Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.396
A Borrow, C Chen, V Caso, R Smolnik, J Antonio Gordillo De Souza, R De Caterina, M Unverdorben
Introduction Clinical practice guidelines are intended to improve patients’ outcomes by helping clinicians make the best evidence-based decisions in a time-efficient manner. The ESC Clinical Practice Guidelines for the management of atrial fibrillation (AF), revised in 2024, provide updated recommendations for the diagnosis and management of AF. Purpose To assess physicians’ reactions to and adoption of the 2024 guidelines for the management of AF and to identify remaining unanswered practical, clinical questions. Methods A 4-question poll was conducted between 4 November–4 December 2024 on the social media news feed of registered users of a closed, physician-only, social media platform. Registered platform users from 10 European and 4 Asian countries who were cardiologists, neurologists, or primary care physicians (PCPs) were asked about the importance of the new ESC guidelines for their own clinical practice, guideline topics of greatest interest, remaining uncertainties/unanswered clinical questions about AF and direct oral anticoagulant (DOAC) management, and preferred sources for clinical decision-making. Poll participation was voluntary and no financial compensation was provided to respondents. Descriptive analyses of responses were performed by speciality and by country, and responses to questions permitting multiple answers were rank ordered by proportion of respondents. Results A total of 433 physicians responded to the poll (26% cardiologists, 7% neurologists, 67% PCPs). Respondents were from Spain (33%), Germany (23%), Italy (18%), France (14%), and China (6%). Nearly all respondents (91%; N=417) considered the guidelines as very important or important for their clinical practice (very important: 76% cardiologists, 48% neurologists, 57% PCPs; important: 19% cardiologists, 45% neurologists, 32% PCPs). The 3 specialties (N=344) were largely aligned in their topics of interest, with greatest interest in comorbidity/risk factor management (Figure 1a). By specialty, cardiologists were most interested in CHA2DS2-VASc vs CHA2DS2-VA, neurologists in recommended DOAC dosing, and PCPs in rate vs rhythm control. Across specialties (N=262), the most common remaining uncertainties/unanswered clinical questions were for patients who were very elderly and/or frail, have chronic kidney disease/renal impairment, or have cancer (Figure 1b). Clinical guidelines (European and local), review articles, and congress-based information (both from congress and symposia attendance) were the most useful sources of information for clinical decision-making (N=268; Figure 2). Conclusions The 2024 ESC Clinical Practice Guidelines for AF management were considered important and useful for clinical practice by almost all respondents. Of particular interest were topics related to risk factor management, symptom control, and DOAC dosing. Additional guidance would be welcome on the management of patients with AF in high-risk groups.
{"title":"The 2024 ESC Guidelines for the Management of Atrial Fibrillation serve physicians well but leave a few questions unanswered: a social media-based poll of 433 European and Asian physicians","authors":"A Borrow, C Chen, V Caso, R Smolnik, J Antonio Gordillo De Souza, R De Caterina, M Unverdorben","doi":"10.1093/eurheartj/ehaf784.396","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.396","url":null,"abstract":"Introduction Clinical practice guidelines are intended to improve patients’ outcomes by helping clinicians make the best evidence-based decisions in a time-efficient manner. The ESC Clinical Practice Guidelines for the management of atrial fibrillation (AF), revised in 2024, provide updated recommendations for the diagnosis and management of AF. Purpose To assess physicians’ reactions to and adoption of the 2024 guidelines for the management of AF and to identify remaining unanswered practical, clinical questions. Methods A 4-question poll was conducted between 4 November–4 December 2024 on the social media news feed of registered users of a closed, physician-only, social media platform. Registered platform users from 10 European and 4 Asian countries who were cardiologists, neurologists, or primary care physicians (PCPs) were asked about the importance of the new ESC guidelines for their own clinical practice, guideline topics of greatest interest, remaining uncertainties/unanswered clinical questions about AF and direct oral anticoagulant (DOAC) management, and preferred sources for clinical decision-making. Poll participation was voluntary and no financial compensation was provided to respondents. Descriptive analyses of responses were performed by speciality and by country, and responses to questions permitting multiple answers were rank ordered by proportion of respondents. Results A total of 433 physicians responded to the poll (26% cardiologists, 7% neurologists, 67% PCPs). Respondents were from Spain (33%), Germany (23%), Italy (18%), France (14%), and China (6%). Nearly all respondents (91%; N=417) considered the guidelines as very important or important for their clinical practice (very important: 76% cardiologists, 48% neurologists, 57% PCPs; important: 19% cardiologists, 45% neurologists, 32% PCPs). The 3 specialties (N=344) were largely aligned in their topics of interest, with greatest interest in comorbidity/risk factor management (Figure 1a). By specialty, cardiologists were most interested in CHA2DS2-VASc vs CHA2DS2-VA, neurologists in recommended DOAC dosing, and PCPs in rate vs rhythm control. Across specialties (N=262), the most common remaining uncertainties/unanswered clinical questions were for patients who were very elderly and/or frail, have chronic kidney disease/renal impairment, or have cancer (Figure 1b). Clinical guidelines (European and local), review articles, and congress-based information (both from congress and symposia attendance) were the most useful sources of information for clinical decision-making (N=268; Figure 2). Conclusions The 2024 ESC Clinical Practice Guidelines for AF management were considered important and useful for clinical practice by almost all respondents. Of particular interest were topics related to risk factor management, symptom control, and DOAC dosing. Additional guidance would be welcome on the management of patients with AF in high-risk groups.","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"24 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122063","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.2924
Y Kim, J Lim, S Bang, H Shin, J Yang, I Kang, J Song, H Kim, D Kim, S Chang
Background The role of prostacyclin is critical in pulmonary arterial hypertension (PAH) and parenteral prostacyclin is an essential treatment option for high-risk patients. However, its real-world utilization remains limited due to physician unfamiliarity and high costs. Comprehensive analysis of real-world treprostinil use has been scarce. Purpose This study aimed to investigate the treatment patterns and clinical response to treprostinil in real-world practice. Methods Patients prescribed treprostinil for PAH from 2011 to 2024 at two tertiary referral centers were identified. Clinical characteristics, treatment response and survival outcomes were analyzed. We categorized the study populations into three groups based on the timing of treprostinil initiation: optimal sequential combination therapy (when the patient reached intermediate to high risk), early triple combination (in high-risk patients), and delayed sequential combination therapy (delayed initiation of treprostinil in already diagnosed PAH when they reached the functional class IV or definite indication for lung transplantation). Results A total of 94 patients were identified, with 35 (37.2%) in optimal sequential combination therapy group, 12 (12.8%) in early triple combination therapy group, and 47 (50.0%) in delayed sequential combination therapy group. Hemodynamic characteristics and risk profiles for PAH were similar among the study groups except for functional class. The cumulative 1-year mortality rate in the overall population was 35.1%. 1-year mortality was significantly higher in delayed sequential combination therapy group (53.2%) compared to optimal sequential combination therapy group (20.0%) and early triple combination therapy group (8.3%) (p<0.001). Optimal sequential combination and early triple combination therapy over delayed sequential combination therapy were identified as independent predictor for death at 1 year (adjusted HR 0.239, 95% CI 0.096-0.599, p=0.002; adjusted HR 0.107, 95% CI 0.014-0.811, p=0.031). The treatment response to treprostinil was significantly lower in the delayed sequential combination therapy group (40.4%) compared to the optimal sequential combination (85.7%) and early triple combination therapy group (75.0%) (p<0.001). Optimal sequential combination and early triple combination therapy were associated with higher rates of successful transition to oral maintenance therapy (40.0% and 91.7%, respectively), whereas 8.5% of patients in the delayed sequential combination therapy group maintained therapy (p<0.001). Conclusions Although the guideline recommends the optimal timing for intervention with parenteral prostacyclin, it is frequently delayed in real-world clinics. Treatment outcomes show dramatic differences based on the timing—early, optimal, and delayed. Furthermore, a transition to an oral IP3 receptor agonist was even possible for some patients who survived due to early intervention with parenteral prostacyclin
