We report two cases of hypertrophic cardiomyopathy in young adults, both presenting with characteristic familial and clinical features, along with imaging abnormalities. Further evaluation revealed the underlying diagnosis to be Danon disease—an X-linked dominant disorder caused by mutations in the LAMP-2 gene. A comprehensive Medline search revealed a lack of published Indian literature specifically addressing Danon disease. Here, we present the clinical profiles and cardiac MRI findings of these two genetically confirmed cases.
{"title":"Cardiac MRI in Danon's Disease—A Phenocopy of Hypertrophic Cardiomyopathy in Young Adults","authors":"Mayank Goyal, Vineeta Ojha, Shyam Murti Bohare, Abhishek Dhiman, Priya Jagia","doi":"10.1111/echo.70337","DOIUrl":"10.1111/echo.70337","url":null,"abstract":"<div>\u0000 \u0000 <p>We report two cases of hypertrophic cardiomyopathy in young adults, both presenting with characteristic familial and clinical features, along with imaging abnormalities. Further evaluation revealed the underlying diagnosis to be Danon disease—an X-linked dominant disorder caused by mutations in the LAMP-2 gene. A comprehensive Medline search revealed a lack of published Indian literature specifically addressing Danon disease. Here, we present the clinical profiles and cardiac MRI findings of these two genetically confirmed cases.</p>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145514778","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Cancer therapy-related cardiac dysfunction (CTRCD) remains a critical limitation of cancer therapy, with implications for morbidity and survivorship. Echocardiography is an established surveillance tool, while electrocardiography (ECG) may offer earlier, low-cost detection of subclinical cardiotoxicity. The relative diagnostic yield of these modalities is uncertain.
Aim
To systematically review and compare echocardiography and ECG for early detection of CTRCD in patients receiving cancer therapy.
Methods
Following PRISMA 2020 guidelines, PubMed, Embase, Scopus, and CENTRAL were searched through May 2025. Eligible studies included prospective or retrospective cohorts reporting echocardiographic and ECG outcomes in adults undergoing chemotherapy. Data on left ventricular ejection fraction (LVEF), diastolic function, global longitudinal strain (GLS), and ECG abnormalities were extracted. Pooled incidence and effect estimates were calculated using random-effects models. Risk of bias was assessed with QUADAS-2.
Results
Thirteen cohort studies involving 1440 patients were included. The pooled incidence of echo-defined CTRCD was 10% (95% confidence interval [CI] 7%–16%), with higher rates among anthracycline-treated cohorts. Diastolic dysfunction and GLS reduction occurred in up to 40% of patients, frequently preceding a decline in LVEF. ECG abnormalities were observed in 35% (95% CI 22%–49%), most commonly QTc prolongation, ST–T changes, fragmented QRS, and atrial fibrillation. Routine ECG demonstrated low sensitivity compared with echocardiography, though continuous monitoring and AI-enhanced ECG showed potential for earlier detection.
Conclusions
Echocardiography remains the cornerstone for CTRCD surveillance, with GLS and diastolic indices providing early warning. ECG abnormalities are frequent but inconsistent predictors; their role may expand with continuous and AI-based approaches. A multimodal surveillance strategy integrating echo and advanced ECG may improve cardio-oncology care.
癌症治疗相关性心功能障碍（CTRCD）仍然是癌症治疗的一个关键限制，对发病率和生存率有影响。超声心动图是一种成熟的监测工具，而心电图（ECG）可以提供更早，低成本的亚临床心脏毒性检测。这些模式的相对诊断结果是不确定的。目的系统评价和比较超声心动图与心电图在肿瘤治疗中早期发现CTRCD的价值。方法按照PRISMA 2020指南，检索PubMed、Embase、Scopus和CENTRAL至2025年5月。符合条件的研究包括报告接受化疗的成人超声心动图和心电图结果的前瞻性或回顾性队列。提取左室射血分数（LVEF）、舒张功能、总纵应变（GLS）和心电图异常数据。使用随机效应模型计算合并发生率和效应估计值。采用QUADAS-2评估偏倚风险。结果纳入13项队列研究，共1440例患者。超声定义CTRCD的总发生率为10%(95%可信区间[CI] 7%-16%)，蒽环类药物治疗组的发生率更高。高达40%的患者发生舒张功能障碍和GLS降低，通常发生在LVEF下降之前。心电图异常占35% (95% CI 22%-49%)，最常见的是QTc延长、ST-T改变、QRS片段化和房颤。与超声心动图相比，常规心电图的灵敏度较低，尽管持续监测和人工智能增强心电图显示出早期发现的潜力。结论超声心动图仍然是CTRCD监测的基础，GLS和舒张指数可提供早期预警。心电图异常是频繁但不一致的预测指标；它们的作用可能会随着持续和基于人工智能的方法而扩大。综合超声和先进心电图的多模式监测策略可能改善心脏肿瘤护理。
