Pub Date : 2025-06-01Epub Date: 2025-04-08DOI: 10.1016/j.ijcchd.2025.100583
Pandula Athauda arachchi , Sulakkana De Silva
This reflective article explores the first reported case in literature of a transfemoral transcatheter aortic valve implantation (TAVI) in a dextrocardiac patient with severe aortic regurgitation (AR), left-sided superior vena cava (LSVC), and prohibitive surgical risk. It provides an overview of off-label TAVI for pure AR, aortic regurgitation without annular calcification, and in patients with rare congenital anomalies such as dextrocardia. The article discusses the technical, ethical, and procedural challenges, including device oversizing, the use of new-generation valves, and the risk of paravalvular leaks. The review also highlights recent advances in TAVI devices, procedural planning, and outcomes from multicenter studies and registries. The case underscores the importance of innovation, multidisciplinary collaboration, and the evolution of ethical frameworks to guide the safe expansion of TAVI into complex scenarios.
{"title":"Reflections on transfemoral TAVI in dextrocardia with severe aortic regurgitation: Navigating anatomical complexity, off-label innovation, and future directions","authors":"Pandula Athauda arachchi , Sulakkana De Silva","doi":"10.1016/j.ijcchd.2025.100583","DOIUrl":"10.1016/j.ijcchd.2025.100583","url":null,"abstract":"<div><div>This reflective article explores the first reported case in literature of a transfemoral transcatheter aortic valve implantation (TAVI) in a dextrocardiac patient with severe aortic regurgitation (AR), left-sided superior vena cava (LSVC), and prohibitive surgical risk. It provides an overview of off-label TAVI for pure AR, aortic regurgitation without annular calcification, and in patients with rare congenital anomalies such as dextrocardia. The article discusses the technical, ethical, and procedural challenges, including device oversizing, the use of new-generation valves, and the risk of paravalvular leaks. The review also highlights recent advances in TAVI devices, procedural planning, and outcomes from multicenter studies and registries. The case underscores the importance of innovation, multidisciplinary collaboration, and the evolution of ethical frameworks to guide the safe expansion of TAVI into complex scenarios.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100583"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143882730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-06-01Epub Date: 2025-04-25DOI: 10.1016/j.ijcchd.2025.100588
Constance G. Weismann , Frishta Jafar , Joanna Hlebowicz
Introduction
Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for acquired cardiovascular disease and mortality – more so than patients without CHD. In the general population, health related quality of life (HRQoL) is associated with regular physical activity. It was the aim of this study to evaluate the most important predictors of HRQoL in adults with CHD (ACHD).
Methods
This is a registry study using single center data collected between 2004 and 2022. Data include demographic data such as age and sex, body mass index (BMI) type of CHD, prior surgeries, physical activity and HRQoL using the EQ-5D-3L questionnaire. CHD severity was classified based on European Society of Cardiology (ESC) criteria. The cohort was divided based on self-reported levels of physical activity.
Results
A total of 2469 patients were included in this study. 878 (25.6 %) patients had mild, 1151 (46.9 %) moderate and 329 (13.3 %) severe CHD. Patients with severe CHD had a lower BMI, HRQoL and were less physically active than those with mild-moderate CHD. Conversely, patients who were not doing regular exercise were significantly older, were more likely to be female, had a higher BMI, and had a lower HRQoL than their physically active peers. In a logistic regression model, physical activity was the most important predictor of a perfect HRQoL score in all five domains, especially if performed for ≥3 h/week (Odds ratios (OR) 2.1–7.5, all p < 0.001). In patients with severe CHD, HRQoL was disproportionately increased with even little exercise. Other important predictors of perfect HRQoL were younger age (OR 0.99, p < 0.001), male sex (OR 1.58, p < 0.001), mild-moderate CHD (OR 1.59, p < 0.001) and being of normal/overweight (OR 1.44, p < 0.001). Patients with underweight or obesity had a higher HRQoL only if execrising ≥3 h/week.