背景：前列环素在肺动脉高压（PAH）中的作用至关重要，对于高危患者，肠外注射前列环素是必不可少的治疗选择。然而，由于医生的不熟悉和高昂的成本，其在现实世界中的应用仍然有限。对真实世界曲前列汀使用情况的综合分析很少。目的本研究旨在探讨现实世界中曲前列氨酯的治疗模式和临床反应。方法对2011 - 2024年在两家三级转诊中心使用曲前列替尼治疗PAH的患者进行分析。分析两组患者的临床特点、治疗效果及生存结局。我们根据曲前列替尼起始时间将研究人群分为三组：最佳序贯联合治疗（当患者达到中高风险时）、早期三联治疗（高危患者）和延迟序贯联合治疗（当已诊断为PAH的患者达到功能级IV或明确的肺移植指证时延迟曲前列替尼起始治疗）。结果94例患者中，最佳顺序联合治疗组35例（37.2%），早期三联治疗组12例（12.8%），延迟顺序联合治疗组47例（50.0%）。除功能组别外，各研究组的血流动力学特征和多环芳烃风险概况相似。总体人群1年累计死亡率为35.1%。延迟顺序联合治疗组的1年死亡率（53.2%）明显高于最佳顺序联合治疗组（20.0%）和早期三联治疗组（8.3%）（p<0.001）。最佳序贯组合和早期三联治疗优于延迟序贯组合治疗是1年死亡的独立预测因子（调整后HR 0.239, 95% CI 0.096 ~ 0.599, p=0.002；调整后HR 0.107, 95% CI 0.014 ~ 0.811, p=0.031）。延迟顺序联合治疗组对曲前列地尼的治疗反应（40.4%）明显低于最佳顺序联合治疗组（85.7%）和早期三联治疗组（75.0%）（p<0.001）。最佳顺序联合治疗和早期三联治疗与较高的成功过渡到口服维持治疗的比例相关（分别为40.0%和91.7%），而延迟顺序联合治疗组的患者维持治疗的比例为8.5% （p<0.001）。结论：尽管指南建议肠外注射前列环素干预的最佳时机，但在现实世界的临床中，它经常被推迟。治疗结果根据治疗时间的不同表现出显著差异——早期、最佳和延迟。此外，对于一些由于早期肠外注射前列环素干预而存活的患者，甚至可能过渡到口服IP3受体激动剂。
{"title":"Dramatic differences resulting from treatment timing of treprostinil in high-risk patients: a real-world data analysis","authors":"Y Kim, J Lim, S Bang, H Shin, J Yang, I Kang, J Song, H Kim, D Kim, S Chang","doi":"10.1093/eurheartj/ehaf784.2924","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.2924","url":null,"abstract":"Background The role of prostacyclin is critical in pulmonary arterial hypertension (PAH) and parenteral prostacyclin is an essential treatment option for high-risk patients. However, its real-world utilization remains limited due to physician unfamiliarity and high costs. Comprehensive analysis of real-world treprostinil use has been scarce. Purpose This study aimed to investigate the treatment patterns and clinical response to treprostinil in real-world practice. Methods Patients prescribed treprostinil for PAH from 2011 to 2024 at two tertiary referral centers were identified. Clinical characteristics, treatment response and survival outcomes were analyzed. We categorized the study populations into three groups based on the timing of treprostinil initiation: optimal sequential combination therapy (when the patient reached intermediate to high risk), early triple combination (in high-risk patients), and delayed sequential combination therapy (delayed initiation of treprostinil in already diagnosed PAH when they reached the functional class IV or definite indication for lung transplantation). Results A total of 94 patients were identified, with 35 (37.2%) in optimal sequential combination therapy group, 12 (12.8%) in early triple combination therapy group, and 47 (50.0%) in delayed sequential combination therapy group. Hemodynamic characteristics and risk profiles for PAH were similar among the study groups except for functional class. The cumulative 1-year mortality rate in the overall population was 35.1%. 1-year mortality was significantly higher in delayed sequential combination therapy group (53.2%) compared to optimal sequential combination therapy group (20.0%) and early triple combination therapy group (8.3%) (p&lt;0.001). Optimal sequential combination and early triple combination therapy over delayed sequential combination therapy were identified as independent predictor for death at 1 year (adjusted HR 0.239, 95% CI 0.096-0.599, p=0.002; adjusted HR 0.107, 95% CI 0.014-0.811, p=0.031). The treatment response to treprostinil was significantly lower in the delayed sequential combination therapy group (40.4%) compared to the optimal sequential combination (85.7%) and early triple combination therapy group (75.0%) (p&lt;0.001). Optimal sequential combination and early triple combination therapy were associated with higher rates of successful transition to oral maintenance therapy (40.0% and 91.7%, respectively), whereas 8.5% of patients in the delayed sequential combination therapy group maintained therapy (p&lt;0.001). Conclusions Although the guideline recommends the optimal timing for intervention with parenteral prostacyclin, it is frequently delayed in real-world clinics. Treatment outcomes show dramatic differences based on the timing—early, optimal, and delayed. Furthermore, a transition to an oral IP3 receptor agonist was even possible for some patients who survived due to early intervention with parenteral prostacyclin","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"30 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.4035
M Sandini, A Mauro, E Bizzi, R Mascolo, V Collini, L Bernardo, M Imazio, A Brucato
Background Current guidelines for diagnosis and treatment of recurrent pericarditis are based on adult populations. Aims Due to limited pediatric data, this study aims to highlight clinical, laboratory, and therapeutic differences between idiopathic recurrent pericarditis in children and adults to optimize pediatric disease management. Methods and Results This retrospective multicentric cohort study analyzed data from patients with recurrent pericarditis (idiopathic or post-cardiac injury). Clinical, laboratory, and outcome data were compared between pediatric (<18 years) and adult (>18 years) patients. A total of 61 children and 289 adults were included. Follow-up was significantly longer in children (8 vs. 4 years, p < 0.001). Males were predominantly affected in pediatric patient’s cohort (30.3% female vs 69.7% male), while gender distribution was similar in adults (51.6% female vs. 48.4% male). Children were hospitalized more frequently than adult (81.8% vs. 58.5%, p < 0.05). From the clinical point of view, chest pain was the most common symptom in both group (100% vs. 57.1%), whereas dyspnea was exclusive to adults (31.5%, p < 0.05). Pericardial effusion was less common in children (63.6% vs. 80.6%, p < 0.05) and no pediatric patients developed constrictive pericarditis requiring pericardiectomy. Pediatric patient’s cohort had a higher incidence of ST-segment elevation on ECG (64.7% vs. 32.4%, p < 0.01) and a higher relative lymphocyte count (p < 0.05) with a lower neutrophil-to-lymphocyte ratio (3.65 vs. 4.71, p < 0.05). Although the difference is not statistically significant, the troponin levels were found to be lower in children compared with adults (29 ng/L vs 72.6 ng/L, p=0.294). Recurrence of pericarditis was more frequent in adults (9.59 vs. 4.4 episodes/10 years, p < 0.001), but children had significantly longer relapse-free periods (68.31 vs. 31.72 months, p < 0.001). There were no significant differences observed in the treatment between the two groups. A total of 48 children (78.8%) and 237 adults (82.0%) were treated with NSAIDs. Colchicine was prescribed to 22 children (36.4%) and 136 adults (47.1%), while 15 children (24.2%) and 73 adults (25.3%) received Anakinra. The only notable difference was in the dosage of prednisone, with children receiving significantly higher doses compared to adults (37.25 mg vs. 25 mg, p = 0.023). Conclusions Recurrent pericarditis in children follows a different course than in adults, with fewer recurrences, prolonged symptom-free periods, and typical ECG abnormalities. The higher corticosteroid use in children raises some concerns because of its potential side effects, including growth impairment and osteoporosis. IL-1 inhibitors should be considered to minimize corticosteroid administration. These findings emphasize the importance of developing a pediatric age-specific guideline.
