostic accuracy study using linked primary and secondary care data (2004-2018) in England. NT-proBNP test performance at ESC HFA age-adjusted rule-in thresholds (≥125 pg/mL, ≥250 pg/mL and ≥500 pg/mL for <50 years, 50-74 years and ≥75 years, respectively) and a high-risk threshold (≥2000 pg/mL) was assessed overall, by sex and body mass index (BMI) with ESC's suggested threshold reductions for obesity.</p><p><strong>Results: </strong>Of 155 347 patients with NT-proBNP tests performed, 14 585 (9.4%) were diagnosed with HF. Current ESC single threshold of ≥125 pg/mL had sensitivity 94.6% [95% confidence interval (CI) 94.2-95.0] and specificity 50.0% (49.7-50.3). Age-adjusted thresholds had reduced sensitivity (83.5%, 88.5%, 84.4%) but increased specificity (77.6%, 67.8%, 63.5%) across the respective age groups. The high-risk threshold had sensitivity 38.9% (38.1-39.7) and specificity 96.1% (96.0-96.2). A high BMI was associated with lower sensitivity at each age-adjusted threshold, which improved with adjustment by obesity category. Test performance was similar in women and men.</p><p><strong>Conclusions: </strong>At ESC HFA age-adjusted thresholds, the number of referrals required for HF diagnostic assessment are substantially reduced, but with some (likely lower risk) cases initially being undetected. Lower thresholds for patients with obesity are needed to avoid missing HF cases, but there is no need for adjustment by sex.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728942","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prognostic value of longitudinal strain relative apical sparing in severe aortic stenosis patients undergoing TAVR.","authors":"Dan Liu, Kai Hu, Vera Schimpf, Victoria Sokalski, Friederike Hermann, Björn Daniel Lengenfelder, Georg Ertl, Stefan Frantz, Peter Nordbeck","doi":"10.1002/ehf2.15365","DOIUrl":"https://doi.org/10.1002/ehf2.15365","url":null,"abstract":"<p><strong>Aims: </strong>This study evaluated the prognostic value of the relative apical sparing pattern (RASP) of longitudinal strain (LS) in patients with severe aortic stenosis (AS) undergoing transcatheter aortic valve replacement (TAVR) and investigated whether its combination with pre-procedural biomarkers enhances risk stratification.</p><p><strong>Methods and results: </strong>This retrospective study included 598 patients (mean age 81.7 ± 5.7 years, 48.8% male) with severe AS undergoing TAVR. Two-dimensional speckle-tracking echocardiography was used to assess LS. RASP was defined as an apical-to-basal LS ratio >3.0 in ≥3 out of six left ventricular walls. The primary endpoint was 2-year cardiovascular (CV) mortality. RASP was present in 19.2% of patients and independently predicted 2-year CV mortality (hazard ratio [HR] 2.01, 95% CI 1.22-3.29, P = 0.006). Low serum albumin (<4.0 g/dL; HR 2.40, 95% CI 1.50-3.84, P < 0.001) and low BMI (≤25.5 kg/m²; HR 1.71, 95% CI 1.07-2.73, P = 0.025) were also independent predictors. A composite risk score (0-3 points) was constructed using these three factors. Two-year CV mortality increased progressively with higher scores: 6.3% for score 0, 11.4% for score 1, 27.2% for score 2 and 35.3% for score 3 (log-rank P < 0.001). High-risk patients (score ≥2) had a more than threefold increase in adjusted mortality risk (HR 3.42, 95% CI 2.14-5.48, P < 0.001).</p><p><strong>Conclusions: </strong>RASP, particularly when combined with hypoalbuminemia and low BMI, identifies a high-risk phenotype associated with adverse outcomes after TAVR. This integrated risk model may assist in guiding pre-procedural assessment and individualized management.