Conclusion
Regular physical activity in ACHD patients is associated with better HRQoL. Patients with underweight and obesity alike are also at risk for impaired HRQoL. We suggest that ACHD follow-up visits should include counseling on life-style issues in order to enhance HRQoL and minimize modifiable risk factors for acquired cardiovascular disease.
传统的心血管危险因素使先天性心脏病（CHD）患者患后天性心血管疾病和死亡的风险比非CHD患者更高。在一般人群中，健康相关生活质量（HRQoL）与规律的身体活动有关。本研究的目的是评估成人冠心病（ACHD）患者HRQoL的最重要预测因素。方法采用2004 - 2022年间收集的单中心数据进行注册研究。数据包括人口统计数据，如年龄和性别，体重指数（BMI）冠心病类型，既往手术，身体活动和HRQoL使用EQ-5D-3L问卷。根据欧洲心脏病学会（ESC）标准对冠心病严重程度进行分类。研究对象是根据自我报告的身体活动水平进行划分的。结果共纳入2469例患者。轻度冠心病878例（25.6%），中度冠心病1151例（46.9%），重度冠心病329例（13.3%）。与轻中度冠心病患者相比，重度冠心病患者BMI、HRQoL较低，体力活动较少。相反，不经常锻炼的患者明显年龄更大，更有可能是女性，BMI更高，HRQoL低于积极锻炼的同龄人。在logistic回归模型中，体力活动是所有五个领域HRQoL评分完美的最重要预测因素，特别是如果运动≥3小时/周(比值比（OR） 2.1-7.5，所有p <；0.001)。在严重冠心病患者中，即使很少运动，HRQoL也会不成比例地增加。完美HRQoL的其他重要预测因子为年轻(OR 0.99, p <；0.001)，男性(OR 1.58, p <；0.001)，轻中度冠心病(OR 1.59, p <；0.001)和正常/超重(OR 1.44, p <；0.001)。体重过轻或肥胖的患者只有在运动≥3小时/周时才有较高的HRQoL。结论有规律的体育锻炼可提高ACHD患者的HRQoL。体重过轻和肥胖的患者同样存在HRQoL受损的风险。我们建议ACHD随访应包括生活方式问题的咨询，以提高HRQoL并最大限度地减少获得性心血管疾病的可改变危险因素。
{"title":"Physical activity and weight are important predictors of health related quality of life in adults with congenital heart disease","authors":"Constance G. Weismann , Frishta Jafar , Joanna Hlebowicz","doi":"10.1016/j.ijcchd.2025.100588","DOIUrl":"10.1016/j.ijcchd.2025.100588","url":null,"abstract":"<div><h3>Introduction</h3><div>Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for acquired cardiovascular disease and mortality – more so than patients without CHD. In the general population, health related quality of life (HRQoL) is associated with regular physical activity. It was the aim of this study to evaluate the most important predictors of HRQoL in adults with CHD (ACHD).</div></div><div><h3>Methods</h3><div>This is a registry study using single center data collected between 2004 and 2022. Data include demographic data such as age and sex, body mass index (BMI) type of CHD, prior surgeries, physical activity and HRQoL using the EQ-5D-3L questionnaire. CHD severity was classified based on European Society of Cardiology (ESC) criteria. The cohort was divided based on self-reported levels of physical activity.</div></div><div><h3>Results</h3><div>A total of 2469 patients were included in this study. 878 (25.6 %) patients had mild, 1151 (46.9 %) moderate and 329 (13.3 %) severe CHD. Patients with severe CHD had a lower BMI, HRQoL and were less physically active than those with mild-moderate CHD. Conversely, patients who were not doing regular exercise were significantly older, were more likely to be female, had a higher BMI, and had a lower HRQoL than their physically active peers. In a logistic regression model, physical activity was the most important predictor of a perfect HRQoL score in all five domains, especially if performed for ≥3 h/week (Odds ratios (OR) 2.1–7.5, all p < 0.001). In patients with severe CHD, HRQoL was disproportionately increased with even little exercise. Other important predictors of perfect HRQoL were younger age (OR 0.99, p < 0.001), male sex (OR 1.58, p < 0.001), mild-moderate CHD (OR 1.59, p < 0.001) and being of normal/overweight (OR 1.44, p < 0.001). Patients with underweight or obesity had a higher HRQoL only if execrising ≥3 h/week.