背景：目前复发性心包炎的诊断和治疗指南是基于成人人群的。由于儿科数据有限，本研究旨在突出儿童和成人特发性复发性心包炎的临床、实验室和治疗差异，以优化儿科疾病管理。方法和结果本回顾性多中心队列研究分析了复发性心包炎（特发性或心脏后损伤）患者的数据。比较儿科（18岁）和成人（18岁）患者的临床、实验室和结局数据。共有61名儿童和289名成人被纳入研究。儿童的随访时间明显更长（8年vs. 4年，p < 0.001）。在儿科患者队列中，男性主要受影响（女性占30.3%，男性占69.7%），而成人患者的性别分布相似（女性占51.6%，男性占48.4%）。儿童住院率高于成人（81.8%比58.5%,p < 0.05）。从临床角度来看，胸痛是两组患者最常见的症状（100% vs. 57.1%），而呼吸困难是成人所特有的（31.5%,p < 0.05）。心包积液在儿童中较少见（63.6%对80.6%,p < 0.05），没有儿童患者发生缩窄性心包炎需要心包切除术。儿童患者队列心电图st段抬高发生率较高（64.7% vs. 32.4%, p < 0.01），相对淋巴细胞计数较高（p < 0.05），中性粒细胞与淋巴细胞比值较低（3.65 vs. 4.71, p < 0.05）。虽然差异无统计学意义，但发现儿童肌钙蛋白水平低于成人（29 ng/L vs 72.6 ng/L, p=0.294）。心包炎的复发在成人中更为常见（9.59 vs 4.4次/10年，p < 0.001），但儿童的无复发期明显更长（68.31 vs 31.72个月，p < 0.001）。两组治疗效果无明显差异。共有48名儿童（78.8%）和237名成人（82.0%）接受了非甾体抗炎药治疗。22名儿童（36.4%）和136名成人（47.1%）使用秋水仙碱，15名儿童（24.2%）和73名成人（25.3%）使用阿那白。唯一的显著差异是泼尼松的剂量，儿童接受的剂量明显高于成人（37.25 mg vs. 25 mg, p = 0.023）。结论：儿童心包炎复发的病程与成人不同，复发较少，无症状期延长，心电图异常典型。儿童使用较多的皮质类固醇引起了一些担忧，因为它可能产生副作用，包括生长障碍和骨质疏松症。应考虑使用IL-1抑制剂来减少皮质类固醇的使用。这些发现强调了制定针对儿童年龄的指南的重要性。
{"title":"Idiopathic recurrent pericarditis in children and adults:clinical and diagnostic differences","authors":"M Sandini, A Mauro, E Bizzi, R Mascolo, V Collini, L Bernardo, M Imazio, A Brucato","doi":"10.1093/eurheartj/ehaf784.4035","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.4035","url":null,"abstract":"Background Current guidelines for diagnosis and treatment of recurrent pericarditis are based on adult populations. Aims Due to limited pediatric data, this study aims to highlight clinical, laboratory, and therapeutic differences between idiopathic recurrent pericarditis in children and adults to optimize pediatric disease management. Methods and Results This retrospective multicentric cohort study analyzed data from patients with recurrent pericarditis (idiopathic or post-cardiac injury). Clinical, laboratory, and outcome data were compared between pediatric (&lt;18 years) and adult (&gt;18 years) patients. A total of 61 children and 289 adults were included. Follow-up was significantly longer in children (8 vs. 4 years, p &lt; 0.001). Males were predominantly affected in pediatric patient’s cohort (30.3% female vs 69.7% male), while gender distribution was similar in adults (51.6% female vs. 48.4% male). Children were hospitalized more frequently than adult (81.8% vs. 58.5%, p &lt; 0.05). From the clinical point of view, chest pain was the most common symptom in both group (100% vs. 57.1%), whereas dyspnea was exclusive to adults (31.5%, p &lt; 0.05). Pericardial effusion was less common in children (63.6% vs. 80.6%, p &lt; 0.05) and no pediatric patients developed constrictive pericarditis requiring pericardiectomy. Pediatric patient’s cohort had a higher incidence of ST-segment elevation on ECG (64.7% vs. 32.4%, p &lt; 0.01) and a higher relative lymphocyte count (p &lt; 0.05) with a lower neutrophil-to-lymphocyte ratio (3.65 vs. 4.71, p &lt; 0.05). Although the difference is not statistically significant, the troponin levels were found to be lower in children compared with adults (29 ng/L vs 72.6 ng/L, p=0.294). Recurrence of pericarditis was more frequent in adults (9.59 vs. 4.4 episodes/10 years, p &lt; 0.001), but children had significantly longer relapse-free periods (68.31 vs. 31.72 months, p &lt; 0.001). There were no significant differences observed in the treatment between the two groups. A total of 48 children (78.8%) and 237 adults (82.0%) were treated with NSAIDs. Colchicine was prescribed to 22 children (36.4%) and 136 adults (47.1%), while 15 children (24.2%) and 73 adults (25.3%) received Anakinra. The only notable difference was in the dosage of prednisone, with children receiving significantly higher doses compared to adults (37.25 mg vs. 25 mg, p = 0.023). Conclusions Recurrent pericarditis in children follows a different course than in adults, with fewer recurrences, prolonged symptom-free periods, and typical ECG abnormalities. The higher corticosteroid use in children raises some concerns because of its potential side effects, including growth impairment and osteoporosis. IL-1 inhibitors should be considered to minimize corticosteroid administration. These findings emphasize the importance of developing a pediatric age-specific guideline.","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"87 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.2056
A Sakalidis, K Dimitriadis, E Adamopoulou, A Koulouriotis, P Iliakis, M Bora, S Drogkaris, A E Karanikola, K Papadomarkaki, A Lazarou, S Soulaidopoulos, I Leontsinis, I Kontonasakis, C Fragkoulis, K Tsioufis
Background Recent studies indicate that patients with Ischemia with Non-Obstructive Coronary Arteries (INOCA) constitute a heterogeneous population at heightened risk of cardiovascular events. Nevertheless, there remains a paucity of scientific evidence investigating the potential presence of widespread microangiopathy in this distinct patient cohort. Purpose This study aims to evaluate nailfold capillary density in patients with INOCA compared to individuals without evidence of coronary microvascular dysfunction (CMD). Methods This single-center, prospective, observational study enrolled patients with INOCA. In the absence of significant coronary artery stenosis, functional coronary circulation was assessed in all patients. Coronary flow reserve (CFR) and the index of microvascular resistance (IMR) were invasively measured using the thermodilution technique with a temperature/pressure sensor-tipped guidewire in the left anterior descending coronary artery. Patients with CMD were further classified into two subgroups based on structural and functional endotypes, using CFR <2.5 and IMR ≥25 as tresholds to define abnormal values. Nailfold capillary circulation was evaluated in all participants using videocapillaroscopy, a non-invasive technique that assesses small vessels in the nailfold microcirculation, performed with a stereomicroscope. Results A total of 82 participants were enrolled in the study, including 36 controls without coronary microvascular dysfunction (non-CMD group) (16 female, 44%; mean age: 55.7 ± 7 years) and 46 patients with ischemia and non-obstructive coronary arteries (CMD group) (32 female, 69%; mean age: 51.9 ± 8.7 years). In the CMD group, the mean coronary flow reserve (CFR) and index of microvascular resistance (IMR) values were 1.68 ± 0.6 and 28 ± 23, respectively. Capillary density was significantly reduced in CMD patients compared to non-CMD participants (9.3 ± 2.9 vs. 11 ± 1.8 capillaries/mm, p = 0.04). This difference remained statistically significant after adjustment for multiple comparisons (p < 0.05). No significant differences were observed between the CMD endotypes (functional vs. structural) regarding capillary circulation function (p = NS). Conclusions Patients with coronary microvascular dysfunction exhibit a significantly lower nailfold capillary density compared to individuals without CMD. These findings suggest a potential association between vascular abnormalities at both the coronary microcirculatory and peripheral vascular levels. The observed reduction in nailfold capillary density underscores its potential utility as a diagnostic marker for identifying coronary microvascular dysfunction in clinical practice. This parameter may be particularly valuable for risk stratification and guiding management strategies in the heterogeneous population of patients with INOCA.CMD correlation with capillary density CMD assessed by bolus thermodilution