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144717748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Circulating CD19 B cell count, myocardial injury and clinical outcomes in patients with heart failure.","authors":"Yuta Kobayashi, Yoichiro Otaki, Tetsu Watanabe, Ryuhei Yamaguchi, Hiroe Ono, Shingo Tachibana, Junya Sato, Naoaki Hashimoto, Masahiro Wanezaki, Daisuke Kutsuzawa, Takanori Arimoto, Masafumi Watanabe","doi":"10.1002/ehf2.15382","DOIUrl":"https://doi.org/10.1002/ehf2.15382","url":null,"abstract":"<p><strong>Aims: </strong>Heart failure (HF) is a steadily increasing health problem associated with a high mortality rate. Lymphocytopenia is common and reportedly associated with poor clinical outcomes in patients with HF. Alterations in circulating lymphocyte subsets have not been examined. The current study focused on the CD19 cell count, B cells and examined whether alteration of lymphocyte subsets can predict clinical outcomes in patients with HF.</p><p><strong>Methods: </strong>Three hundred ninety-five consecutive patients with HF were enrolled (mean age 73, 59.6% men). Circulating lymphocyte subset counts (CD3 cells: T cells; CD19 cells: B cells; and CD56 cells: NK cells) were evaluated. All patients were prospectively followed for a median period of 374 days. The primary and secondary endpoints were all-cause mortality and HF-related events, respectively.</p><p><strong>Results: </strong>Simple linear analysis indicated that circulating CD19 B cell counts negatively correlated with heart-type fatty acid-binding protein levels (r = -0.3669; P < 0.0001). The C-index of the CD19 B cell count for all-cause mortality was the highest among the lymphocyte subset counts (C-index 0.73085 vs. 0.69063, 0.65312, 0.60117). Multivariate Cox proportional hazard regression analysis demonstrated that the CD19 B cell count was an independent predictor of all-cause mortality and HF-related events after adjusting for confounding risk factors [hazard ratio (HR) 0.57; confidence interval (CI) 0.45-0.71; P < 0.0001 for all-cause mortality; HR 0.79; CI 0.64-0.98; P = 0.0293 for HF-related events], but not for other subset counts. Adding the CD19 B cell count to the basic risk factors significantly improved the C-index for all-cause mortality, with a significant net reclassification index and integrated discrimination improvement (C-index 0.8000 vs. 0.7609; P = 0.0256).</p><p><strong>Conclusions: </strong>Circulating CD19 B cell counts correlated with myocardial injury and could be a feasible marker for clinical outcomes in patients with HF.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144689655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coronin 1 deficiency protects from the development of autoimmune myocarditis by reducing CD4+ T cells.","authors":"Amanda Ochoa-Espinosa, Lucile Genty, Lifen Xu, Asli Akin, Katharina Glatz, Sarah Decembrini, Christian Mueller, Albert Neutzner, Rajesh Jayachandran, Felix Mahfoud, Jean Pieters, Beat A Kaufmann","doi":"10.1002/ehf2.15384","DOIUrl":"https://doi.org/10.1002/ehf2.15384","url":null,"abstract":"<p><strong>Aims: </strong>Dilated cardiomyopathy (DCM) caused by viral myocarditis and autoimmune processes is a frequent cause for heart failure. CD4+ T cells are indispensable for autoimmune myocarditis. Coronin 1 is required for peripheral T cell survival. We therefore hypothesized that deficiency in coronin 1 protects mice from experimental autoimmune myocarditis (EAM).</p><p><strong>Methods: </strong>EAM was induced in coronin 1-deficient and wild-type (WT) mice. WT CD4+ T cells isolated from spleens were transferred to coronin 1-deficient mice in a subset of animals; IL-10 was blocked in another subset before EAM induction. On day 21 mice underwent echocardiography and were sacrificed. Myocarditis severity was scored (Grades 0-4) on histology. Leukocyte fractions in blood and heart tissue were characterized. Plasma cytokines including interleukin (IL)-10 were measured and RNA sequencing of myocardial tissue was performed.