{"title":"“Electrocardiographic and Echocardiographic Monitoring for Early Chemotherapy-Induced Cardiotoxicity: A Systematic Review and Meta-Analysis”","authors":"Param Darpan Sheth, Chandana Srinath, Prasad Mayagondanahallay Chandrashekaraiah, Radha Wajapey Madhukar, Manjappa Mahadevappa","doi":"10.1111/echo.70331","DOIUrl":"https://doi.org/10.1111/echo.70331","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Cancer therapy-related cardiac dysfunction (CTRCD) remains a critical limitation of cancer therapy, with implications for morbidity and survivorship. Echocardiography is an established surveillance tool, while electrocardiography (ECG) may offer earlier, low-cost detection of subclinical cardiotoxicity. The relative diagnostic yield of these modalities is uncertain.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To systematically review and compare echocardiography and ECG for early detection of CTRCD in patients receiving cancer therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Following PRISMA 2020 guidelines, PubMed, Embase, Scopus, and CENTRAL were searched through May 2025. Eligible studies included prospective or retrospective cohorts reporting echocardiographic and ECG outcomes in adults undergoing chemotherapy. Data on left ventricular ejection fraction (LVEF), diastolic function, global longitudinal strain (GLS), and ECG abnormalities were extracted. Pooled incidence and effect estimates were calculated using random-effects models. Risk of bias was assessed with QUADAS-2.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Thirteen cohort studies involving 1440 patients were included. The pooled incidence of echo-defined CTRCD was 10% (95% confidence interval [CI] 7%–16%), with higher rates among anthracycline-treated cohorts. Diastolic dysfunction and GLS reduction occurred in up to 40% of patients, frequently preceding a decline in LVEF. ECG abnormalities were observed in 35% (95% CI 22%–49%), most commonly QTc prolongation, ST–T changes, fragmented QRS, and atrial fibrillation. Routine ECG demonstrated low sensitivity compared with echocardiography, though continuous monitoring and AI-enhanced ECG showed potential for earlier detection.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Echocardiography remains the cornerstone for CTRCD surveillance, with GLS and diastolic indices providing early warning. ECG abnormalities are frequent but inconsistent predictors; their role may expand with continuous and AI-based approaches. A multimodal surveillance strategy integrating echo and advanced ECG may improve cardio-oncology care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145470018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This study aimed to determine whether left atrial (LA) strain can identify early alterations in LA function in diffuse large B-cell lymphoma (DLBCL) patients treated with R-CHOP chemotherapy regimen and whether these alterations can prospectively predict the subsequent development of cancer therapy–related cardiac dysfunction (CTRCD).
Methods
DLBCL patients scheduled to receive the R-CHOP regimen were prospectively recruited for this study. Echocardiography was performed at T0 (pre-chemotherapy), T1 (after the third cycle), T2 (end of 6–8 cycles), and T3 (1-year post-T2). Left ventricular global longitudinal strain (LV GLS), LA reservoir strain (LASr), LA conduit strain (LAScd), LA contraction strain (LASct), and LA stiffness index (LASI) were calculated using speckle tracking echocardiography (STE). Patients were divided into CTRCD and non-CTRCD groups based on a > 15% reduction in LV GLS.
Results
Seventeen patients (23.6%) developed to CTRCD after chemotherapy. Patients who developed CTRCD were relatively older (67 [57–71] vs. 60 [49–68], p < 0.05). A significant deterioration in LASr, LAScd, and LASI at T1 persisted throughout the follow-up period. Relative changes in LASr (≥ 23.4%) and LASI (≥ 20.0%) were robust predictors of subsequent CTRCD development. The LASr > 35% criterion in the validation cohort demonstrated substantial agreement with the 2016 ASE guidelines.
Conclusion
LASr and LASI effectively identify early alterations in LA function in DLBCL patients. Relative changes in LASr and LASI are robust predictors of subsequent CTRCD development.