最近的研究表明，非阻塞性冠状动脉缺血（INOCA）患者构成了心血管事件高风险的异质人群。然而，在这一独特的患者队列中，仍然缺乏科学证据来调查广泛存在微血管病变的可能性。目的：本研究旨在评价冠状动脉微血管功能障碍（CMD）患者与无冠状动脉微血管功能障碍患者的甲襞毛细血管密度。方法本研究为单中心、前瞻性、观察性研究，纳入了INOCA患者。在没有明显冠状动脉狭窄的情况下，评估所有患者的冠状动脉循环功能。采用有创热稀释技术，在冠状动脉左前降支内置入温度/压力传感器导丝，测量冠状动脉血流储备（CFR）和微血管阻力指数（IMR）。根据结构型和功能型将CMD患者进一步分为2个亚组，以CFR &；lt；2.5和IMR≥25为阈值定义异常值。所有参与者的甲襞毛细血管循环使用视频毛细血管镜进行评估，这是一种非侵入性技术，可以评估甲襞微循环中的小血管，并在体视显微镜下进行。结果共纳入82例受试者，包括36例无冠状动脉微血管功能障碍的对照组（非CMD组）（16例女性，44%，平均年龄55.7±7岁）和46例冠状动脉缺血非阻塞性患者（CMD组）（32例女性，69%，平均年龄51.9±8.7岁）。CMD组平均冠状动脉血流储备（CFR）为1.68±0.6，微血管阻力指数（IMR）为28±23。与非CMD患者相比，CMD患者的毛细血管密度显著降低（9.3±2.9 vs 11±1.8毛细血管/mm， p = 0.04）。经多次比较调整后，这一差异仍具有统计学意义（p < 0.05）。在毛细血管循环功能方面，CMD内型（功能性与结构性）之间没有显著差异（p = NS）。结论冠状动脉微血管功能障碍患者甲襞毛细血管密度明显低于非CMD患者。这些发现提示在冠状动脉微循环和外周血管水平的血管异常之间存在潜在的关联。观察到甲襞毛细血管密度的降低强调了其在临床实践中作为诊断冠状动脉微血管功能障碍的潜在用途。这一参数可能特别有价值的风险分层和指导管理策略，在异质人群的INOCA患者。CMD与毛细管密度的相关性
{"title":"Association between peripheral microangiopathy and coronary microvascular dysfunction in patients with ischemia and non-obstructive coronary arteries","authors":"A Sakalidis, K Dimitriadis, E Adamopoulou, A Koulouriotis, P Iliakis, M Bora, S Drogkaris, A E Karanikola, K Papadomarkaki, A Lazarou, S Soulaidopoulos, I Leontsinis, I Kontonasakis, C Fragkoulis, K Tsioufis","doi":"10.1093/eurheartj/ehaf784.2056","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.2056","url":null,"abstract":"Background Recent studies indicate that patients with Ischemia with Non-Obstructive Coronary Arteries (INOCA) constitute a heterogeneous population at heightened risk of cardiovascular events. Nevertheless, there remains a paucity of scientific evidence investigating the potential presence of widespread microangiopathy in this distinct patient cohort. Purpose This study aims to evaluate nailfold capillary density in patients with INOCA compared to individuals without evidence of coronary microvascular dysfunction (CMD). Methods This single-center, prospective, observational study enrolled patients with INOCA. In the absence of significant coronary artery stenosis, functional coronary circulation was assessed in all patients. Coronary flow reserve (CFR) and the index of microvascular resistance (IMR) were invasively measured using the thermodilution technique with a temperature/pressure sensor-tipped guidewire in the left anterior descending coronary artery. Patients with CMD were further classified into two subgroups based on structural and functional endotypes, using CFR &lt;2.5 and IMR ≥25 as tresholds to define abnormal values. Nailfold capillary circulation was evaluated in all participants using videocapillaroscopy, a non-invasive technique that assesses small vessels in the nailfold microcirculation, performed with a stereomicroscope. Results A total of 82 participants were enrolled in the study, including 36 controls without coronary microvascular dysfunction (non-CMD group) (16 female, 44%; mean age: 55.7 ± 7 years) and 46 patients with ischemia and non-obstructive coronary arteries (CMD group) (32 female, 69%; mean age: 51.9 ± 8.7 years). In the CMD group, the mean coronary flow reserve (CFR) and index of microvascular resistance (IMR) values were 1.68 ± 0.6 and 28 ± 23, respectively. Capillary density was significantly reduced in CMD patients compared to non-CMD participants (9.3 ± 2.9 vs. 11 ± 1.8 capillaries/mm, p = 0.04). This difference remained statistically significant after adjustment for multiple comparisons (p &lt; 0.05). No significant differences were observed between the CMD endotypes (functional vs. structural) regarding capillary circulation function (p = NS). Conclusions Patients with coronary microvascular dysfunction exhibit a significantly lower nailfold capillary density compared to individuals without CMD. These findings suggest a potential association between vascular abnormalities at both the coronary microcirculatory and peripheral vascular levels. The observed reduction in nailfold capillary density underscores its potential utility as a diagnostic marker for identifying coronary microvascular dysfunction in clinical practice. This parameter may be particularly valuable for risk stratification and guiding management strategies in the heterogeneous population of patients with INOCA.CMD correlation with capillary density CMD assessed by bolus thermodilution","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"8 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.1612
J Jannink, J Van Der Veen, J W Eikelboom, J H Cornel, T J S Opstal, C A Budgeon, S M Nidorf, F L J Visseren, F M A C Martens, A T L Fiolet, A Mosterd
Background Recent trials have demonstrated that low-dose colchicine can reduce the risk of cardiovascular events in patients with coronary artery disease.(1) However, discontinuation rates of trial medication ranged from 10.1% to 25.9%, potentially reducing its effectiveness.(2,3) The role of side effects in this remains unclear. Purpose To evaluate the incidence and type of side effects that led to premature discontinuation of trial medication in participants with chronic coronary syndromes in the Low-Dose Colchicine 2 (LoDoCo2) trial. Methods We evaluated the incidence of side effects leading to discontinuation of trial medication in the LoDoCo2 trial, which consisted of a 30-day run-in phase during which all participants received colchicine 0.5 mg once daily, followed by randomization to colchicine or placebo. Side effects of colchicine were evaluated at the end of the run-in phase (early side effects, within 30 days) and during the randomization phase (late side effects, median follow-up: 29.5 months). Cumulative incidences of side effects are presented using Kaplan-Meier curves and compared by treatment arm using Cox proportional hazards modelling. Results Trial medication was discontinued by 997 of 6519 (15.3%) participants during the run-in phase, of whom 618 (9.5%) participants reported early side effects. After randomization, 273 (10.0%) participants in the colchicine group and 281 (10.3%) participants in the placebo group discontinued trial medication. Among those who stopped trial medication, 114 participants in the colchicine group reported side effects (4.2%, 1.6 events per 100 person-years), compared to 120 participants in the placebo group (4.4%, 1.7 events per 100 person-years, hazard ratio [HR] 0.95, 95% confidence interval [CI] 0.73–1.22). Gastrointestinal upset and myalgia were the most common side effects (2.0% and 1.2% in the colchicine group and 1.7% and 1.6% in the placebo group, respectively), with no differences between treatment groups. Female sex was independently associated with an increased risk of both early- and late side effects, irrespective of treatment allocation. Statin use was independently associated with lower rates of late side effects, with no difference between colchicine and placebo. Conclusion After the open-label run-in phase, the incidence of late side effects in participants with chronic coronary syndromes did not differ between patients randomized to low-dose colchicine or placebo.
