ESC Heart Failure最新文献

{"title":"Cardiopulmonary exercise testing as a prognosis-assessing tool in heart failure with preserved ejection fraction.","authors":"C Rozados da Conceicao, A Krannich, V Zach, R Pinto, A Deichl, A Feuerstein, L Schleussner, F Edelmann","doi":"10.1002/ehf2.15219","DOIUrl":"https://doi.org/10.1002/ehf2.15219","url":null,"abstract":"<p><strong>Aims: </strong>Patients with heart failure with preserved ejection fraction represent half of the heart failure patients nowadays, an at least steady trend due to the aging of the population. We investigated whether the parameters obtained from cardiopulmonary exercise testing (CPET) correlated with the prognosis of these patients. This prospective observational cohort study assesses the relationship between the CPET parameters peakVO₂ and VE/VCO₂ slope and the number of heart failure hospitalizations or cardiovascular death of these patients.</p><p><strong>Methods and results: </strong>From August 2016 until May 2019, 99 patients from our outpatient unit with newly diagnosed heart failure with preserved ejection fraction underwent CPET. Median follow-up was 30 months [interquartile range, 24-38.5]. We selected peakVO₂ < 14 mL/min/kg and a VE/VCO₂ slope > 34 as threshold values for our primary clinically relevant endpoint, a composite of hospitalization for heart failure or cardiovascular death. Mean age was 75.07 ± 7.31 years, 49% were women, 75% were at NYHA class II and median NTproBNP was 511 pg/mL. Mean peakVO₂ was 15.09 ± 4.75, and mean VE/VCO₂ was 36.05 ± 6.60. During follow-up, there were 207 all-cause hospitalizations, 126 cardiovascular hospitalizations, 58 heart failure hospitalizations and 4 deaths. Over a median follow-up of 30 months, the primary clinically relevant endpoint occurred in 5 of 40 patients (12.5%) with a VE/VCO₂ slope ≤ 34 and in 19 of 59 patients (32.2%) with a VE/VCO₂ slope > 34 [hazard ratio, 2.69; 95% confidence interval (CI), 1.00-7.21; P = 0.04]. On multivariate analysis, VE/VCO₂ slope was independently associated with heart failure hospitalization or cardiovascular death as a terminal event.</p><p><strong>Conclusions: </strong>In patients with heart failure with preserved ejection fraction, a VE/VCO₂ slope > 34 predicts heart failure hospitalizations and cardiovascular death.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002587","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":"Mortality risk stratification for Takotsubo syndrome: Evaluating CRP measurement alongside the InterTAK prognostic score.","authors":"Loïc Faucher, Kensuke Matsushita, Shinnosuke Kikuchi, Taraneh Tatarcheh, Benjamin Marchandot, Amandine Granier, Said Amissi, Antonin Trimaille, Laurence Jesel, Patrick Ohlmann, Kiyoshi Hibi, Valérie Schini-Kerth, Olivier Morel","doi":"10.1002/ehf2.15161","DOIUrl":"https://doi.org/10.1002/ehf2.15161","url":null,"abstract":"<p><strong>Background and objectives: </strong>Initially described as a benign acute cardiomyopathy, Takotsubo syndrome has been linked to elevated mortality rates. Emerging evidence suggests that unresolved myocardial inflammation may contribute to this adverse prognosis. This study aimed to evaluate the incremental prognostic utility of C-reactive protein (CRP) in conjunction with the InterTAK prognosis score for stratifying long-term mortality in Takotsubo syndrome.</p><p><strong>Methods: </strong>A retrospective analysis was conducted from a multicentre registry encompassing 307 patients diagnosed with Takotsubo syndrome between 2008 and 2020. Patients were stratified into quartiles based on the InterTAK prognosis score. The discriminatory potential of CRP in predicting long-term mortality was assessed. The primary endpoint was defined as all-cause mortality within 1 year.</p><p><strong>Results: </strong>A stepwise increase of CRP at discharge that corresponds to INTERTAK quartiles was observed: 9.5 mg/L (25th percentile) in the first quartile, 15.8 mg/L (median) in the second quartile, 25.3 mg/L (75th percentile) in the third quartile and 41.2 mg/L (maximum) in the fourth quartile. Receiver operating-characteristic curves analysis revealed that CRP value at discharge was predictive of 1 year mortality (area under the curve = 0.81; 95% confidence interval = 0.68-0.90) with an optimal threshold set at 33 mg/L (sensitivity: 65%; specificity: 87%). When considering the InterTAK score, the incorporation of CRP at discharge with a cut-off of 33 mg/L exhibited a significant enhancement in the prediction of 1 year mortality in 'intermediate' risk (25% vs. 1%; P = 0.008) or 'very high' risk (40% vs. 10%; P = 0.02) patients.