{"title":"Left Atrial Function in Patients With Diffuse Large B-Cell Lymphoma Treated With R-CHOP Chemotherapy Regimen: A Speckle Tracking Echocardiographic Study","authors":"Baozhen Wang, Xiaoyi Hao, Yang Yu, Wenwen Cao, Dangui Huang, Guangping Xie, Shan Yang, Hong Zhao, Ying Zhang, Hui Hao, Yaxin Zhang","doi":"10.1111/echo.70339","DOIUrl":"https://doi.org/10.1111/echo.70339","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>This study aimed to determine whether left atrial (LA) strain can identify early alterations in LA function in diffuse large B-cell lymphoma (DLBCL) patients treated with R-CHOP chemotherapy regimen and whether these alterations can prospectively predict the subsequent development of cancer therapy–related cardiac dysfunction (CTRCD).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>DLBCL patients scheduled to receive the R-CHOP regimen were prospectively recruited for this study. Echocardiography was performed at T0 (pre-chemotherapy), T1 (after the third cycle), T2 (end of 6–8 cycles), and T3 (1-year post-T2). Left ventricular global longitudinal strain (LV GLS), LA reservoir strain (LASr), LA conduit strain (LAScd), LA contraction strain (LASct), and LA stiffness index (LASI) were calculated using speckle tracking echocardiography (STE). Patients were divided into CTRCD and non-CTRCD groups based on a > 15% reduction in LV GLS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Seventeen patients (23.6%) developed to CTRCD after chemotherapy. Patients who developed CTRCD were relatively older (67 [57–71] vs. 60 [49–68], <i>p</i> < 0.05). A significant deterioration in LASr, LAScd, and LASI at T1 persisted throughout the follow-up period. Relative changes in LASr (≥ 23.4%) and LASI (≥ 20.0%) were robust predictors of subsequent CTRCD development. The LASr > 35% criterion in the validation cohort demonstrated substantial agreement with the 2016 ASE guidelines.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>LASr and LASI effectively identify early alterations in LA function in DLBCL patients. Relative changes in LASr and LASI are robust predictors of subsequent CTRCD development.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145470066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mian Zain Hayat, Mohsin Tariq, Syed Ali Raza Zaidi, Muhammad Ahmad
{"title":"Effect of Cardioprotection on Right Ventricular Function in Breast Cancer Patients Receiving Potentially Cardiotoxic Therapy—A Letter to the Editor","authors":"Mian Zain Hayat, Mohsin Tariq, Syed Ali Raza Zaidi, Muhammad Ahmad","doi":"10.1111/echo.70335","DOIUrl":"10.1111/echo.70335","url":null,"abstract":"","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145453842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrew Biscardi, Jordan Tannenbaum, Andrew Kott, Prashant Bhave, Austin Seals, Jeffrey Corbett, Karl Richardson
Purpose
Assessment for percutaneous left atrial appendage occlusion device peri-device leak (pLAAO-PDL) is an important decision point in determining anticoagulation strategy. Measurement of leak size is commonly done with TEE by measuring the 2D vena contracta of the leak. A potential concern is that by measuring a leak only in 2D, there is a risk of missing a larger crescentic leak spanning a more significant circumferential area. Such findings, though largely undiscussed in the current literature, could impact clinical decision-making regarding continuation of anticoagulation.
Methods
TEE reports for patients receiving a plug-like left atrial appendage occlusion device (LAAO) at Atrium Health Wake Forest Baptist or at Atrium Health Carolina's Medical Center from 2021 to 2024 were reviewed. Any reported leaks of 2.5 mm or greater were re-measured by an attending cardiologist with experience in LAAO imaging using standard 2D VC measurements, as well as a traced 3D vena contracta area (3D VCA) with measurement of major and minor axes.
Results
Twenty-nine leaks were remeasured. There was a statistically significant difference between the measured 2D VC and the major axis of the 3D VCA, which was on average three times larger. Three different morphologies of device leak were identified, including punctate, crescentic, and multi-punctate.
Conclusion
3D TEE is useful in distinguishing punctate leaks from crescentic leaks and highlights the fact that leaks cannot be assumed to be uniform in morphology. Given the elevated thromboembolic risks with PDLs, it is reasonable to add 3D TEE imaging to the routine implantation and monitoring of LAAO devices.
{"title":"A Novel Measurement of Percutaneous Left Atrial Appendage Occlusion Device Peri-Device Leak Using Major and Minor Axes of a Three-Dimensional Vena Contracta Area","authors":"Andrew Biscardi, Jordan Tannenbaum, Andrew Kott, Prashant Bhave, Austin Seals, Jeffrey Corbett, Karl Richardson","doi":"10.1111/echo.70334","DOIUrl":"10.1111/echo.70334","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>Assessment for percutaneous left atrial appendage occlusion device peri-device leak (pLAAO-PDL) is an important decision point in determining anticoagulation strategy. Measurement of leak size is commonly done with TEE by measuring the 2D vena contracta of the leak. A potential concern is that by measuring a leak only in 2D, there is a risk of missing a larger crescentic leak spanning a more significant circumferential area. Such findings, though largely undiscussed in the current literature, could impact clinical decision-making regarding continuation of anticoagulation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>TEE reports for patients receiving a plug-like left atrial appendage occlusion device (LAAO) at Atrium Health Wake Forest Baptist or at Atrium Health Carolina's Medical Center from 2021 to 2024 were reviewed. Any reported leaks of 2.5 mm or greater were re-measured by an attending cardiologist with experience in LAAO imaging using standard 2D VC measurements, as well as a traced 3D vena contracta area (3D VCA) with measurement of major and minor axes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-nine leaks were remeasured. There was a statistically significant difference between the measured 2D VC and the major axis of the 3D VCA, which was on average three times larger. Three different morphologies of device leak were identified, including punctate, crescentic, and multi-punctate.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>3D TEE is useful in distinguishing punctate leaks from crescentic leaks and highlights the fact that leaks cannot be assumed to be uniform in morphology. Given the elevated thromboembolic risks with PDLs, it is reasonable to add 3D TEE imaging to the routine implantation and monitoring of LAAO devices.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145453850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tomoka Zukeran, Michiyo Yamano, Tetsuhiro Yamano, Kan Zen, Yuki Matsubara, Kazuaki Takamatsu, Masaki Yashige, Takeshi Nakamura, Hirokazu Shiraishi, Satoaki Matoba
Purpose
Right ventricular dysfunction (RVD) is a prognostic factor in patients with severe aortic stenosis (AS). Some patients have prolonged RVD after transcatheter aortic valve implantation (TAVI). We aimed to identify the prevalence of RVD before and after TAVI and factors associated with preexisting and prolonged RVD in patients with severe AS.