{"title":"Side effects of low-dose colchicine in chronic coronary syndromes, the LoDoCo2 trial","authors":"J Jannink, J Van Der Veen, J W Eikelboom, J H Cornel, T J S Opstal, C A Budgeon, S M Nidorf, F L J Visseren, F M A C Martens, A T L Fiolet, A Mosterd","doi":"10.1093/eurheartj/ehaf784.1612","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.1612","url":null,"abstract":"Background Recent trials have demonstrated that low-dose colchicine can reduce the risk of cardiovascular events in patients with coronary artery disease.(1) However, discontinuation rates of trial medication ranged from 10.1% to 25.9%, potentially reducing its effectiveness.(2,3) The role of side effects in this remains unclear. Purpose To evaluate the incidence and type of side effects that led to premature discontinuation of trial medication in participants with chronic coronary syndromes in the Low-Dose Colchicine 2 (LoDoCo2) trial. Methods We evaluated the incidence of side effects leading to discontinuation of trial medication in the LoDoCo2 trial, which consisted of a 30-day run-in phase during which all participants received colchicine 0.5 mg once daily, followed by randomization to colchicine or placebo. Side effects of colchicine were evaluated at the end of the run-in phase (early side effects, within 30 days) and during the randomization phase (late side effects, median follow-up: 29.5 months). Cumulative incidences of side effects are presented using Kaplan-Meier curves and compared by treatment arm using Cox proportional hazards modelling. Results Trial medication was discontinued by 997 of 6519 (15.3%) participants during the run-in phase, of whom 618 (9.5%) participants reported early side effects. After randomization, 273 (10.0%) participants in the colchicine group and 281 (10.3%) participants in the placebo group discontinued trial medication. Among those who stopped trial medication, 114 participants in the colchicine group reported side effects (4.2%, 1.6 events per 100 person-years), compared to 120 participants in the placebo group (4.4%, 1.7 events per 100 person-years, hazard ratio [HR] 0.95, 95% confidence interval [CI] 0.73–1.22). Gastrointestinal upset and myalgia were the most common side effects (2.0% and 1.2% in the colchicine group and 1.7% and 1.6% in the placebo group, respectively), with no differences between treatment groups. Female sex was independently associated with an increased risk of both early- and late side effects, irrespective of treatment allocation. Statin use was independently associated with lower rates of late side effects, with no difference between colchicine and placebo. Conclusion After the open-label run-in phase, the incidence of late side effects in participants with chronic coronary syndromes did not differ between patients randomized to low-dose colchicine or placebo.","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"56 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146121964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.2740
J D Gillmore, M Grogan, T Sutton, M Dupont, N M Fine, K Bhatt, D Delgado, C Chen, J F Tamby, S Siddhanti, J C Fox, M Fontana
Background The National Amyloidosis Centre (NAC) staging system for transthyretin amyloid cardiomyopathy (ATTR-CM) is used to classify patients into prognostic categories based on N-terminal pro-B-type natriuretic peptide (NT-proBNP) level and estimated glomerular filtration rate (eGFR). The NAC staging system has been shown to predict ongoing survival throughout the course of ATTR-CM, with survival progressively decreasing from stage I to stage III. Acoramidis, a highly selective, oral transthyretin stabilizer that achieves near-complete (≥90%) transthyretin stabilization, has recently been approved in Europe and the USA for the treatment of wild-type or variant ATTR-CM in adults. In the phase 3 ATTRibute-CM study, acoramidis treatment was well tolerated and led to a 42% relative risk reduction in the composite of all-cause mortality and recurrent cardiovascular hospitalizations over 30 months compared with placebo (p=0.0005). Purpose To evaluate the ability of acoramidis treatment, as compared with placebo, to stabilize or improve NAC stage after 30 months in participants with ATTR-CM from the ATTRibute-CM study. Methods Participants in ATTRibute-CM were randomized 2:1 to receive acoramidis or placebo for 30 months. Efficacy analyses were conducted in the modified intention-to-treat population, which consisted of all randomized participants who had received at least one dose of acoramidis or placebo, had at least one efficacy evaluation after baseline and had a baseline eGFR ≥30 mL/min/1.73 m2. NAC stage at baseline and Month 30 was assessed. Changes from baseline to Month 30 were categorized as "stable", "improved" or "worsened". The "stable" category comprised participants who stayed within the same NAC stage at baseline and Month 30. The "improved" category comprised participants who moved from a higher NAC stage at baseline to a lower stage at Month 30. The "worsened or missing" category comprised participants who moved from a lower NAC stage at baseline to a higher stage at Month 30 and participants whose Month 30 NAC stage was missing. The change in NAC stage was compared between treatment groups using a stratified Cochran-Mantel-Haenszel test with stratification factors of genotype, NT-proBNP level and eGFR as recorded in the interactive voice/web response system at randomization. Results Overall, 611 participants were analysed (acoramidis: n=409; placebo: n=202). Baseline characteristics were comparable between treatment groups. Most participants had NAC stage I at baseline (acoramidis: 58.9%; placebo: 59.4%; Table). At Month 30, NAC stage remained stable or improved in 52.1% of acoramidis participants compared with 43.1% of placebo participants (p=0.0351; Figure). Conclusions Acoramidis treatment resulted in a greater proportion of participants whose NAC stage improved or remained stable at Month 30 compared with placebo, indicating better stabilization of their disease.