</p><p><strong>Conclusions: </strong>In Takotsubo syndrome, the persistence of inflammatory burden at hospital discharge emerged as an independent predictor of 1 year mortality, augmenting the predictive capacity of the conventional InterTAK prognosis score.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002602","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":"Correction to 'Natriuretic peptides and C-reactive protein in in heart failure and malnutrition: A systematic review and meta-analysis'.","authors":"","doi":"10.1002/ehf2.15210","DOIUrl":"https://doi.org/10.1002/ehf2.15210","url":null,"abstract":"","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142983038","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 ADESTE trial: A phase 2 study of enibarcimab, a monoclonal antibody targeting adrenomedullin, in acute heart failure.","authors":"Anggoro Budi Hartopo, Arinal Chairul Achyar, Hendry Purnasidha Bagaswoto, Firandi Saputra, Hasanah Mumpuni, Dyah Adhi Kusumastuti, Teguh Triyono, Usi Sukorini, Metalia Puspitasari, Budi Yuli Setianto, Mohammad Saifur Rohman, Muhammad Anshory, Yoga Waranugraha, Putri Annisa Kamila, Agustin Iskandar, Hani Susianti, Andreas Bergman, Claudia Knothe, Paola Antonini, Salvatore Di Somma","doi":"10.1002/ehf2.15191","DOIUrl":"https://doi.org/10.1002/ehf2.15191","url":null,"abstract":"<p><strong>Aims: </strong>This study aimed to conduct a phase 2 proof-of-concept and safety study to evaluate the effect of ENIBARCIMAB (EN), a non-neutralizing humanized monoclonal antibody targeting the N-terminus of adrenomedullin (ADM), administered immediately after stabilization with standard of care (SoC) treatment, in patients hospitalized for acute heart failure (AHF).</p><p><strong>Methods and results: </strong>This prospective, open-label, controlled, interventional, multicenter, dose-escalation study was conducted at two cardiology sites in Indonesia. Patients were divided into two interventional groups sequentially receiving 0.5 mg/kg (SoC + EN 0.5 mg/kg, n = 10; first cohort) and 2 mg/kg (SoC + EN 2 mg/kg, n = 10; second cohort) of EN via 1-h intravenous (IV) infusion within 48 h after admission for AHF. The control group (n = 10) was treated with SoC therapy for AHF therapy. All patients were monitored continuously within 24 h post-infusion and subsequent daily until discharge. Treatment-related serious adverse events (SAEs) were recorded during hospitalization and up to 90 days after discharge. Both EN dosages were well-tolerated, and no significant safety issues were identified during hospitalization and up to 90 days of follow up. SAEs occurred in 10% of patients in each EN group but were deemed not related to treatment. No significant differences in the occurrence of SAEs were found between the groups. Five deaths occurred: three (30%) in the control group as compared with two deaths (20%) in the SoC + EN 2 mg/kg group. EN led to a significant increase in plasma bio-ADM levels within 24 h post-infusion, with the SoC + 2 mg/kg group showing the highest increase. Within 1 h from IV EN infusion, SoC + EN 2 mg/kg compared with 0.5 mg/kg, resulted in a significant percentage reduction in systolic, diastolic blood pressure, and mean arterial pressure. However, it did not result in severe hypotension and the need for drug discontinuation.</p><p><strong>Conclusions: </strong>In this pilot safety study of patients hospitalized for AHF, IV infusion of EN 0.5 and 2 mg/kg increased circulating plasma bio-ADM levels and was well-tolerated without treatment-related SAEs occurring during hospitalization and up to 90 days after discharge.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142983085","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":"Pro-adrenomedullin as an independent predictive biomarker for heart failure in atrial fibrillation and flutter.","authors":"Gaifeng Hu, Xiaodong Peng, Liu He, Yiwei Lai, Nian Liu, Xin Li, Caihua Sang, Jianzeng Dong, Changsheng Ma","doi":"10.1002/ehf2.15196","DOIUrl":"https://doi.org/10.1002/ehf2.15196","url":null,"abstract":"<p><strong>Aims: </strong>This study aimed to investigate potential biomarkers for predicting incident heart failure (HF) in patients with atrial fibrillation and flutter (AF and AFL), utilizing proteomic data from the UK Biobank Pharma Proteomics Project (UKB-PPP).</p><p><strong>Methods: </strong>This study analysed data from AF and AFL patients, split into discovery (n = 1050) and replication (n = 305) cohorts. Plasma biomarkers were screened using a multivariable-adjusted Cox proportional hazards model. Kaplan-Meier survival analysis and area under the receiver operating characteristic (ROC) curve assessments were conducted to evaluate predictive performance.