Heart最新文献

{"title":"Patterns and organ treatment response of Erdheim-Chester disease with cardiac involvement.","authors":"Hui-Lei Miao, Long Chang, He Lin, Zheng-Zheng Liu, Wei Wu, Na Niu, Xin-Xin Cao","doi":"10.1136/heartjnl-2024-323867","DOIUrl":"10.1136/heartjnl-2024-323867","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the heart response of Erdheim-Chester disease (ECD) through continuous follow-up within our large cohort, for which there is a lack of understanding.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of clinical data from patients with ECD with cardiac involvement diagnosed at our centre between January 2010 and August 2023. We assessed the heart response by integrating pericardial effusion and metabolic responses.</p><p><strong>Results: </strong>A total of 40 patients were included, with a median age of 51.5 years (range: 29-66) and a <i>BRAF^V600E</i> mutation rate of 56%. The most common imaging manifestations observed were pericardial effusion (73%), right atrium (70%) and right atrioventricular sulcus infiltration (58%). Among 21 evaluable patients, 18 (86%) achieved a heart response including 5 (24%) complete response (CR) and 13 (62%) partial response (PR). The CR rate of pericardial effusion response was 33%, while the PR rate was 56%. Regarding the cardiac mass response, 33% of patients showed PR. For cardiac metabolic response, 32% and 53% of patients achieved complete and partial metabolic response, respectively. There was a correlation between pericardial effusion response and cardiac metabolic response (r=0.73 (95% CI 0.12 to 0.83), p<0.001). The median follow-up was 50.2 months (range: 1.0-102.8 months). The estimated 5-year overall survival was 78.9%. The median progression-free survival was 59.4 months (95% CI 26.2 to 92.7 months). Patients who received <i>BRAF</i> inhibitors achieved better heart response (p=0.037) regardless of treatment lines.</p><p><strong>Conclusion: </strong>We pioneered the evaluation of heart response of ECD considering both pericardial effusion and cardiac metabolic response within our cohort, revealing a correlation between these two indicators. <i>BRAF</i> inhibitors may improve heart response, regardless of the treatment lines.</p>","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.1,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11187388/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141075661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evidence of cardiomyopathy associated with Marfan syndrome in children.","authors":"Justin Weigand, Sara Stephens-Novy, Shagun Sachdeva, Tam T Doan, Abigail Yasso, Shaine A Morris","doi":"10.1136/heartjnl-2024-323922","DOIUrl":"10.1136/heartjnl-2024-323922","url":null,"abstract":"<p><strong>Objective: </strong>Marfan syndrome (MFS)-associated cardiomyopathy, defined as ventricular dilation and dysfunction unexplained by volume loading, is not well defined in children. This study evaluated ventricular size and function in paediatric MFS using cardiac MRI (cMRI).</p><p><strong>Methods: </strong>This retrospective cohort study examined patients with MFS <19 years old at first cMRI. Left ventricular (LV) ejection fraction (EF) <55% was considered abnormal, as were z-scores >2. Combined mitral and aortic regurgitation indexed to LV stroke volume <20% defined absent/mild volume load. Biventricular volumes and EF on serial cMRI studies were compared with normative paediatric cMRI values, with measures converted to z-scores as appropriate. Longitudinal changes in volumes and EF were evaluated by mixed linear regression. Associations between ventricular, aortic and mitral characteristics were evaluated.</p><p><strong>Results: </strong>58 patients (60% male) were evaluated. Median age at initial cMRI was 13.6 years (IQR 10.0-15.8 years). Among patients with absent/mild LV volume load at initial cMRI (n=44, 76%), indexed LV end-diastolic volume (EDV) was significantly increased above normative values (median z-score 1.8, IQR 0.6-3.5, p<0.0001) and LVEF was abnormal in 48% (21/44). In the absence of volume loading, mitral valve prolapse (MVP) was associated with larger ventricular volumes and lower LVEF. Among those with serial cMRIs, LVEF and EDV z-scores did not significantly change over a mean follow-up time between cMRI studies of 1.5 years.</p><p><strong>Conclusion: </strong>Ventricular dilation and reduced EF are common in children with MFS and occur with no/mild LV volume load, suggesting intrinsic cardiomyopathy. MVP may be associated with cardiomyopathy.