{"title":"Acoramidis has a beneficial effect compared with placebo on change from baseline in NAC ATTR stage at month 30 in patients with ATTR-CM: results from the ATTRibute-CM study","authors":"J D Gillmore, M Grogan, T Sutton, M Dupont, N M Fine, K Bhatt, D Delgado, C Chen, J F Tamby, S Siddhanti, J C Fox, M Fontana","doi":"10.1093/eurheartj/ehaf784.2740","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.2740","url":null,"abstract":"Background The National Amyloidosis Centre (NAC) staging system for transthyretin amyloid cardiomyopathy (ATTR-CM) is used to classify patients into prognostic categories based on N-terminal pro-B-type natriuretic peptide (NT-proBNP) level and estimated glomerular filtration rate (eGFR). The NAC staging system has been shown to predict ongoing survival throughout the course of ATTR-CM, with survival progressively decreasing from stage I to stage III. Acoramidis, a highly selective, oral transthyretin stabilizer that achieves near-complete (≥90%) transthyretin stabilization, has recently been approved in Europe and the USA for the treatment of wild-type or variant ATTR-CM in adults. In the phase 3 ATTRibute-CM study, acoramidis treatment was well tolerated and led to a 42% relative risk reduction in the composite of all-cause mortality and recurrent cardiovascular hospitalizations over 30 months compared with placebo (p=0.0005). Purpose To evaluate the ability of acoramidis treatment, as compared with placebo, to stabilize or improve NAC stage after 30 months in participants with ATTR-CM from the ATTRibute-CM study. Methods Participants in ATTRibute-CM were randomized 2:1 to receive acoramidis or placebo for 30 months. Efficacy analyses were conducted in the modified intention-to-treat population, which consisted of all randomized participants who had received at least one dose of acoramidis or placebo, had at least one efficacy evaluation after baseline and had a baseline eGFR ≥30 mL/min/1.73 m2. NAC stage at baseline and Month 30 was assessed. Changes from baseline to Month 30 were categorized as \"stable\", \"improved\" or \"worsened\". The \"stable\" category comprised participants who stayed within the same NAC stage at baseline and Month 30. The \"improved\" category comprised participants who moved from a higher NAC stage at baseline to a lower stage at Month 30. The \"worsened or missing\" category comprised participants who moved from a lower NAC stage at baseline to a higher stage at Month 30 and participants whose Month 30 NAC stage was missing. The change in NAC stage was compared between treatment groups using a stratified Cochran-Mantel-Haenszel test with stratification factors of genotype, NT-proBNP level and eGFR as recorded in the interactive voice/web response system at randomization. Results Overall, 611 participants were analysed (acoramidis: n=409; placebo: n=202). Baseline characteristics were comparable between treatment groups. Most participants had NAC stage I at baseline (acoramidis: 58.9%; placebo: 59.4%; Table). At Month 30, NAC stage remained stable or improved in 52.1% of acoramidis participants compared with 43.1% of placebo participants (p=0.0351; Figure). Conclusions Acoramidis treatment resulted in a greater proportion of participants whose NAC stage improved or remained stable at Month 30 compared with placebo, indicating better stabilization of their disease.","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"2 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146121966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.2337
I Cohen, J G Malins, M Cohen-Shelly, Y Asaf, M Fiman, K Faierstein, K Sudri, E Zimlichman, E Schwammenthal, R Klempfner, E Maor
Background Mitral regurgitation (MR) and tricuspid regurgitation (TR) are prevalent valvular heart diseases associated with significant morbidity and mortality. Traditional echocardiography faces limitations in availability, cost, consistency, and reliability, leading to misdiagnosis and undertreatment. The application of artificial intelligence (AI) to echocardiographic scans has the potential to address these challenges. Methods This study evaluates the performance of an AI algorithm on an external population. The algorithm utilizes deep learning networks to analyze echocardiographic exams for diagnosing atrioventricular valve disorders. We tested the algorithm on transthoracic echocardiography data collected from a single center between 2013 and 2023. The model's performance was compared to ground truth values using two classification schemes: distinguishing between normal-mild and moderate-severe regurgitation, and categorizing results into four groups: normal, mild, moderate, and severe. Results The MR cohort included 280 patients, while the TR cohort comprised 298 patients. The model demonstrated a robust ability to identify clinically significant (moderate and above) atrioventricular valve regurgitation. The MR model achieved an area under the curve (AUC) of 0.98 (95% CI: 0.97–0.99), with 91% accuracy, 95% sensitivity, and 89% specificity. In comparison, the TR model exhibited an AUC of 0.96 (95% CI: 0.94–0.98), with 84% accuracy, 91% sensitivity, and 80% specificity. Conclusion The model demonstrated high diagnostic accuracy and reliability in assessing atrioventricular valve regurgitation severity, highlighting its potential as a valuable clinical tool. The findings underscore the role of AI in complementing expert evaluations and improving access to effective diagnostics, with future applications potentially including point-of-care diagnosis and monitoring of disease progression.
{"title":"Deep learning for atrioventricular regurgitation diagnosis: an external validation study","authors":"I Cohen, J G Malins, M Cohen-Shelly, Y Asaf, M Fiman, K Faierstein, K Sudri, E Zimlichman, E Schwammenthal, R Klempfner, E Maor","doi":"10.1093/eurheartj/ehaf784.2337","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.2337","url":null,"abstract":"Background Mitral regurgitation (MR) and tricuspid regurgitation (TR) are prevalent valvular heart diseases associated with significant morbidity and mortality. Traditional echocardiography faces limitations in availability, cost, consistency, and reliability, leading to misdiagnosis and undertreatment. The application of artificial intelligence (AI) to echocardiographic scans has the potential to address these challenges. Methods This study evaluates the performance of an AI algorithm on an external population. The algorithm utilizes deep learning networks to analyze echocardiographic exams for diagnosing atrioventricular valve disorders. We tested the algorithm on transthoracic echocardiography data collected from a single center between 2013 and 2023. The model's performance was compared to ground truth values using two classification schemes: distinguishing between normal-mild and moderate-severe regurgitation, and categorizing results into four groups: normal, mild, moderate, and severe. Results The MR cohort included 280 patients, while the TR cohort comprised 298 patients. The model demonstrated a robust ability to identify clinically significant (moderate and above) atrioventricular valve regurgitation. The MR model achieved an area under the curve (AUC) of 0.98 (95% CI: 0.97–0.99), with 91% accuracy, 95% sensitivity, and 89% specificity. In comparison, the TR model exhibited an AUC of 0.96 (95% CI: 0.94–0.98), with 84% accuracy, 91% sensitivity, and 80% specificity. Conclusion The model demonstrated high diagnostic accuracy and reliability in assessing atrioventricular valve regurgitation severity, highlighting its potential as a valuable clinical tool. The findings underscore the role of AI in complementing expert evaluations and improving access to effective diagnostics, with future applications potentially including point-of-care diagnosis and monitoring of disease progression.","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"9 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.2744
A Nojiri, E Fukuro, T Okuyama, I Anan, S Morimoto, M Kawai, K Hongo