</p><p><strong>Results: </strong>Over a follow-up of 14.2 years, 222 cases (21.1%) of HF were documented in the discovery cohort, while 117 cases (38.4%) occurred over 13.8 years in the replication cohort. Out of 2923 proteins measured, only pro-adrenomedullin (pro-ADM) consistently showed a significant association with incident HF in both cohorts. In the discovery cohort, each unit increase in pro-ADM was linked to an increased risk of HF (HR = 2.78, 95% CI 1.64-4.71, P < 0.001, FDR = 0.026), which was confirmed in the replication cohort (HR = 3.95, 95% CI 1.97-7.94, P < 0.001, FDR = 0.012). Kaplan-Meier analysis demonstrated that patients with higher pro-ADM levels had significantly shorter time to HF onset, with median times ranging from 2306 to 3183 days across quartiles (P < 0.001). The cumulative incidence of HF ranged from 15.3% to 42.7% across quartiles of pro-ADM (log-rank P < 0.001). Adding pro-ADM to a model with traditional risk factors, including NT-proBNP, significantly improved predictive accuracy for 3-year (AUC = 0.783; integrated discrimination improvement [IDI] = 0.010 and net reclassification index [NRI] = 0.206, both P = 0.002) and 5-year (AUC = 0.749, IDI = 0.013, NRI = 0.179, P = 0.001) risk of HF. In sensitivity analyses, the association between pro-ADM and incident HF remained consistent after excluding participants with self-reported AF and AFL, with each unit increase in pro-ADM being associated with an increased risk of HF (HR = 1.77, 95% CI 1.02-3.04, P = 0.041) and across subgroups of paroxysmal AF (HR = 2.80, 95% CI 1.11-7.07, P = 0.029) and persistent AF (HR = 4.36, 95% CI 1.41-13.43, P = 0.010).</p><p><strong>Conclusions: </strong>Pro-ADM is identified as an independent biomarker for predicting incident HF in AF and AFL patients. Its inclusion in risk prediction models enhances the ability to stratify HF risk beyond traditional biomarkers, demonstrating its potential utility in clinical practice.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142970194","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":"Clinical evaluation and outcome in heart failure patients receiving chemotherapy with different anti-cancer agents.","authors":"Tomiko Sunaga, Takahiro Okada, Yoshitaka Iso, Mio Ebato, Tsutomu Toshida, Shuichi Nawata, Hiroshi Suzuki, Mari Kogo","doi":"10.1002/ehf2.15204","DOIUrl":"https://doi.org/10.1002/ehf2.15204","url":null,"abstract":"<p><strong>Background: </strong>The optimal strategy for modern chemotherapy should be based on a comprehensive approach for cancer patients with cardiovascular diseases. Therefore, cardio-oncology has received increasing attention owing to the cardiotoxic effects of anti-cancer therapies.</p><p><strong>Objectives: </strong>We aimed to evaluate the clinical characteristics and outcomes of patients with heart failure (HF) who received chemotherapy compared with those of a matched cohort with HF who did not receive chemotherapy, using real-world HF data.</p><p><strong>Methods: </strong>This study was based on the Diagnosis Procedure Combination (DPC) database of the Japanese Registry of All Cardiac and Vascular Diseases (JROAD). We identified 1 328 113 patients who were hospitalized for HF between April 2012 and March 2021. The propensity score (PS) was estimated using a logistic regression model, with chemotherapy as the dependent variable, and a clinically score-matched analysis of 11 532 patients with HF with or without chemotherapy. The primary endpoint was readmission.</p><p><strong>Results: </strong>Colon, lung, breast and prostate cancers accounted for >60% of all cancer types. After PS matching, readmission was significantly more frequently observed in patients with chemotherapy than those without [odds ratio (OR), 1.26; 95% confidence interval (CI) 1.17-1.36, P < 0.01]. In particular, treatment with epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs) (OR, 1.69; 95% CI 1.39-2.07), taxane (OR, 2.95; 95% CI 2.11-4.12), anthracyclines (OR, 1.86; 95% CI 1.19-2.90) and fluorouracil agents (OR, 1.65; 95% CI 1.18-2.30) caused a higher risk of readmission.</p><p><strong>Conclusions: </strong>Medical providers need to monitor and follow-up patients with HF, depending on the characteristics of the anti-cancer agents and types of cancer.