</div></div><div><h3>Conclusion</h3><div>Regular physical activity in ACHD patients is associated with better HRQoL. Patients with underweight and obesity alike are also at risk for impaired HRQoL. We suggest that ACHD follow-up visits should include counseling on life-style issues in order to enhance HRQoL and minimize modifiable risk factors for acquired cardiovascular disease.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100588"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-06-01Epub Date: 2025-05-05DOI: 10.1016/j.ijcchd.2025.100590
Miriam Michel , David Renaud , Benjamin Kelly , Ismael Z. Assi , Liming Pei , Alexander R. Opotowsky
{"title":"Overcoming borders: International cooperation in re-use and re-interpretation of omics data in Fontan circulation","authors":"Miriam Michel , David Renaud , Benjamin Kelly , Ismael Z. Assi , Liming Pei , Alexander R. Opotowsky","doi":"10.1016/j.ijcchd.2025.100590","DOIUrl":"10.1016/j.ijcchd.2025.100590","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100590"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-06-01Epub Date: 2025-03-13DOI: 10.1016/j.ijcchd.2025.100578
Zeyad Kholeif, Omar Abozied, Ahmed T. Abdelhalim, Sara ElZalabany, Amr Moustafa, Ali Ali, Alexander C. Egbe
Background
Among patients with congenital heart disease (CHD), life expectancy vary by CHD diagnosis, age of the population, and study era, making it difficult to make comparisons between studies. In this study, we aimed to address the knowledge gap regarding temporal change in age at the time of death over time across all CHD diagnoses using a large cohort of adults with CHD with longitudinal follow-up.
Methods
We studied adults with CHD that received care at Mayo Clinic from January 1, 2003 to December 31, 2023.
Results
We identified 9144 adults with CHD that received care within the study period. Of 9144 patients, 1046 (11 %) died, yielding incidence of death of 8.7 per 1000 patient-years. The incidence of death differed by CHD severity groups (7.1 versus 10.3 versus 19.3 per 1000 patient-years, p < 0.001, for simple versus moderate versus complex CHD, respectively). Similarly, the age at the time of death differed by CHD severity groups (66 ± 17 versus 57 ± 16 versus 43 ± 19 years, p < 0.001, for simple versus moderate versus complex CHD, respectively). The age at the time of death was significantly higher in the late era compared to the early era (58 ± 18 versus 50 ± 17 years p < 0.001), and this difference was consistent across CHD severity groups.
Conclusions
There was a temporal increase in the age at the time of death in adults with CHD, and this was consistent across CHD severity groups. The older age at the time of death suggests improved longevity in this population.
