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Beyond the postmortem diagnosis of hypertrophic cardiomyopathy: a genetic approach guided by clinical and morphological features in Noonan syndrome. 超越 HCM 的尸检诊断:以努南综合征的临床和形态特征为指导的遗传学方法。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-25 DOI: 10.1016/j.rec.2025.01.007
Elena Sola-García, Birethine Sathiyanathan, Alba María García-García, Mary N Sheppard, María Teresa Tomé-Esteban
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引用次数: 0
Machine learning prediction of in-hospital mortality and external validation in patients with cardiogenic shock: the RESCUE score.
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-22 DOI: 10.1016/j.rec.2025.01.003
Ji Hyun Cha, Ki Hong Choi, Chul-Min Ahn, Cheol Woong Yu, Ik Hyun Park, Woo Jin Jang, Hyun-Joong Kim, Jang-Whan Bae, Sung Uk Kwon, Hyun-Jong Lee, Wang Soo Lee, Jin-Ok Jeong, Sang-Don Park, Taek Kyu Park, Joo Myung Lee, Young Bin Song, Joo-Yong Hahn, Seung-Hyuk Choi, Hyeon-Cheol Gwon, Jeong Hoon Yang

Introduction and objectives: Despite advances in mechanical circulatory support, mortality rates in cardiogenic shock (CS) remain high. A reliable risk stratification system could serve as a valuable guide in the clinical management of patients with CS. This study aimed to develop and externally validate a risk prediction model for in-hospital mortality in CS patients using machine learning (ML) algorithms.

Methods: Data from 1247 patients with all-cause CS in the RESCUE registry (January 2014-December 2018) were analyzed. Key predictive variables were identified using 4 ML algorithms. A risk prediction model, the RESCUE score, was developed using logistic regression based on the selected variables. Internal validation was conducted within the RESCUE registry, and external validation was performed using an independent CS registry of 750 patients.

Results: The 4 ML models identified 7 predictors: age, vasoactive inotropic score, left ventricular ejection fraction, lactic acid level, in-hospital cardiac arrest at presentation, need for continuous renal replacement therapy, and mechanical ventilation. The RESCUE score demonstrated strong predictive performance, with an AUC of 0.86 (95%CI, 0.83-0.88) for in-hospital mortality. Ten-fold internal cross-validation yielded an AUC of 0.86 (95%CI, 0.77-0.95). External validation showed an AUC of 0.80 (95%CI, 0.76-0.84).

Conclusions: Our ML-based risk-scoring system, the RESCUE score, demonstrated excellent predictive performance for in-hospital mortality in all patients with CS, regardless of cause. The system could be a useful and reliable tool to estimate risk stratification of CS in everyday clinical practice.

Clinical trial registration: NCT02985008.

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引用次数: 0
Temporal trends in implantable cardioverter-defibrillator prescription as primary prevention in dilated cardiomyopathy.
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-22 DOI: 10.1016/j.rec.2024.11.017
Carlos Moliner-Abós, Meritxell Santaló-Corcoy, Javier Meseguer-Donlo, Andrea Crehuet-Llonch, Bieito Campos García, Sònia Mirabet Pérez
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引用次数: 0
Risk assessment of pregnant patients with heart disease: mWHO vs CARPREG II risk scores.
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-22 DOI: 10.1016/j.rec.2025.01.002
Antonia Pijuan-Domenech, Laura Galian-Gay, Javier Cantalapiedra, Berta Serrano, Maria Goya, Nerea Maiz
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引用次数: 0
Frailty and invasive management of NSTEACS: lights and shadows. NSTEACS的脆弱性和侵入性治疗:光与影。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-18 DOI: 10.1016/j.rec.2025.01.001
Francisco Buendía-Santiago, Antonio Meseguer-Hernández, José Manuel Andreu-Cayuelas, Juan Antonio Castillo-Moreno
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引用次数: 0
Hereditary transthyretin amyloidosis caused by the Val142Ile variant in Spain. 西班牙Val142Ile变异引起的遗传性甲状腺转蛋白淀粉样变。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-17 DOI: 10.1016/j.rec.2024.12.012
Fernando de Frutos, Lorena Herrador, Belén Peiró-Aventín, Rocío Eiros, Javier Limeres Freire, Esther Zorio, Álvaro Carbayo, Pasqual Llongueras-Espí, Ana García-Álvarez, Tomás Ripoll-Vera, Rosa Macías, Silvia Vilches, Sonia Ruiz-Bustillo, Xabier Arana-Achaga, Jara Gayán Ordás, Jesús Piqueras-Flores, María Ruiz-Cueto, Carlos Casasnovas, Coloma Tirón, Ricard Rojas-García, Teresa Sevilla, José Fernando Rodríguez-Palomares, Esther González-López, Eduardo Villacorta, Pablo García-Pavía, José González-Costello

Introduction and objectives: In recent years, several cases of hereditary transthyretin amyloidosis (ATTRv) due to the p.Val142Ile variant have been described in patients without African ancestry. The aim of this study was to analyze the impact of ATTRv caused by p.Val142Ile in Spain, focusing on its phenotypic characteristics and its population frequency.