</p><p><strong>Results: </strong>The severity of myocarditis was lower in coronin 1-deficient versus WT mice [median 0 (25th-75th percentile 0-2) vs. 4 (3-4), P < 0.0001]. Coronin 1-deficient animals showed significant reductions of inflammatory cells in the myocardium [median 2.5% (25th-75th percentile 1.5%-7.0%) vs. 28.3% (14.5%-54.8%), P < 0.0001]. IL-10 was selectively increased in the plasma of coronin 1-deficient mice [median 3.0 pg/mL (25th-75th percentile 1.3-5.2 pg/mL) vs. 0.4 pg/mL (0-2.0 pg/mL), P < 0.05]. Transfer of WT CD4+ T cells but not blocking of IL-10 restored EAM. Left ventricular mass was increased, but effects of myocarditis on left ventricular function were evident only on the RNA level.</p><p><strong>Conclusions: </strong>Deficiency in coronin 1 protects from the development of EAM by reducing CD4+ T cells. This protection resulted in less structural alterations of the left ventricular myocardium. IL-10 was selectively increased in the plasma of coronin 1-deficient mice, but blocking of IL-10 was not sufficient to restore EAM.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144689656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetics, cardiac phenotype and cardiovascular outcomes in Fabry disease patients in Finland.","authors":"Kati Valtola, Päivi Pietilä-Effati, Jonna M E Männistö, Susanne Walls, Ilkka Kantola, Johanna Kuusisto","doi":"10.1002/ehf2.15387","DOIUrl":"https://doi.org/10.1002/ehf2.15387","url":null,"abstract":"<p><strong>Aims: </strong>To investigate the genetics, cardiac phenotype and cardiovascular outcomes of Finnish Fabry patients.</p><p><strong>Methods and results: </strong>Among the 109 patients with Fabry disease (FD) diagnosed in Finland by 2018, 97 (89%; 32 males and 65 females, mean ages 42 and 52 years) were followed for a mean of 12 years. Data on genetics, phenotypes, cardiac imaging and cardiovascular outcomes were collected from the Fabry Registry and medical records. The 26 families with FD harboured 22 different hemi-/heterozygous GLA variants, most commonly p.R227X, p.A143T or p.P409A. The Fabry phenotype in males was classic in 19 (59%), late-onset in 10 (31%) and intermediate in 3 (9%) patients. Among the females, 62 (95%) were symptomatic. Fabry cardiomyopathy (FC, maximal left ventricular wall thickness ≥13 mm, or an increased cardiac mass and decreased T1 time, or typical late gadolinium enhancement (LGE) in CMR) was present in 21 (66%) males manifesting since their 20s, and in 32 (49%) females since their 40s. LGE in CMR was detected in most subjects with cardiomyopathy, particularly in females. Among the 53 patients with FC, 16 (30%) developed atrial fibrillation, 17 (32%) stroke, 14 (26%) heart failure (HF) and 3 (6%) end-stage renal disease. Nine patients died during the follow-up at mean ages of 48 (males) and 75 years (females), three of whom died from HF and three from stroke. Eight of those who died had cardiomyopathy.</p><p><strong>Conclusions: </strong>In Finland, FD is caused by multiple GLA variants. Classic phenotype is more common. Contrasting previous studies, most women are symptomatic. Cardiomyopathy is very common also in women since their 40s and associates with atrial fibrillation, HF, stroke and death, emphasizing the malignant natural course of FC. Our findings highlight the need for even more diligent monitoring of cardiac manifestations also in females with FD by regular cardiac imaging with CMR.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multidisciplinary care: A missing solution to SGLT2 inhibitor underuse in heart failure.","authors":"Wahab Khawar Siddiqui","doi":"10.1002/ehf2.15372","DOIUrl":"https://doi.org/10.1002/ehf2.15372","url":null,"abstract":"","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}