Introduction Fabry disease is an X-linked hereditary disorder due to the lack or deficiency of the alpha-galactosidase A activity, which leads to the cardiac manifestation such as left ventricular hypertrophy (LVH). Pharmacological chaperone therapy (PCT) is a promising oral treatment to prevent various complications. However, the mid-term effect of PCT on LVH in Japanese Fabry disease patients has not been investigated. Purpose We investigated the mid-term effect of PCT on LVH in Japanese Fabry disease patients. Methods We analysed echocardiographic parameters of 15 Fabry disease patients (6 males and 9 females) followed at Jikei University hospital during the treatment with PCT (4.6 ± 1.2 years). To evaluate LVH, left ventricular mass (LVM) was calculated according to Devereux’s equation and was expressed as gram/height2.7 (g/ht2.7). Results At the start of PCT, all 6 male patients had LVH while only 2 female patients had LVH. LVM was almost stable during PCT treatment both in male patients and female patients (Figure 1). The slope of the changes in LVM was 1.59 ± 1.72 g/ht2.7/year in male patients and was -0.03 ± 1.42 g/ht2.7/year in female patients, which were significantly smaller than the values previously reported without treatment (4.07 ± 1.03 g/ht2.7/year in male patients and 2.31 ± 0.81 g/ht2.7/year in female patients, p<0.05) (Figure 2). Conclusions Mid-term PCT could effectively prevent LVH progression in Japanese Fabry disease patients. Especially in female patients, LVH progression was almost completely suppressed by PCT with or without prior LVH at the start of PCT.Figure 1 Figure 2
{"title":"Mid-term benefit of pharmacological chaperone therapy on cardiac manifestation in Japanese Fabry disease","authors":"A Nojiri, E Fukuro, T Okuyama, I Anan, S Morimoto, M Kawai, K Hongo","doi":"10.1093/eurheartj/ehaf784.2744","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.2744","url":null,"abstract":"Introduction Fabry disease is an X-linked hereditary disorder due to the lack or deficiency of the alpha-galactosidase A activity, which leads to the cardiac manifestation such as left ventricular hypertrophy (LVH). Pharmacological chaperone therapy (PCT) is a promising oral treatment to prevent various complications. However, the mid-term effect of PCT on LVH in Japanese Fabry disease patients has not been investigated. Purpose We investigated the mid-term effect of PCT on LVH in Japanese Fabry disease patients. Methods We analysed echocardiographic parameters of 15 Fabry disease patients (6 males and 9 females) followed at Jikei University hospital during the treatment with PCT (4.6 ± 1.2 years). To evaluate LVH, left ventricular mass (LVM) was calculated according to Devereux’s equation and was expressed as gram/height2.7 (g/ht2.7). Results At the start of PCT, all 6 male patients had LVH while only 2 female patients had LVH. LVM was almost stable during PCT treatment both in male patients and female patients (Figure 1). The slope of the changes in LVM was 1.59 ± 1.72 g/ht2.7/year in male patients and was -0.03 ± 1.42 g/ht2.7/year in female patients, which were significantly smaller than the values previously reported without treatment (4.07 ± 1.03 g/ht2.7/year in male patients and 2.31 ± 0.81 g/ht2.7/year in female patients, p&lt;0.05) (Figure 2). Conclusions Mid-term PCT could effectively prevent LVH progression in Japanese Fabry disease patients. Especially in female patients, LVH progression was almost completely suppressed by PCT with or without prior LVH at the start of PCT.Figure 1 Figure 2","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"79 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.4120
J L Cross, S Wahi, Y Im, J M Kwan
Background Cancer patients treated with immune checkpoint inhibitors (ICIs) have an increased risk of adverse cardiovascular events (ACE) [1]. Traditional cardiovascular risk scores may not adequately capture ICI-associated cardiovascular toxicities or the unique features that contribute to ACE risk in this population [1,2]. Recent studies have developed cardiovascular risk scores for cancer patients, which achieved area under the receiver operating characteristic curve (AUC) values in the 0.65 to 0.85 range [2]. Currently, there is no validated ACE risk algorithm designed specifically for ICI patients. Purpose Our study aims 1) to develop an interpretable, machine learning-based ACE risk score algorithm for cancer patients treated with ICI therapy, and 2) to integrate this algorithm into a clinically accessible online calculator interface. Methods We analyzed 5145 cancer patients treated with ICI therapy between 2013 and 2024 at a large academic centre. Patient variables included demographics, comorbidities, laboratory values, cancer type, ICI regimen (single vs dual), and key imaging findings from echocardiography (echo) and cardiac magnetic resonance (CMR) data. The composite ACE outcome comprised myocardial infarction, coronary artery disease (CAD), arrhythmias, heart failure (HF), valvular disease, atrioventricular block, and myocarditis. Data were partitioned into training (80%), test (10%), and holdout validation (10%) sets. An extreme gradient boosting (XGB) classifier was trained using 4-fold cross-validation on the training set, and performance was evaluated on the test set. Shapley Additive Explanation (SHAP) values were used to identify top predictive features. A multivariate logistic regression model was then fit using 15 selected features (based on SHAP ranking and clinical expertise) to form the final ACE risk score algorithm, which was subsequently validated on the holdout validation set. Results ACE occurred in 36.5% of patients in our cohort. The XGB model achieved an AUC of 0.73 on the test set (Figure 1B). The most influential SHAP features included age, body mass index, cancer type, creatinine, CAD, peripheral vascular disease, stroke, HF, hypertension, left ventricular ejection fraction, and global longitudinal strain (Figure 1A, 1D). These features, along with dual ICI status and left ventricular late gadolinium enhancement, were used to train the final ACE risk algorithm, which attained an AUC of 0.70 on the holdout validation set (Figure 1C). We integrate our ACE risk algorithm into a publicly accessible, user-friendly online calculator (Figure 2). Conclusion We present a novel, interpretable, and clinically usable ACE risk score algorithm tailored to cancer patients treated with ICI therapy, which may aid in improving risk stratification and cardiovascular monitoring in this high-risk population.Fig1.Top SHAP features & AUC curves Fig2.ACE risk calculator interface
背景：接受免疫检查点抑制剂（ICIs）治疗的癌症患者发生不良心血管事件（ACE）的风险增加。传统的心血管风险评分可能无法充分捕捉到ici相关的心血管毒性或导致该人群ACE风险的独特特征[1,2]。最近的研究开发了癌症患者的心血管风险评分，其接受者工作特征曲线下面积（AUC）值在0.65 ~ 0.85[2]之间。目前，还没有专门针对ICI患者设计的经过验证的ACE风险算法。本研究旨在1)为接受ICI治疗的癌症患者开发一种可解释的、基于机器学习的ACE风险评分算法；2)将该算法整合到临床可访问的在线计算器界面中。方法：我们分析了2013年至2024年在一家大型学术中心接受ICI治疗的5145例癌症患者。患者变量包括人口统计学、合并症、实验室值、癌症类型、ICI方案（单/双）以及超声心动图（echo）和心脏磁共振（CMR）数据的关键成像结果。ACE的复合结局包括心肌梗死、冠状动脉疾病（CAD）、心律失常、心力衰竭（HF）、瓣膜疾病、房室传导阻滞和心肌炎。数据被划分为训练集（80%）、测试集（10%）和坚持验证集（10%）。在训练集上使用4倍交叉验证训练极端梯度增强（XGB）分类器，并在测试集上评估性能。Shapley加性解释（SHAP）值用于识别顶级预测特征。然后使用15个选择的特征（基于SHAP排名和临床专业知识）拟合多元逻辑回归模型，形成最终的ACE风险评分算法，随后在拒绝验证集上进行验证。结果：ACE发生率为36.5%。XGB模型在测试集上的AUC为0.73（图1B）。最具影响的SHAP特征包括年龄、体重指数、癌症类型、肌酐、CAD、外周血管疾病、卒中、心衰、高血压、左心室射血分数和整体纵向应变（图1A， 1D）。这些特征，以及双ICI状态和左心室晚期钆增强，被用于训练最终的ACE风险算法，该算法在holdout验证集上获得了0.70的AUC（图1C）。我们将ACE风险算法集成到一个可公开访问的、用户友好的在线计算器中（图2）。我们提出了一种新的、可解释的、临床可用的ACE风险评分算法，该算法适用于接受ICI治疗的癌症患者，可能有助于改善这一高危人群的风险分层和心血管监测。图2. Top shape特征& AUC曲线ACE风险计算器界面