Methods: Patients diagnosed with ATTRv caused by p.Val142Ile, as well as carriers irrespective of their phenotype, were recruited from 16 centers in Spain. Baseline characteristics and events during follow-up were retrieved. Population frequency was assessed using data from the Spanish National DNA Bank (N=3569) and the Catalan Health Databank (N=790).

Results: The cohort included 164 participants: 75 probands (45.7%) and 89 relatives (54.3%). Among the probands, the mean age was 73.9±8.5 years, and 47 (62.7%) were male. Sixty-seven probands (89.3%) reported European ancestry, while only 6 (8%) reported African ancestry. Cardiac symptoms were the most frequent reason for ATTRv diagnosis (n=64; 85.3%). The median follow-up was 2.6 years [Interquartile range, 1.5-4.1]. Overall penetrance at ages 65, 75, and 85 years was 12.8%, 44.3%, and 94.2%, respectively. Tafamidis was initiated during follow-up in 38 patients: after 1 year of treatment, 14 patients (38.9%) met the combined endpoint (12 experienced disease progression and 2 died from cardiovascular causes). Population frequency was estimated to range between 0.0% and 0.12%, based on data from the Spanish and Catalan databases, respectively.

Conclusions: ATTRv caused by p.Val142Ile has a significant prevalence in Spain. Its phenotypic features are characterized by late onset, male predominance, and cardiac involvement.

简介和目的:近年来,在没有非洲血统的患者中报道了几例由p.Val142Ile变异引起的遗传性甲状腺转蛋白淀粉样变性(ATTRv)。本研究的目的是分析p.Val142Ile在西班牙引起的ATTRv的影响,重点分析其表型特征和种群频率。方法:从西班牙的16个中心招募被诊断为p.Val142Ile引起的ATTRv的患者,以及无论其表型如何的携带者。检索随访期间的基线特征和事件。使用西班牙国家DNA银行(N = 3569)和加泰罗尼亚健康数据库(N = 790)的数据评估人群频率。结果:共纳入164名受试者,其中先证者75人(45.7%),亲属89人(54.3%)。先证者平均年龄73.9±8.5岁,男性47例(62.7%)。67名先证者(89.3%)报告了欧洲血统,而只有6名(8%)报告了非洲血统。心脏症状是诊断ATTRv最常见的原因(n = 64;85.3%)。中位随访时间为2.6年[四分位数间距,1.5-4.1]。65岁、75岁和85岁的总外显率分别为12.8%、44.3%和94.2%。38例患者在随访期间开始他法非地治疗:治疗1年后,14例患者(38.9%)达到联合终点(12例出现疾病进展,2例死于心血管原因)。根据分别来自西班牙和加泰罗尼亚数据库的数据,人口频率估计在0.0%至0.12%之间。结论:在西班牙由p.Val142Ile引起的ATTRv患病率较高。其表型特征为发病晚、男性为主、累及心脏。
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引用次数: 0
Interatrial conduction block as a consequence of wide antral pulmonary vein isolation. 宽肺静脉隔离导致的房间传导阻滞。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-14 DOI: 10.1016/j.rec.2024.11.016
Amaia Martínez León, David Testa Alonso, María Salgado, Minel Soroa, Daniel García-Iglesias, David Calvo
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引用次数: 0
Balloon atrioseptostomy for transposition of the great arteries in Europe: characteristics and outcomes. 欧洲大动脉转位的球囊房间隔造口术:特点和结果。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-10 DOI: 10.1016/j.rec.2024.12.011
Hugues Lucron, Sophie-Guiti Malekzadeh-Milani, Thomas Perouse de Montclos, Alban-Elouen Baruteau, Alberto Mendoza Soto, Gianfranco Butera, Ina Michel-Behnke, Caroline Ovaert, Carles Bautista-Rodriguez, James Bentham, Zakaria Jalal, Pedro Betrian Blasco, Hélène Bouvaist, Marie Vincenti, José Diego Ferreira Matins, Jean-Marc Jellimann, Alessia Callegari, Laurent Bonnemains, Ronan Bonnefoy, Biagio Castaldi, Anne Charbonneau, Claire Dauphin, Bruno Lefort, Stephan Schubert, Mélanie Brard, Olivia Domanski, Charlotte Denis, Julie Wacker, Pascale Maragnes, Clément Karsenty, Petra Loureiro, André Jakob, Sébastien Hascoët, Damien Bonnet