Methods
This retrospective single-center study included 198 patients with severe AS (86.0 ± 4.8 years; 72.7% female). RVD was defined as two of the three parameters being worse than the reference value: fractional area change, right ventricular free wall strain, and tricuspid annular plane systolic excursion.
Results
Sixty-eight patients (34.3%) were diagnosed with preexisting RVD. Factors associated with preexisting RVD were left ventricular (LV) ejection fraction, LV stroke volume index, and ratio of early diastolic velocity to early diastolic annular velocity (E/e′) on the septal side in a multivariable analysis combining baseline clinical and echocardiographic parameters (all p < 0.05). One year after TAVI, 16 of 47 patients (34.0%) were classified as having prolonged RVD. Patients with prolonged RVD had lower LV stroke volume index, greater relative wall thickness, and lower aortic valve mean pressure gradient before TAVI than patients without prolonged RVD (all p < 0.05).
Conclusion
RVD in patients with severe AS is associated with lower LV ejection fraction, lower LV stroke volume index, and higher septal E/e′ at baseline. Smaller LV stroke volume, lower aortic valve mean pressure gradient, and greater relative wall thickness might be predictors of prolonged RVD 1 year after TAVI.
{"title":"Right Ventricular Dysfunction in Patients With Severe Aortic Stenosis Before and After Transcatheter Aortic Valve Implantation","authors":"Tomoka Zukeran, Michiyo Yamano, Tetsuhiro Yamano, Kan Zen, Yuki Matsubara, Kazuaki Takamatsu, Masaki Yashige, Takeshi Nakamura, Hirokazu Shiraishi, Satoaki Matoba","doi":"10.1111/echo.70340","DOIUrl":"10.1111/echo.70340","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>Right ventricular dysfunction (RVD) is a prognostic factor in patients with severe aortic stenosis (AS). Some patients have prolonged RVD after transcatheter aortic valve implantation (TAVI). We aimed to identify the prevalence of RVD before and after TAVI and factors associated with preexisting and prolonged RVD in patients with severe AS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This retrospective single-center study included 198 patients with severe AS (86.0 ± 4.8 years; 72.7% female). RVD was defined as two of the three parameters being worse than the reference value: fractional area change, right ventricular free wall strain, and tricuspid annular plane systolic excursion.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Sixty-eight patients (34.3%) were diagnosed with preexisting RVD. Factors associated with preexisting RVD were left ventricular (LV) ejection fraction, LV stroke volume index, and ratio of early diastolic velocity to early diastolic annular velocity (E/e′) on the septal side in a multivariable analysis combining baseline clinical and echocardiographic parameters (all <i>p</i> < 0.05). One year after TAVI, 16 of 47 patients (34.0%) were classified as having prolonged RVD. Patients with prolonged RVD had lower LV stroke volume index, greater relative wall thickness, and lower aortic valve mean pressure gradient before TAVI than patients without prolonged RVD (all <i>p</i> < 0.05).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>RVD in patients with severe AS is associated with lower LV ejection fraction, lower LV stroke volume index, and higher septal E/e′ at baseline. Smaller LV stroke volume, lower aortic valve mean pressure gradient, and greater relative wall thickness might be predictors of prolonged RVD 1 year after TAVI.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145453857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Giacomo Maria Viani, Hekuran Bytyci, Vincenzo Viccaro, Alessandro Caretta, Susanna Landi, Andrea Milzi, Luca Bergamaschi, Susanne Anna Schlossbauer, Laura Anna Leo, Giovanni Pedrazzini, Carmine Pizzi, Anna Giulia Pavon
Purpose
Accurate echocardiographic assessment of chronic aortic regurgitation (AR) is challenging and subject to interobserver variability. We aim to evaluate reproducibility of guideline-recommended echocardiographic parameters in real-world practice and to assess cardiac magnetic resonance (CMR) as a complementary modality.