{"title":"A novel machine learning-based adverse cardiovascular events risk score for cancer patients treated with immune checkpoint inhibitors","authors":"J L Cross, S Wahi, Y Im, J M Kwan","doi":"10.1093/eurheartj/ehaf784.4120","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.4120","url":null,"abstract":"Background Cancer patients treated with immune checkpoint inhibitors (ICIs) have an increased risk of adverse cardiovascular events (ACE) [1]. Traditional cardiovascular risk scores may not adequately capture ICI-associated cardiovascular toxicities or the unique features that contribute to ACE risk in this population [1,2]. Recent studies have developed cardiovascular risk scores for cancer patients, which achieved area under the receiver operating characteristic curve (AUC) values in the 0.65 to 0.85 range [2]. Currently, there is no validated ACE risk algorithm designed specifically for ICI patients. Purpose Our study aims 1) to develop an interpretable, machine learning-based ACE risk score algorithm for cancer patients treated with ICI therapy, and 2) to integrate this algorithm into a clinically accessible online calculator interface. Methods We analyzed 5145 cancer patients treated with ICI therapy between 2013 and 2024 at a large academic centre. Patient variables included demographics, comorbidities, laboratory values, cancer type, ICI regimen (single vs dual), and key imaging findings from echocardiography (echo) and cardiac magnetic resonance (CMR) data. The composite ACE outcome comprised myocardial infarction, coronary artery disease (CAD), arrhythmias, heart failure (HF), valvular disease, atrioventricular block, and myocarditis. Data were partitioned into training (80%), test (10%), and holdout validation (10%) sets. An extreme gradient boosting (XGB) classifier was trained using 4-fold cross-validation on the training set, and performance was evaluated on the test set. Shapley Additive Explanation (SHAP) values were used to identify top predictive features. A multivariate logistic regression model was then fit using 15 selected features (based on SHAP ranking and clinical expertise) to form the final ACE risk score algorithm, which was subsequently validated on the holdout validation set. Results ACE occurred in 36.5% of patients in our cohort. The XGB model achieved an AUC of 0.73 on the test set (Figure 1B). The most influential SHAP features included age, body mass index, cancer type, creatinine, CAD, peripheral vascular disease, stroke, HF, hypertension, left ventricular ejection fraction, and global longitudinal strain (Figure 1A, 1D). These features, along with dual ICI status and left ventricular late gadolinium enhancement, were used to train the final ACE risk algorithm, which attained an AUC of 0.70 on the holdout validation set (Figure 1C). We integrate our ACE risk algorithm into a publicly accessible, user-friendly online calculator (Figure 2). Conclusion We present a novel, interpretable, and clinically usable ACE risk score algorithm tailored to cancer patients treated with ICI therapy, which may aid in improving risk stratification and cardiovascular monitoring in this high-risk population.Fig1.Top SHAP features & AUC curves Fig2.ACE risk calculator interface","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"301 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1093/eurheartj/ehaf784.4388
S Shibata, M Nishimori, Y Nishihara, M Shinohara
Background In clinical practice, physicians integrate multiple diagnostic tests to infer pathophysiology, but no AI models have been developed to replicate this complex decision-making process. A model capable of interpreting multimodal data within a unified space is required. For instance, in heart failure, chest X-rays and ECGs, while representing different modalities, both reflect the same underlying pathological condition. Purpose This study aims to develop a multimodal model leveraging the CLIP framework to classify heart failure pathophysiology by embedding data from distinct modalities into a shared representational space. Methods We analyzed data from cardiology patients at our University between January 2012 and December 2022, selecting only those who had concurrent ECG, chest X-rays, and echocardiography. The dataset comprised 9,632 patients, with 34,747 12-lead ECGs and 36,366 chest X-rays. CLIP was employed to pair ECGs and chest X-rays from the same patients. Using these vectors, the model was trained to predict LVEF and E/E' values from transthoracic echocardiograms. The model then predicted the classification of each criteria, based on the cut-off values of 50 for LVEF and 15 for E/E'. Results Dimensionality reduction of the vectors obtained from ECGs and chest X-rays demonstrated that both modalities were projected within the same representational space. Figure 1 shows the result of embedding ECG and chest X-ray data into a common feature space using CLIP, with dimensionality reduced to two dimensions using UMAP. Each point in the figure represents a single ECG or chest X-ray data sample, color-coded based on BNP values transformed into a log scale. Samples with high BNP values were distributed in the upper right, while those with low BNP values were located toward the lower left. This indicates that the pathophysiology of heart failure, as inferred from ECGs and chest X-rays, was successfully reproduced within this feature space. In the Figure 1, ECG and chest X-ray data were plotted with overlapping distributions, demonstrating that these modalities can be represented within the same feature space. The feature vectors obtained through CLIP were treated equivalently for ECG and chest X-rays, and the model was trained to predict E/E' and LVEF values. As a result, as shown in Figure 2, the model achieved an AUROC of 0.83 for predicting LVEF < 50 and an AUROC of 0.77 for predicting E/E' > 15. These results suggest that our model successfully classified the pathophysiology of heart failure. Conclusions This study demonstrates that the CLIP model can successfully classify heart failure pathophysiology by embedding multimodal data into a unified representational space.Figure1 Figure2
{"title":"A CLIP-based multimodal model for heart failure assessment","authors":"S Shibata, M Nishimori, Y Nishihara, M Shinohara","doi":"10.1093/eurheartj/ehaf784.4388","DOIUrl":"https://doi.org/10.1093/eurheartj/ehaf784.4388","url":null,"abstract":"Background In clinical practice, physicians integrate multiple diagnostic tests to infer pathophysiology, but no AI models have been developed to replicate this complex decision-making process. A model capable of interpreting multimodal data within a unified space is required. For instance, in heart failure, chest X-rays and ECGs, while representing different modalities, both reflect the same underlying pathological condition. Purpose This study aims to develop a multimodal model leveraging the CLIP framework to classify heart failure pathophysiology by embedding data from distinct modalities into a shared representational space. Methods We analyzed data from cardiology patients at our University between January 2012 and December 2022, selecting only those who had concurrent ECG, chest X-rays, and echocardiography. The dataset comprised 9,632 patients, with 34,747 12-lead ECGs and 36,366 chest X-rays. CLIP was employed to pair ECGs and chest X-rays from the same patients. Using these vectors, the model was trained to predict LVEF and E/E' values from transthoracic echocardiograms. The model then predicted the classification of each criteria, based on the cut-off values of 50 for LVEF and 15 for E/E'. Results Dimensionality reduction of the vectors obtained from ECGs and chest X-rays demonstrated that both modalities were projected within the same representational space. Figure 1 shows the result of embedding ECG and chest X-ray data into a common feature space using CLIP, with dimensionality reduced to two dimensions using UMAP. Each point in the figure represents a single ECG or chest X-ray data sample, color-coded based on BNP values transformed into a log scale. Samples with high BNP values were distributed in the upper right, while those with low BNP values were located toward the lower left. This indicates that the pathophysiology of heart failure, as inferred from ECGs and chest X-rays, was successfully reproduced within this feature space. In the Figure 1, ECG and chest X-ray data were plotted with overlapping distributions, demonstrating that these modalities can be represented within the same feature space. The feature vectors obtained through CLIP were treated equivalently for ECG and chest X-rays, and the model was trained to predict E/E' and LVEF values. As a result, as shown in Figure 2, the model achieved an AUROC of 0.83 for predicting LVEF &lt; 50 and an AUROC of 0.77 for predicting E/E' &gt; 15. These results suggest that our model successfully classified the pathophysiology of heart failure. Conclusions This study demonstrates that the CLIP model can successfully classify heart failure pathophysiology by embedding multimodal data into a unified representational space.Figure1 Figure2","PeriodicalId":11976,"journal":{"name":"European Heart Journal","volume":"28 1","pages":""},"PeriodicalIF":39.3,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146122139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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