Introduction and objectives: Balloon atrial septostomy (BAS) improves oxygenation in neonates with transposition of the great arteries (TGA) and restrictive foramen ovale. Currently, there is a global shortage of dedicated BAS catheters, while new unmarked catheters have recently become available at some European centers. This study aimed to characterize BAS outcomes using the currently available BAS catheters in Europe.

Methods: A 2-year multicenter observational registry was conducted, including all neonates undergoing BAS for TGA. We report preliminary results (September 2022-February 2024) focusing on BAS characteristics and outcomes.

Results: A total of 250 BAS procedures were performed in 29 centers. The median neonatal weight was 3.16 kg, and 88% of neonates had a prenatal diagnosis. Most procedures were performed often on the first day of life during working hours (72.8%), mainly in catheterization laboratories (59.2%). Guidance primarily involved ultrasound with or without fluoroscopy. A guidewire was used in 41.2% of procedures. A total of 290 catheters (286 Z-5 or Z-6) were used, achieving an overall BAS success rate of 96%. Complete procedural failure was associated with the use of the umbilical venous route (OR, 3.62; P = .001) and lower-volume catheters (OR, 7.01; P < .001). The occurrence of significant complications (8%; OR, 9.33; P < .001) was associated with complete procedural failure. For complex procedures, significant risk factors were the absence of fluoroscopy (OR, 3.32; P = .001), use of the umbilical venous route (OR, 2.28; P = .005), and lower-volume catheters (OR, 2.43; P = .03).

Conclusions: In the current era, BAS can be challenging, and significant complications and complete failures are not uncommon. The use of the umbilical venous route, low-volume BAS catheters, absence of fluoroscopy guidance, and the occurrence of complications negatively impact procedural outcomes.