{"title":"Temporal change in the age at time of death in adults with congenital heart disease","authors":"Zeyad Kholeif, Omar Abozied, Ahmed T. Abdelhalim, Sara ElZalabany, Amr Moustafa, Ali Ali, Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100578","DOIUrl":"10.1016/j.ijcchd.2025.100578","url":null,"abstract":"<div><h3>Background</h3><div>Among patients with congenital heart disease (CHD), life expectancy vary by CHD diagnosis, age of the population, and study era, making it difficult to make comparisons between studies. In this study, we aimed to address the knowledge gap regarding temporal change in age at the time of death over time across all CHD diagnoses using a large cohort of adults with CHD with longitudinal follow-up.</div></div><div><h3>Methods</h3><div>We studied adults with CHD that received care at Mayo Clinic from January 1, 2003 to December 31, 2023.</div></div><div><h3>Results</h3><div>We identified 9144 adults with CHD that received care within the study period. Of 9144 patients, 1046 (11 %) died, yielding incidence of death of 8.7 per 1000 patient-years. The incidence of death differed by CHD severity groups (7.1 versus 10.3 versus 19.3 per 1000 patient-years, p < 0.001, for simple versus moderate versus complex CHD, respectively). Similarly, the age at the time of death differed by CHD severity groups (66 ± 17 versus 57 ± 16 versus 43 ± 19 years, p < 0.001, for simple versus moderate versus complex CHD, respectively). The age at the time of death was significantly higher in the late era compared to the early era (58 ± 18 versus 50 ± 17 years p < 0.001), and this difference was consistent across CHD severity groups.</div></div><div><h3>Conclusions</h3><div>There was a temporal increase in the age at the time of death in adults with CHD, and this was consistent across CHD severity groups. The older age at the time of death suggests improved longevity in this population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100578"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143643330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The emerging role of tricuspid regurgitation in the long-term outcome of the general population, poses the need for a review of its impact on patients with TOF and the timing for surgical PVR.
Methods
This is a retrospective, single Centre study. Clinical, imaging and surgical data of adults with repaired TOF with a transannular patch repair were collected from our database.
Results
149 patients (61 % male, mean age of 26.0 years) were selected. At a follow-up time of 25.5 years (95 % CI 24.5,26.4) 92 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95 % CI 15.5,17.7) and at a mean age of 17.9 years (95 % CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase likelihood of requiring PVR (log-rank p = 0-0.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = 0.0002) and affected time to PVR (22.10 yrs versus 18.07 yrs). Despite significant RV volume overload, only 16 % of the entire cohort had TR more than moderate, and the degree of TR and tricuspid valve annulus diameter correlated better with atrial rather than ventricular volumes and areas (r = 0.346; p = 0.0068).
Conclusions
TR severity of adults with transannula pacth repair of TOF correlats with surgical type of VSD closure and confers a two-fold increase in the likelihood of requiring PVR during follow-up. The close correlation of TV with atrial rather than ventricular volumes in this cohort suggests a link with the non-functional nature of TR.
{"title":"The impact of tricuspid regurgitation on timing pulmonary valve replacement in ACHD patients after tetralogy of Fallot repair","authors":"Gianluca Brancaccio , Veronica Bordonaro , Matteo Trezzi , Marcello Chinali , Delia Fugallo , Carolina D'Anna , Veronica Lisignoli , Marin Verrengia , Fabio Miraldi , Gianfranco Butera , Roberta Iacobelli , Benedetta Leonardi , Claudia Montanaro , Aurelio Secinaro , Lorenzo Galletti","doi":"10.1016/j.ijcchd.2025.100577","DOIUrl":"10.1016/j.ijcchd.2025.100577","url":null,"abstract":"<div><h3>Background</h3><div>The emerging role of tricuspid regurgitation in the long-term outcome of the general population, poses the need for a review of its impact on patients with TOF and the timing for surgical PVR.