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142970192","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":"Ten-year follow-up of a case of eosinophilic granulomatous with polyangiitis.","authors":"Jiange Qiao, Mingjie Cao, Tao Ren, Chen Gong, Zhijun Jie, Qingmin Yang, Jindong Shi","doi":"10.1002/ehf2.15199","DOIUrl":"https://doi.org/10.1002/ehf2.15199","url":null,"abstract":"","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142970197","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":"Comprehensive exploration of unexplained dyspnoea in subjects with normal ejection fraction and low natriuretic peptides.","authors":"Emmanuelle Berthelot, Tarek Laouar, Antoine Beurnier, Nataliya Hrynchynshyn, Jean Christophe Eicher, Jean-Michel Tartière, Patrick Jourdain, Olivier Lairez, Barnabas Gellen","doi":"10.1002/ehf2.15059","DOIUrl":"https://doi.org/10.1002/ehf2.15059","url":null,"abstract":"<p><strong>Background: </strong>Unexplained exertional dyspnoea without significant elevation of natriuretic peptides is common. One of the causes might be early heart failure with preserved ejection fraction (HFpEF).</p><p><strong>Aims: </strong>This study aimed to characterize patients with exertional dyspnoea and normal/near-to-normal N-terminal pro-brain natriuretic peptide (NT-proBNP) levels with regard to early stages of HFpEF and non-cardiac causes.</p><p><strong>Method and results: </strong>Sixty-six patients (age 62 ± 7 years old, 85% women) with dyspnoea assessed using the Multidimensional Dyspnea Profile (MDP) questionnaire and NT-proBNP level of <125 pg/mL for patients <75 years old or <300 pg/mL for patients >75 years old were recruited. Patients with known significant heart disease, lung disease (abnormal respiratory function tests) or renal insufficiency stage ≥ 4 were excluded. In 11 patients (16.7%), HFpEF was confirmed according to the European Society of Cardiology Heart Failure Association (ESC HFA) criteria, 31 patients (47%) presented isolated deconditioning and 5 patients (7.6%) had idiopathic hyperventilation. In the remaining 19 patients (28.8%) with normal echocardiography and cardiopulmonary exercise testing (CPX), no objective cause of dyspnoea could be found. Compared with patients without HFpEF, those with HFpEF were older, more often hypertensive and diabetic, with higher NT-proBNP levels. They had higher E/e' ratios during exercise echocardiography and lower volume of oxygen uptake (VO₂) peaks and steeper minute ventilation (VE)/volume of carbon dioxide produced (VCO₂) slopes during CPX. Psychological impact measured on the Short Form-36 (SF-36) questionnaire was less important in HFpEF patients than in other patients.</p><p><strong>Conclusions: </strong>The most common causes of unexplained exertional dyspnoea in patients without significant elevation of natriuretic peptides are peripheral deconditioning, HFpEF and hyperventilation. Studying patients during exercise allows for getting more data about pathophysiology and improving patient phenotyping and management. Early unmasking of HFpEF using exercise echocardiography and/or CPX and initiation of treatment could prevent hospitalizations for acute heart failure. Although using exercise testing, many patients could not be classified according to their diagnosis, and this reinforces the need to better define exercise diagnostic criteria.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946766","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":"Cardiovascular toxicity induced by TKIs in patients with chronic myeloid leukaemia: Are women and men different?","authors":"Cristina Madaudo, Daniela Di Lisi, Antonio Cannatà, Federica Manfrè, Celeste Vullo, Marco Santoro, Ciro Botta, Salvatrice Mancuso, Sergio Siragusa, Alfredo Ruggero Galassi, Giuseppina Novo","doi":"10.1002/ehf2.15165","DOIUrl":"https://doi.org/10.1002/ehf2.15165","url":null,"abstract":"<p><strong>Aims: </strong>Knowledge of the effects of sex in cardio-oncology is limited, particularly in patients treated with tyrosine kinase inhibitors (TKIs) for chronic myeloid leukaemia (CML). This study aims to evaluate the influence of gender differences on the incidence of cardiovascular toxicity in patients with CML.</p><p><strong>Methods: </strong>The study population consisted of 148 patients (45% women, mean age: 58 ± 14.2 years) diagnosed with CML treated with TKIs. The HFA-ICOS score estimated cardiovascular risk. The HFA-ICOS score revealed that 12% of men and 6% of women were categorized as very high risk while 45% of men and 50% of women fell into the high-risk group. Myocardial ischaemia, peripheral artery disease, venous thromboembolism, pulmonary hypertension and new-onset arterial hypertension during treatment with TKIs were recorded.