简介和目的:球囊房间隔造口术(BAS)改善新生儿大动脉转位(TGA)和限制性卵圆孔的氧合。目前,全球缺乏专用的BAS导管,而新的无标记导管最近在一些欧洲中心可用。本研究旨在描述目前在欧洲使用BAS导管的BAS结果。方法:进行了为期2年的多中心观察登记,包括所有因TGA而接受BAS的新生儿。我们报告了初步结果(2022年9月至2024年2月),重点关注BAS的特征和结果。结果:29个中心共进行了250例BAS手术。新生儿体重中位数为3.16 kg, 88%的新生儿有产前诊断。大多数手术通常在出生第一天的工作时间进行(72.8%),主要在导管室进行(59.2%)。指导主要包括超声检查和透视检查。41.2%的手术使用导丝。共使用290根导管(286根Z-5或Z-6),总体BAS成功率为96%。完全手术失败与使用脐静脉路径相关(OR, 3.62;P = .001)和小容量导管(OR, 7.01;P < 0.001)。严重并发症的发生(8%;或者,9.33;P < 0.001)与完全手术失败相关。对于复杂的手术,重要的危险因素是没有透视检查(OR, 3.32;P = .001),使用脐静脉途径(OR, 2.28;P = 0.005),低容量导管(OR, 2.43;P = .03)。结论:在当今时代,BAS可能具有挑战性,严重的并发症和完全失败并不罕见。使用脐静脉路径、小容量BAS导管、缺乏透视指导以及并发症的发生对手术结果产生负面影响。
{"title":"Balloon atrioseptostomy for transposition of the great arteries in Europe: characteristics and outcomes.","authors":"Hugues Lucron, Sophie-Guiti Malekzadeh-Milani, Thomas Perouse de Montclos, Alban-Elouen Baruteau, Alberto Mendoza Soto, Gianfranco Butera, Ina Michel-Behnke, Caroline Ovaert, Carles Bautista-Rodriguez, James Bentham, Zakaria Jalal, Pedro Betrian Blasco, Hélène Bouvaist, Marie Vincenti, José Diego Ferreira Matins, Jean-Marc Jellimann, Alessia Callegari, Laurent Bonnemains, Ronan Bonnefoy, Biagio Castaldi, Anne Charbonneau, Claire Dauphin, Bruno Lefort, Stephan Schubert, Mélanie Brard, Olivia Domanski, Charlotte Denis, Julie Wacker, Pascale Maragnes, Clément Karsenty, Petra Loureiro, André Jakob, Sébastien Hascoët, Damien Bonnet","doi":"10.1016/j.rec.2024.12.011","DOIUrl":"https://doi.org/10.1016/j.rec.2024.12.011","url":null,"abstract":"<p><strong>Introduction and objectives: </strong>Balloon atrial septostomy (BAS) improves oxygenation in neonates with transposition of the great arteries (TGA) and restrictive foramen ovale. Currently, there is a global shortage of dedicated BAS catheters, while new unmarked catheters have recently become available at some European centers. This study aimed to characterize BAS outcomes using the currently available BAS catheters in Europe.</p><p><strong>Methods: </strong>A 2-year multicenter observational registry was conducted, including all neonates undergoing BAS for TGA. We report preliminary results (September 2022-February 2024) focusing on BAS characteristics and outcomes.</p><p><strong>Results: </strong>A total of 250 BAS procedures were performed in 29 centers. The median neonatal weight was 3.16 kg, and 88% of neonates had a prenatal diagnosis. Most procedures were performed often on the first day of life during working hours (72.8%), mainly in catheterization laboratories (59.2%). Guidance primarily involved ultrasound with or without fluoroscopy. A guidewire was used in 41.2% of procedures. A total of 290 catheters (286 Z-5 or Z-6) were used, achieving an overall BAS success rate of 96%. Complete procedural failure was associated with the use of the umbilical venous route (OR, 3.62; P = .001) and lower-volume catheters (OR, 7.01; P < .001). The occurrence of significant complications (8%; OR, 9.33; P < .001) was associated with complete procedural failure. For complex procedures, significant risk factors were the absence of fluoroscopy (OR, 3.32; P = .001), use of the umbilical venous route (OR, 2.28; P = .005), and lower-volume catheters (OR, 2.43; P = .03).</p><p><strong>Conclusions: </strong>In the current era, BAS can be challenging, and significant complications and complete failures are not uncommon. The use of the umbilical venous route, low-volume BAS catheters, absence of fluoroscopy guidance, and the occurrence of complications negatively impact procedural outcomes.</p>","PeriodicalId":38430,"journal":{"name":"Revista española de cardiología (English ed.)","volume":" ","pages":""},"PeriodicalIF":7.2,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142972530","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}
引用次数: 0
4D-flow cardiac magnetic resonance in repaired anomalous pulmonary venous drainage. 4d血流心脏磁共振在修复异常肺静脉引流中的应用。
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-09 DOI: 10.1016/j.rec.2024.11.015
Javier Urmeneta Ulloa, Alberto Forteza Gil, José Ángel Cabrera
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引用次数: 0
Impact of coronary artery tortuosity on outcomes following stenting with newer-generation drug-eluting stents. An analysis of the randomized BIOFLOW trials.
IF 7.2 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-04 DOI: 10.1016/j.rec.2024.12.009
Nader Mankerious, Ralph Toelg, Mohammad Abdelghani, Hector M Garcia-Garcia, Serdar Farhan, Abdelhakim Allali, Stephan Windecker, Thierry Lefèvre, Shigeru Saito, David E Kandzari, Ron Waksman, Gert Richardt, Rayyan Hemetsberger

Introduction and objectives: Patients undergoing percutaneous coronary intervention in vessels with moderate-to-severe tortuosity are at higher risk of adverse outcomes, but data are scarce in the era of newer-generation stents. We compared outcomes following percutaneous coronary intervention in vessels with moderate-to-severe tortuosity using a bioresorbable-polymer sirolimus-eluting stent (BP-SES) vs a durable-polymer everolimus-eluting stent (DP-EES).

Methods: A total of 2350 patients from the BIOFLOW II, IV, and V randomized trials were stratified into 2 groups based on target-vessel tortuosity: none-to-mild and moderate-to-severe. The primary endpoints included target lesion failure (TLF)-a composite of cardiac death, target-vessel myocardial infarction (TV-MI), or ischemia-driven target lesion revascularization (TLR)-and probable/definite stent thrombosis at 3 years.