</div></div><div><h3>Methods</h3><div>This is a retrospective, single Centre study. Clinical, imaging and surgical data of adults with repaired TOF with a transannular patch repair were collected from our database.</div></div><div><h3>Results</h3><div>149 patients (61 % male, mean age of 26.0 years) were selected. At a follow-up time of 25.5 years (95 % CI 24.5,26.4) 92 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95 % CI 15.5,17.7) and at a mean age of 17.9 years (95 % CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase likelihood of requiring PVR (log-rank p = 0-0.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = 0.0002) and affected time to PVR (22.10 yrs versus 18.07 yrs). Despite significant RV volume overload, only 16 % of the entire cohort had TR more than moderate, and the degree of TR and tricuspid valve annulus diameter correlated better with atrial rather than ventricular volumes and areas (r = 0.346; <em>p</em> = 0.0068).</div></div><div><h3>Conclusions</h3><div>TR severity of adults with transannula pacth repair of TOF correlats with surgical type of VSD closure and confers a two-fold increase in the likelihood of requiring PVR during follow-up. The close correlation of TV with atrial rather than ventricular volumes in this cohort suggests a link with the non-functional nature of TR.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100577"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143643869","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-06-01Epub Date: 2025-04-03DOI: 10.1016/j.ijcchd.2025.100580
Jeff M. Smit , Madelien V. Regeer , Adrianus P. Wijnmaalen , Monique R.M. Jongbloed , Mark G. Hazekamp , Anastasia D. Egorova
Background
The diagnosis and management of atrioventricular (AV)-conduction disorders in patients with a Fontan circulation can be challenging. Little is known about the effects of various pacing strategies in single-ventricle patients. Here we report 1) the feasibility of transesophageal electrophysiological study (EPS) to assess AV-conduction in a patient with limited venous access and 2) the potential of echocardiography to guide epicardial systemic right ventricular (sRV) lead positioning and to evaluate the hemodynamic consequences of sRV pacing in order to mitigate long-term effects of single site ventricular pacing.
Material and methods
A 21-year old male with hypoplastic left heart syndrome, palliated with Norwood and Glenn procedures, and ultimately extracardiac total cavopulmonary connection was seen for a regular check-up. He reported difficulty cycling against the wind. During exercise stress test, a 2:1 AV-block occurred at atrial frequencies >100 bpm with recovery of 1:1 AV-conduction at sinus rates of 80–100 bpm. In order to discriminate between a 2:1 conducted atrial tachycardia and an impaired anterograde AV-conduction during sinus rhythm in the setting of bilateral femoral vein and unilateral subclavian/jugular vein occlusion, EPS by transesophageal pacing was proposed.
Results
Bipolar transesophageal pacing of the left atrium confirmed an anterograde AV-Wenckebach point at 103 bpm, confirming the indication for AV-sequential pacing. Epicardial leads were surgically placed on the atrium and sRV apex. During intraoperative sRV pacing, transesophageal echocardiography confirmed the ventricular contraction pattern to remain synchronous with stable estimated cardiac output. Transthoracic echocardiography was performed postoperatively to assess the effects of sRV pacing on ventricular (dys)synchrony, systolic function and estimated cardiac output. These parameters remained unchanged during sRV pacing, compared to intrinsic conduction, an important finding in light of preserving sRV function.
Conclusions
EPS to assess AV conduction could safely be performed by transesophageal pacing in this patient with Fontan circulation. Moreover, echocardiographic guidance of epicardial sRV pacemaker lead placement was feasible and may help to define the optimal pacing site in Fontan patients.