Methods
We retrospectively analyzed 99 patients referred to us for transthoracic echocardiography (TTE) for AR evaluation. A subset of 22 patients also underwent CMR. AR severity was independently graded according to ESC and ASE guidelines by two expert cardiologists and two trainees using semi-quantitative and quantitative parameters: pressure half-time (PHT), vena contracta width (VCW), and PISA-derived parameters, such as regurgitant orifice area (EROA). Interobserver agreement was analyzed using Cohen's Kappa, Bland–Altman analysis, and intraclass correlation coefficients.
Results
VCW showed the highest reproducibility (overall κ = 0.77), strong agreement across groups (EXACT team κ = 0.7, TRAIN team κ = 0.86), and high feasibility (68.7%). PHT demonstrated poor interobserver consistency (overall κ = 0.36), wide intra-team variability (EXACT team κ = 0.30 vs. TRAIN team κ = 0.39), and high feasibility (77.8%). EROA showed moderate agreement (overall κ = 0.55, EXACT team κ = 0.64, TRAIN team κ = 0.74) and low feasibility (30.3%). CMR-derived parameters (forward flow, backflow, and regurgitant fraction) displayed excellent reproducibility even between novice and expert readers.
Conclusions
VCW is the most reliable echocardiographic parameter for AR assessment, while PHT and EROA are limited by variability and feasibility, respectively. Given its reproducibility and independence from operator expertise, CMR should be considered early when TTE findings are inconclusive. A simplified TTE-first approach, followed by timely CMR, may optimize staging and management of chronic AR.
目的：准确的超声心动图评估慢性主动脉瓣反流（AR）是具有挑战性的，并受到观察者之间的差异。我们的目的是评估指南推荐的超声心动图参数在现实世界中的可重复性，并评估心脏磁共振（CMR）作为一种补充方式。方法：我们回顾性分析99例经胸超声心动图（TTE）评估AR的患者。22例患者也接受了CMR。根据ESC和ASE指南，由两名心脏病专家和两名受训者使用半定量和定量参数对AR严重程度进行独立分级：压力半衰期（PHT）、静脉收缩宽度（VCW）和由pisa衍生的参数，如回流孔面积（EROA）。使用Cohen’s Kappa、Bland-Altman分析和类内相关系数分析观察者间的一致性。结果：VCW重现性高（overall κ = 0.77），组间一致性强（EXACT team κ = 0.7, TRAIN team κ = 0.86），可行性高（68.7%）。PHT表现出较差的观察者间一致性（总体κ = 0.36），广泛的团队内部可变性（EXACT团队κ = 0.30 vs. TRAIN团队κ = 0.39）和高可行性（77.8%）。EROA的一致性中等（overall κ = 0.55, EXACT team κ = 0.64, TRAIN team κ = 0.74），可行性较低（30.3%）。cmr衍生的参数（正向流量，回流和回流分数）即使在新手和专家读者之间也显示出极好的再现性。结论：VCW是评估AR最可靠的超声心动图参数，而PHT和EROA分别受变异性和可行性的限制。考虑到CMR的可重复性和对操作者专业知识的独立性，在TTE结果不确定时应尽早考虑CMR。简化的te -first方法，然后及时进行CMR，可以优化慢性AR的分期和管理。
{"title":"Diagnostic COncordance in Aortic Regurgitation Severity AssessMent: A ComPARative Study Between Cardiologists With Different Level of Expertise, the COMPARE-AR Study","authors":"Giacomo Maria Viani, Hekuran Bytyci, Vincenzo Viccaro, Alessandro Caretta, Susanna Landi, Andrea Milzi, Luca Bergamaschi, Susanne Anna Schlossbauer, Laura Anna Leo, Giovanni Pedrazzini, Carmine Pizzi, Anna Giulia Pavon","doi":"10.1111/echo.70336","DOIUrl":"10.1111/echo.70336","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>Accurate echocardiographic assessment of chronic aortic regurgitation (AR) is challenging and subject to interobserver variability. We aim to evaluate reproducibility of guideline-recommended echocardiographic parameters in real-world practice and to assess cardiac magnetic resonance (CMR) as a complementary modality.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively analyzed 99 patients referred to us for transthoracic echocardiography (TTE) for AR evaluation. A subset of 22 patients also underwent CMR. AR severity was independently graded according to ESC and ASE guidelines by two expert cardiologists and two trainees using semi-quantitative and quantitative parameters: pressure half-time (PHT), vena contracta width (VCW), and PISA-derived parameters, such as regurgitant orifice area (EROA). Interobserver agreement was analyzed using Cohen's Kappa, Bland–Altman analysis, and intraclass correlation coefficients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>VCW showed the highest reproducibility (overall <i>κ</i> = 0.77), strong agreement across groups (EXACT team <i>κ</i> = 0.7, TRAIN team <i>κ</i> = 0.86), and high feasibility (68.7%). PHT demonstrated poor interobserver consistency (overall <i>κ = </i>0.36), wide intra-team variability (EXACT team <i>κ = </i>0.30 vs. TRAIN team <i>κ = </i>0.39), and high feasibility (77.8%). EROA showed moderate agreement (overall <i>κ = </i>0.55, EXACT team <i>κ = </i>0.64, TRAIN team <i>κ = </i>0.74) and low feasibility (30.3%). CMR-derived parameters (forward flow, backflow, and regurgitant fraction) displayed excellent reproducibility even between novice and expert readers.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>VCW is the most reliable echocardiographic parameter for AR assessment, while PHT and EROA are limited by variability and feasibility, respectively. Given its reproducibility and independence from operator expertise, CMR should be considered early when TTE findings are inconclusive. A simplified TTE-first approach, followed by timely CMR, may optimize staging and management of chronic AR.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145453922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maria de Fatima Egas-Bejar, Gabriela Meléndez-Ramirez, Stephanie Teresa Angulo-Cruzado, Sergio Alfonso Patrón-Chi, Aloha Meave González, Neftali Eduardo Antonio-Villa, Edgar García-Cruz