</p>","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.1,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141179554","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":"Non-cardiac birth defects and long-term risk of cardiovascular hospitalisation.","authors":"Nathalie Auger, Gilles Paradis, Jessica Healy-Profitós, Antoine Lewin, Mariyam Malik, Brian J Potter","doi":"10.1136/heartjnl-2023-323632","DOIUrl":"10.1136/heartjnl-2023-323632","url":null,"abstract":"<p><strong>Background: </strong>Patients with heart defects are at risk of developing cardiovascular disease. Our objective was to determine if non-cardiac birth defects are associated with the risk of cardiovascular hospitalisation.</p><p><strong>Methods: </strong>We conducted a longitudinal cohort study of 1 451 409 parous women in Quebec, Canada. We compared patients with cardiac and non-cardiac birth defects of the urinary, central nervous and other systems against patients without defects between 1989 and 2022. The main outcome was hospitalisation for coronary artery disease, ischaemic stroke and other cardiovascular outcomes during 33 years of follow-up. We computed cardiovascular hospitalisation rates and used Cox proportional hazards regression models to measure the association (HR; 95% CI) between non-cardiac defects and later risk of cardiovascular hospitalisation, adjusted for patient characteristics.</p><p><strong>Results: </strong>Women with any birth defect had a higher rate of cardiovascular hospitalisation than women without defects (7.0 vs 3.3 per 1000 person-years). Non-cardiac defects overall were associated with 1.61 times the risk of cardiovascular hospitalisation over time, compared with no defect (95% CI 1.56 to 1.66). Isolated urinary (HR 3.93, 95% CI 3.65 to 4.23), central nervous system (HR 3.33, 95% CI 2.94 to 3.76) and digestive defects (HR 2.39, 95% CI 2.16 to 2.65) were associated with the greatest risk of cardiovascular hospitalisation. These anomalies were associated with cardiovascular hospitalisation whether they presented alone or clustered with other defects. Nevertheless, heart defects were associated with the greatest risk of cardiovascular hospitalisation (HR 10.30, 95% CI 9.86 to 10.75).</p><p><strong>Conclusion: </strong>The findings suggest that both cardiac and non-cardiac birth defects are associated with an increased risk of developing cardiovascular disease among parous women.</p>","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.7,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141075656","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":"Management of arrythmias during pregnancy.","authors":"Lisa Albertini, Danna A Spears","doi":"10.1136/heartjnl-2023-322746","DOIUrl":"10.1136/heartjnl-2023-322746","url":null,"abstract":"","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.7,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138295019","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":"Acute-onset palpitation and presyncope in a young male","authors":"M. Iliyas, S. Abhilash, Sasidharan Bijulal","doi":"10.1136/heartjnl-2024-324070","DOIUrl":"https://doi.org/10.1136/heartjnl-2024-324070","url":null,"abstract":"","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.7,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141338935","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":"Moving towards establishing centres of excellence in cardiac amyloidosis: an International Cardio-Oncology Society statement.","authors":"Richard Cheng, Michelle M Kittleson, Ashutosh D Wechalekar, Jose Alvarez-Cardona, Joshua D Mitchell, Ariane Vieira Scarlatelli Macedo, Joao Pedro Passos Dutra, Courtney M Campbell, Jennifer E Liu, Heather J Landau, Margot K Davis, Siobhain Morrissey, Stephen Casselli, Isabelle Lousada, Juliane Dantas Seabra-Garcez, Roberta Shcolnik Szor, Sarju Ganatra, Barry Trachtenberg, Mathew S Maurer, Keith Stockerl-Goldstein, Daniel Lenihan","doi":"10.1136/heartjnl-2023-323502","DOIUrl":"10.1136/heartjnl-2023-323502","url":null,"abstract":"<p><p>The prevalence of amyloidosis has been increasing, driven by a combination of improved awareness, evolution of diagnostic pathways, and effective treatment options for both transthyretin and light chain amyloidosis. Due to the complexity of amyloidosis, centralised expert providers with experience in delineating the nuances of confirmatory diagnosis and management may be beneficial. There are many potential benefits of a centre of excellence designation for the treatment of amyloidosis including recognition of institutions that have been leading the way for the optimal treatment of this condition, establishing the expectations for any centre who is engaging in the treatment of amyloidosis and developing cooperative groups to allow more effective research in this disease space. Standardising the expectations and criteria for these centres is essential for ensuring the highest quality of clinical care and community education. In order to define what components are necessary for an effective centre of excellence for the treatment of amyloidosis, we prepared a survey in cooperation with a multidisciplinary panel of amyloidosis experts representing an international consortium. The purpose of this position statement is to identify the essential elements necessary for highly effective clinical care and to develop a general standard with which practices or institutions could be recognised as a centre of excellence.