</p><p><strong>Results: </strong>The incidence of global events between men and women was comparable (35% vs 32%, P = 0.68). There were 33% who experienced a cardiovascular event during TKI therapy, with a significant sex difference in arterial thrombosis incidence (P = 0.02) and venous thrombosis incidence (P = 0.02). Patients treated with ponatinib had a 41% event rate, followed by nilotinib (32%) and imatinib (32%). The HFA-ICOS score demonstrated greater predictive efficacy for events in the female group [area under the curve (AUC) = 0.797] compared with the male group (AUC = 0.537). Very high [hazard ratio (HR) 3.07; confidence interval (CI) 1.11, 8.47 P = 0.03] and high (HR 3.29; CI 1.17, 9.26 P = 0.02) HFA-ICOS scores were associated with increased event risk, particularly in women. Diabetes was women's strongest predictor of events (HR 5.40; CI 1.37, 21.3 P = 0.01).</p><p><strong>Conclusions: </strong>Our study showed a similar frequency of cardiovascular events between men and women. Accurate cardiovascular risk stratification with HFA-ICOS score in cancer patients is crucial. Diabetes and the HFA-ICOS score were significant predictors of events in the female groups. A sex approach in clinical practice could be pursued to improve the appropriateness of care.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946765","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":"Early genetic screening and cardiac intervention in patients with cardiomyopathies in a multidisciplinary clinic.","authors":"Chandu Sadasivan, Luke R Gagnon, Deepan Hazra, Kaiming Wang, Erik Youngson, Jissy Thomas, Anita Y M Chan, D Ian Paterson, Finlay A McAlister, Tara Dzwiniel, Wayne Tymchak, Susan Christian, Gavin Y Oudit","doi":"10.1002/ehf2.15202","DOIUrl":"https://doi.org/10.1002/ehf2.15202","url":null,"abstract":"<p><strong>Aims: </strong>Patients with cardiomyopathies are a heterogeneous group of patients who experience high morbidity and mortality. Early cardiac assessment and intervention with access to genetic counselling in a multidisciplinary Cardiomyopathy Clinic may improve outcomes and prevent progression to advanced heart failure.</p><p><strong>Methods and results: </strong>Our prospective cohort study was conducted at a multidisciplinary Cardiomyopathy Clinic with 421 patients enrolled (42.5% female, median age 58 years), including 224 patients with dilated cardiomyopathy (DCM, 42.9% female, median age 57 years), 72 with hypertrophic cardiomyopathy (HCM, 43.1% female, median age 60 years), 79 with infiltrative cardiomyopathy (65.8% female, median age 70 years) and 46 who were stage A/at risk for genetic cardiomyopathy (54.3% female, median age 36 years). Patients were seen in follow-up at a median of 18 months. A pathogenic/likely pathogenic variant was identified in 28.5% of the total cohort, including 33.3% of the DCM cohort (28% TTN mutations) and 34.1% of the HCM cohort (60% MYBPC3 and 20% MYH7) who underwent genetic testing. The use of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers/angiotensin receptor neprilysin inhibitor (48.3-69.5% of total cohort, P < 0.001), β-blockers (58.4-72.4%, P < 0.001), mineralocorticoid receptor antagonists (33.9-41.4%, P = 0.0014) and sodium/glucose cotransporter-2 inhibitors (5.3-27.9%, P < 0.001) all increased at follow-up. Precision-based therapies were also implemented, including tafamidis for transthyretin amyloidosis (n = 21), enzyme replacement therapy for Fabry disease (n = 14) and mavacamten (n = 4) for HCM. Optimization of medications and devices resulted in improvements in left ventricular ejection fraction (LVEF) from 27% to 43% at follow-up for DCM patients with reduced LVEF at baseline (P < 0.001) and reduction in left ventricular mass index (LVMI) from 156 g/m² to 128 g/m² at follow-up for HCM patients with abnormal LVMI at baseline (P = 0.009). Optimization of therapies was associated with stable plasma biomarkers in stage B patients while lowering levels of BNP (619-517.5 pg/mL, P = 0.048), NT-proBNP (777.5-356 ng/L, P < 0.001) and hsTropT (31-22 ng/L, P = 0.005) at follow-up relative to baseline values for stage C patients. Despite stage B patients having overt cardiomyopathy at baseline, stage A and B patients had a similarly high probability of survival (χ2 = 0.204, P = 0.652). The overall cardiovascular mortality rate was low at 1.7% for the cohort (0.5% for stage B and 3.3% for stage C) over a median of 34-month follow-up.</p><p><strong>Conclusion: </strong>Our study demonstrates that a multidisciplinary cardiomyopathy clinic can improve the clinical profiles of patients with diverse genetic cardiomyopathies.</p>","PeriodicalId":11864,"journal":{"name":"ESC Heart Failure","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909585","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}