Purpose
To evaluate the percentage of adults with congenital heart disease (CHD) who are restratified according to classification of complexity classification with CT and/or CMR after an initial echocardiographic assessment. Accurate stratification of CHD complexity is relevant to determining the treatment. Echocardiography is the first-line imaging modality; nevertheless, the added value of computed tomography (CT) and cardiac magnetic resonance (CMR) as complementary modalities to restratify CHD complexity has not been fully explored.
Methods
We conducted a cross-sectional study of adult patients from the record CHD Unit in a third-level cardiology hospital from 2021 to 2024. CHD patients were classified according to the European Society of Cardiology (ESC) guidelines. We compared initial echocardiographic findings with CT and/or CMR using the kappa-statistic.
Results
Among 889 patients that fulfilled the inclusion criteria (women: 57%; mean age: 38.3 ± 14.4 years; genetic syndrome: 4%), the most common CHD were isolated congenital aortic valve disease and bicuspid aorta (n = 127), secundum atrial septal defect (n = 105), and tetralogy of Fallot (n = 90). The number and percentage of patients in each complexity category were mild: 180 (20.2%), moderate: 532 (59.9%), and severe: 177 (19.9%). Ten patients (1%) were restratified: 9 from the mild to moderate category and 1 from the moderate to mild category. Both imaging modalities showed strong agreement (Kappa: 0.979, 95% CI: 0.967–0.990).
Conclusion
Restratification of adult CHD occurred in a low percentage of cases. Our results suggest that in specialized centers, echocardiographic findings are comparable to CT and/or CMR findings.
{"title":"Adult Congenital Heart Disease Complexity Restratification by Computed Tomography and/or Cardiac Magnetic Resonance","authors":"Maria de Fatima Egas-Bejar, Gabriela Meléndez-Ramirez, Stephanie Teresa Angulo-Cruzado, Sergio Alfonso Patrón-Chi, Aloha Meave González, Neftali Eduardo Antonio-Villa, Edgar García-Cruz","doi":"10.1111/echo.70318","DOIUrl":"10.1111/echo.70318","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>To evaluate the percentage of adults with congenital heart disease (CHD) who are restratified according to classification of complexity classification with CT and/or CMR after an initial echocardiographic assessment. Accurate stratification of CHD complexity is relevant to determining the treatment. Echocardiography is the first-line imaging modality; nevertheless, the added value of computed tomography (CT) and cardiac magnetic resonance (CMR) as complementary modalities to restratify CHD complexity has not been fully explored.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a cross-sectional study of adult patients from the record CHD Unit in a third-level cardiology hospital from 2021 to 2024. CHD patients were classified according to the European Society of Cardiology (ESC) guidelines. We compared initial echocardiographic findings with CT and/or CMR using the kappa-statistic.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 889 patients that fulfilled the inclusion criteria (women: 57%; mean age: 38.3 ± 14.4 years; genetic syndrome: 4%), the most common CHD were isolated congenital aortic valve disease and bicuspid aorta (<i>n</i> = 127), secundum atrial septal defect (<i>n</i> = 105), and tetralogy of Fallot (<i>n</i> = 90). The number and percentage of patients in each complexity category were mild: 180 (20.2%), moderate: 532 (59.9%), and severe: 177 (19.9%). Ten patients (1%) were restratified: 9 from the mild to moderate category and 1 from the moderate to mild category. Both imaging modalities showed strong agreement (Kappa: 0.979, 95% CI: 0.967–0.990).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Restratification of adult CHD occurred in a low percentage of cases. Our results suggest that in specialized centers, echocardiographic findings are comparable to CT and/or CMR findings.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145439647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A woman in her 50s with known MYBPC3-associated obstructive hypertrophic cardiomyopathy was evaluated for septal reduction therapy. The electrocardiogram, echocardiogram, and cardiac magnetic resonance found several features inconsistent with the diagnosis. Targeted screening confirmed adult-onset Fabry disease. Enzyme replacement therapy was started.