</p>","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.7,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139545962","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":"Caveats of high-sensitivity cardiac troponin rapid risk stratification protocols: one size does not fit all.","authors":"Yader Sandoval, Allan S Jaffe","doi":"10.1136/heartjnl-2024-323885","DOIUrl":"10.1136/heartjnl-2024-323885","url":null,"abstract":"","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.7,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141087390","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":"European Society of Cardiology 0/1-hour algorithm (high-sensitivity cardiac troponin T) performance across distinct age groups.","authors":"Marissa J Millard, Nicklaus P Ashburn, Anna C Snavely, Tara Hashemian, Michael Supples, Brandon Allen, Robert Christenson, Troy Madsen, James McCord, Bryn Mumma, Jason Stopyra, Richard Gentry Wilkerson, Simon A Mahler","doi":"10.1136/heartjnl-2023-323621","DOIUrl":"10.1136/heartjnl-2023-323621","url":null,"abstract":"<p><strong>Background: </strong>To determine if the European Society of Cardiology 0/1-hour (ESC 0/1-h) algorithm with high-sensitivity cardiac troponin T (hs-cTnT) meets the ≥99% negative predictive value (NPV) safety threshold for 30-day cardiac death or myocardial infarction (MI) in older, middle-aged and young subgroups.</p><p><strong>Methods: </strong>We conducted a subgroup analysis of adult emergency department patients with chest pain prospectively enrolled from eight US sites (January 2017 to September 2018). Patients were stratified into rule-out, observation and rule-in zones using the hs-cTnT ESC 0/1-h algorithm and classified as older (≥65 years), middle aged (46-64 years) or young (21-45 years). Patients had 0-hour and 1-hour hs-cTnT measures (Roche Diagnostics) and a History, ECG, Age, Risk factor and Troponin (HEART) score. Fisher's exact tests compared rule-out and 30-day cardiac death or MI rates between ages. NPVs with 95% CIs were calculated for the ESC 0/1-h algorithm with and without the HEART score.</p><p><strong>Results: </strong>Of 1430 participants, 26.9% (385/1430) were older, 57.4% (821/1430) middle aged and 15.7% (224/1430) young. Cardiac death or MI at 30 days occurred in 12.8% (183/1430). ESC 0/1-h algorithm ruled out 35.6% (137/385) of older, 62.1% (510/821) of middle-aged and 79.9% of (179/224) young patients (p<0.001). NPV for 30-day cardiac death or MI was 97.1% (95% CI 92.7% to 99.2%) among older patients, 98.4% (95% CI 96.9% to 99.3%) in middle-aged patients and 99.4% (95% CI 96.9% to 100%) among young patients. Adding a HEART score increased NPV to 100% (95% CI 87.7% to 100%) for older, 99.2% (95% CI 97.2% to 99.9%) for middle-aged and 99.4% (95% CI 96.6% to 100%) for young patients.</p><p><strong>Conclusions: </strong>In older and middle-aged adults, the hs-cTnT ESC 0/1-h algorithm was unable to reach a 99% NPV for 30-day cardiac death or MI unless combined with a HEART score.</p><p><strong>Trial registration number: </strong>NCT02984436.</p>","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.1,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140109768","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":"Multimodality imaging of cardiac amyloidosis.","authors":"Dominik C Benz, Sharmila Dorbala","doi":"10.1136/heartjnl-2022-321115","DOIUrl":"10.1136/heartjnl-2022-321115","url":null,"abstract":"","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.1,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10869633/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10013783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wide complex tachycardia with two QRS morphologies: what is the mechanism?","authors":"Maciej Dębski, Charlotte Mehegan, Liam Ring","doi":"10.1136/heartjnl-2024-323973","DOIUrl":"10.1136/heartjnl-2024-323973","url":null,"abstract":"","PeriodicalId":12835,"journal":{"name":"Heart","volume":null,"pages":null},"PeriodicalIF":5.1,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141087427","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}