Results: Patients with moderate-to-severe tortuosity (n=903) had more comorbidities than those with none-to-mild tortuosity (n=1447). Rates of TLF (P=.354), cardiac death (P=.690), TLR (P=.447), and stent thrombosis (P=.084) were similar between the 2 groups, whereas TV-MI occurred more frequently in the moderate-to-severe tortuosity group (P=.031). However, on multivariate analysis, moderate-to-severe tortuosity was not an independent predictor of TV-MI (adjusted HR, 1.06; 95% CI, 0.72-1.55; P=.772). Among patients with moderate-to-severe tortuosity, the use of BP-SES was associated with significantly lower rates of TLF compared with the durable-polymer everolimus-eluting stent (7.8% vs 13.4%; HR, 0.57; 95% CI, 0.37-0.87; P=.009), driven by reductions in TV-MI (5.0% vs 9.2%; HR, 0.54; 95% CI, 0.32-0.90; P=.018) and TLR (2.7% vs 6.1%; HR, 0.45; 95% CI, 0.23-0.90; P=.021).

Conclusions: This pooled analysis of the randomized BIOFLOW trials demonstrates that patients with none-to-mild and moderate-to-severe tortuosity have comparable long-term adverse event rates. However, the use of BP-SES in patients with moderate-to-severe tortuosity may help mitigate potential ischemic risks.

Clinical trial registration: Clinicaltrials.gov NCT01356888, NCT01939249, NCT02389946.

{"title":"Impact of coronary artery tortuosity on outcomes following stenting with newer-generation drug-eluting stents. An analysis of the randomized BIOFLOW trials.","authors":"Nader Mankerious, Ralph Toelg, Mohammad Abdelghani, Hector M Garcia-Garcia, Serdar Farhan, Abdelhakim Allali, Stephan Windecker, Thierry Lefèvre, Shigeru Saito, David E Kandzari, Ron Waksman, Gert Richardt, Rayyan Hemetsberger","doi":"10.1016/j.rec.2024.12.009","DOIUrl":"https://doi.org/10.1016/j.rec.2024.12.009","url":null,"abstract":"<p><strong>Introduction and objectives: </strong>Patients undergoing percutaneous coronary intervention in vessels with moderate-to-severe tortuosity are at higher risk of adverse outcomes, but data are scarce in the era of newer-generation stents. We compared outcomes following percutaneous coronary intervention in vessels with moderate-to-severe tortuosity using a bioresorbable-polymer sirolimus-eluting stent (BP-SES) vs a durable-polymer everolimus-eluting stent (DP-EES).</p><p><strong>Methods: </strong>A total of 2350 patients from the BIOFLOW II, IV, and V randomized trials were stratified into 2 groups based on target-vessel tortuosity: none-to-mild and moderate-to-severe. The primary endpoints included target lesion failure (TLF)-a composite of cardiac death, target-vessel myocardial infarction (TV-MI), or ischemia-driven target lesion revascularization (TLR)-and probable/definite stent thrombosis at 3 years.</p><p><strong>Results: </strong>Patients with moderate-to-severe tortuosity (n=903) had more comorbidities than those with none-to-mild tortuosity (n=1447). Rates of TLF (P=.354), cardiac death (P=.690), TLR (P=.447), and stent thrombosis (P=.084) were similar between the 2 groups, whereas TV-MI occurred more frequently in the moderate-to-severe tortuosity group (P=.031). However, on multivariate analysis, moderate-to-severe tortuosity was not an independent predictor of TV-MI (adjusted HR, 1.06; 95% CI, 0.72-1.55; P=.772). Among patients with moderate-to-severe tortuosity, the use of BP-SES was associated with significantly lower rates of TLF compared with the durable-polymer everolimus-eluting stent (7.8% vs 13.4%; HR, 0.57; 95% CI, 0.37-0.87; P=.009), driven by reductions in TV-MI (5.0% vs 9.2%; HR, 0.54; 95% CI, 0.32-0.90; P=.018) and TLR (2.7% vs 6.1%; HR, 0.45; 95% CI, 0.23-0.90; P=.021).</p><p><strong>Conclusions: </strong>This pooled analysis of the randomized BIOFLOW trials demonstrates that patients with none-to-mild and moderate-to-severe tortuosity have comparable long-term adverse event rates. However, the use of BP-SES in patients with moderate-to-severe tortuosity may help mitigate potential ischemic risks.</p><p><strong>Clinical trial registration: </strong>Clinicaltrials.gov NCT01356888, NCT01939249, NCT02389946.</p>","PeriodicalId":38430,"journal":{"name":"Revista española de cardiología (English ed.)","volume":" ","pages":""},"PeriodicalIF":7.2,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143075657","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}
引用次数: 0
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Revista española de cardiología (English ed.)
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