{"title":"Multimodality Imaging of a Woman With Hypertrophic Cardiomyopathy and Fabry Disease","authors":"Shing Ching, Karl Chan","doi":"10.1111/echo.70332","DOIUrl":"10.1111/echo.70332","url":null,"abstract":"<div>\u0000 \u0000 <p>A woman in her 50s with known MYBPC3-associated obstructive hypertrophic cardiomyopathy was evaluated for septal reduction therapy. The electrocardiogram, echocardiogram, and cardiac magnetic resonance found several features inconsistent with the diagnosis. Targeted screening confirmed adult-onset Fabry disease. Enzyme replacement therapy was started.</p>\u0000 </div>","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145432998","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Guihong Chen, Wei Xiang, Yanhong Zhang, Xijuan Guo, Yanyan Peng, Xiang Li, Xiong Gao, Bulang Gao, Na Li, Liman Fu
Purpose
To investigate the cardiac function and myocardial strain in fetuses with coarctation of the aorta (CoA) using speckle tracking echocardiography.
Materials and Methods
Fetuses with and without CoA were prospectively enrolled to undergo a prenatal ultrasound scan for the presence of CoA. The ultrasound parameters were compared between CoA fetuses and normal controls.
Results
Forty-five CoA fetuses and 336 normal fetuses matched (p > 0.05) in the gestational age, estimated body weight, and pregnant women's age were enrolled. The CoA fetuses had significant (p < 0.05) decreases in the diameters of the aortic arch and isthmus, the left ventricle (LV) stroke volume and its ratio to the estimated body weight, the LV cardiac output and its ratio to the estimated body weight, the LV EF, the LV end-diastolic and end-systolic volume, area, length and basal segment diameter, the right ventricle (RV) end-diastolic (ED) length, the 4CV global sphericity index (GSI), LV and RV fraction of area change (FAC), LV endocardial global longitudinal strain (EndoGLS) and circumferential strain, RV EndoGLS and free wall strain. In the CoA fetuses, significant (p < 0.05) decreases were detected in the fraction of shortening (FS) and FS Z-score in the LV middle and apical segments and in the RV middle segments, and in the FS Z-score in the RV basal segments. In the CoA fetuses, significant (p < 0.05) increases were detected in the GSI and GSI Z-score in the LV apical part, in the ED diameter and its Z-score in the RV basal and middle parts, whereas significant (p < 0.05) decreases in the GSI and GSI Z-score in all the RV basal, middle and apical parts and in the ED diameter and its Z-score in all the LV basal, middle and apical parts. The LV apical ED diameter (2.30 ± 1.13, OR 9.97, 95% CI 1.16–107.89), LV endocardial GCS (−0.30 ± 0.14, OR 0.74, 95% CI 0.55–0.95), LV FAC (−1.17 ± 0.45, OR 0.31, 95% CI 0.13–0.74), and 4CV GSI (−11.69 ± 5.60, OR 8.34e−6, 95% CI 4.51e−11–0.22) were significant (p < 0.05) independent risk factors. The ROC curve analysis of the multivariate regression model for predicting CoA presence revealed an AUC of 0.973, a sensitivity value of 0.977, and a specificity of 0.888.
Conclusion
The cardiac function, anatomical parameters, longitudinal and circumferential strain, FS and GSI, LV end-diastolic diameter, and their Z-scores significantly decrease in CoA fetuses, which may assis
目的应用斑点跟踪超声心动图研究主动脉缩窄胎儿心功能及心肌应变的变化。材料和方法对有和没有CoA的胎儿进行前瞻性的产前超声扫描，以确定CoA的存在。比较CoA胎儿与正常对照组的超声参数。结果45例CoA胎儿与336例正常胎儿在胎龄、估计体重和孕妇年龄上匹配（p > 0.05）。CoA胎儿的主动脉弓和峡部直径、左心室（LV）卒中容积及其与估计体重之比、左室心输出量及其与估计体重之比、左室EF、左室舒张末期和收缩末期容积、面积、长度和基段直径、右心室（RV）舒张末期（ED）长度、4CV全局球形指数（GSI）、左室和右室面积变化分数（FAC）显著（p < 0.05）降低。左心内膜总纵向应变（EndoGLS）和周向应变，右心内膜总纵向应变和自由壁应变。在CoA胎儿中，左心室中间节段、顶端节段和右心室中间节段的缩短率（FS）和FS z -评分显著降低（p < 0.05），右心室基段的FS z -评分显著降低（p < 0.05）。CoA胎儿左心室基部、中部和中部的GSI和GSI - z评分显著（p < 0.05）升高，左心室基部、中部和根尖的GSI和GSI - z评分显著（p < 0.05）降低，左心室基部、中部和根尖的GSI和GSI - z评分显著（p < 0.05）降低。LV根尖ED直径（2.30±1.13,OR 9.97, 95% CI 1.16-107.89）、LV心内膜GCS（- 0.30±0.14,OR 0.74, 95% CI 0.55-0.95）、LV FAC（- 1.17±0.45,OR 0.31, 95% CI 0.13-0.74）和4CV GSI（- 11.69±5.60,OR 8.34e−6,95% CI 4.51e−11-0.22）是具有统计学意义（p < 0.05）的独立危险因素。多变量回归模型预测CoA存在的ROC曲线分析显示，AUC为0.973，灵敏度为0.977，特异性为0.888。结论CoA胎儿的心功能、解剖参数、纵、周应变、FS、GSI、左室舒张末期内径及其z评分均显著降低，这可能有助于CoA胎儿与正常对照组的鉴别。
{"title":"Abnormalities in Cardiac Function, Anatomy, and Myocardial Strain in Coarctation of the Aorta","authors":"Guihong Chen, Wei Xiang, Yanhong Zhang, Xijuan Guo, Yanyan Peng, Xiang Li, Xiong Gao, Bulang Gao, Na Li, Liman Fu","doi":"10.1111/echo.70328","DOIUrl":"https://doi.org/10.1111/echo.70328","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>To investigate the cardiac function and myocardial strain in fetuses with coarctation of the aorta (CoA) using speckle tracking echocardiography.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>Fetuses with and without CoA were prospectively enrolled to undergo a prenatal ultrasound scan for the presence of CoA. The ultrasound parameters were compared between CoA fetuses and normal controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Forty-five CoA fetuses and 336 normal fetuses matched (<i>p</i> > 0.05) in the gestational age, estimated body weight, and pregnant women's age were enrolled. The CoA fetuses had significant (<i>p</i> < 0.05) decreases in the diameters of the aortic arch and isthmus, the left ventricle (LV) stroke volume and its ratio to the estimated body weight, the LV cardiac output and its ratio to the estimated body weight, the LV EF, the LV end-diastolic and end-systolic volume, area, length and basal segment diameter, the right ventricle (RV) end-diastolic (ED) length, the 4CV global sphericity index (GSI), LV and RV fraction of area change (FAC), LV endocardial global longitudinal strain (EndoGLS) and circumferential strain, RV EndoGLS and free wall strain. In the CoA fetuses, significant (<i>p </i>< 0.05) decreases were detected in the fraction of shortening (FS) and FS <i>Z</i>-score in the LV middle and apical segments and in the RV middle segments, and in the FS <i>Z</i>-score in the RV basal segments. In the CoA fetuses, significant (<i>p</i> < 0.05) increases were detected in the GSI and GSI <i>Z</i>-score in the LV apical part, in the ED diameter and its <i>Z</i>-score in the RV basal and middle parts, whereas significant (<i>p</i> < 0.05) decreases in the GSI and GSI <i>Z</i>-score in all the RV basal, middle and apical parts and in the ED diameter and its <i>Z</i>-score in all the LV basal, middle and apical parts. The LV apical ED diameter (2.30 ± 1.13, OR 9.97, 95% CI 1.16–107.89), LV endocardial GCS (−0.30 ± 0.14, OR 0.74, 95% CI 0.55–0.95), LV FAC (−1.17 ± 0.45, OR 0.31, 95% CI 0.13–0.74), and 4CV GSI (−11.69 ± 5.60, OR 8.34e−6, 95% CI 4.51e−11–0.22) were significant (<i>p </i>< 0.05) independent risk factors. The ROC curve analysis of the multivariate regression model for predicting CoA presence revealed an AUC of 0.973, a sensitivity value of 0.977, and a specificity of 0.888.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The cardiac function, anatomical parameters, longitudinal and circumferential strain, FS and GSI, LV end-diastolic diameter, and their <i>Z</i>-scores significantly decrease in CoA fetuses, which may assis","PeriodicalId":50558,"journal":{"name":"Echocardiography-A Journal of Cardiovascular Ultrasound and Allied Techniques","volume":"42 11","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145407485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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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