{"title":"A 2:1 atrioventricular block in an adult patient with a Fontan circulation: from transesophageal pacing to echocardiographic guidance of epicardial pacemaker lead placement","authors":"Jeff M. Smit , Madelien V. Regeer , Adrianus P. Wijnmaalen , Monique R.M. Jongbloed , Mark G. Hazekamp , Anastasia D. Egorova","doi":"10.1016/j.ijcchd.2025.100580","DOIUrl":"10.1016/j.ijcchd.2025.100580","url":null,"abstract":"<div><h3>Background</h3><div>The diagnosis and management of atrioventricular (AV)-conduction disorders in patients with a Fontan circulation can be challenging. Little is known about the effects of various pacing strategies in single-ventricle patients. Here we report 1) the feasibility of transesophageal electrophysiological study (EPS) to assess AV-conduction in a patient with limited venous access and 2) the potential of echocardiography to guide epicardial systemic right ventricular (sRV) lead positioning and to evaluate the hemodynamic consequences of sRV pacing in order to mitigate long-term effects of single site ventricular pacing.</div></div><div><h3>Material and methods</h3><div>A 21-year old male with hypoplastic left heart syndrome, palliated with Norwood and Glenn procedures, and ultimately extracardiac total cavopulmonary connection was seen for a regular check-up. He reported difficulty cycling against the wind. During exercise stress test, a 2:1 AV-block occurred at atrial frequencies >100 bpm with recovery of 1:1 AV-conduction at sinus rates of 80–100 bpm. In order to discriminate between a 2:1 conducted atrial tachycardia and an impaired anterograde AV-conduction during sinus rhythm in the setting of bilateral femoral vein and unilateral subclavian/jugular vein occlusion, EPS by transesophageal pacing was proposed.</div></div><div><h3>Results</h3><div>Bipolar transesophageal pacing of the left atrium confirmed an anterograde AV-Wenckebach point at 103 bpm, confirming the indication for AV-sequential pacing. Epicardial leads were surgically placed on the atrium and sRV apex. During intraoperative sRV pacing, transesophageal echocardiography confirmed the ventricular contraction pattern to remain synchronous with stable estimated cardiac output. Transthoracic echocardiography was performed postoperatively to assess the effects of sRV pacing on ventricular (dys)synchrony, systolic function and estimated cardiac output. These parameters remained unchanged during sRV pacing, compared to intrinsic conduction, an important finding in light of preserving sRV function.</div></div><div><h3>Conclusions</h3><div>EPS to assess AV conduction could safely be performed by transesophageal pacing in this patient with Fontan circulation. Moreover, echocardiographic guidance of epicardial sRV pacemaker lead placement was feasible and may help to define the optimal pacing site in Fontan patients.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100580"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143839584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-10DOI: 10.1016/j.ijcchd.2025.100585
Grimholt T.K. , Gjesdal O. , Bonsaksen T. , Heir T. , Ekeberg O. , Schou Bredal I. , Skogstad L. , Tøllefsen I.M.
{"title":"Posttraumatic stress disorder symptoms in adults with congenital heart disease (ACHD) during the COVID-19 pandemic in Norway","authors":"Grimholt T.K. , Gjesdal O. , Bonsaksen T. , Heir T. , Ekeberg O. , Schou Bredal I. , Skogstad L. , Tøllefsen I.M.","doi":"10.1016/j.ijcchd.2025.100585","DOIUrl":"10.1016/j.ijcchd.2025.100585","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100585"},"PeriodicalIF":0.8,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143839012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2024-11-16DOI: 10.1016/j.ijcchd.2024.100552
Ahmed Kheiwa , Inhae Baek , Ian S. Harris , Abdul Mannan Khan Minhas , Dmitry Abramov
Background
There are limited data on the prevalence of heart failure (HF) due to congenital heart diseases (CHD).
Methods
The Global Burden of Disease (GBD) dataset was used to analyze the disease burden of HF due to CHD between 1990 and 2021. Age-standardized rates (ASR) (crude prevalence rates for age groups) and total percent change for the overall worldwide burden as well as among subgroups based on age and geographic regions were determined.
Results
The global prevalence of HF due to CHD in 1990 and 2021 was 2,494,547 (95 % IU 2,054,729 to 3,030,909) and 3,155,991 (95 % IU 2,578,552 to 3,843,062) individuals respectively, with an increase in ASR from 41.02 (33.79–49.79) to 45.33 (37.15–55.17) per 100,000 individuals during that period. HF due to CHD in 2021 was most common in children aged 2–4, crude rate of 248.44 (195.99–302.57), followed by children <1 years of age, crude rate of 142.75 (116.87–174.26), and children aged 5–14, crude rate of 89.55 (62.35–129.61). During the study period, there was an increase in the prevalence of HF due to CHD among most age groups, other than children <1 year of age who had stable rates and individuals >70 who had no reported prevalence. There were geographic differences in the prevalence and trends of HF due to CHD.
Conclusions
There are differences in prevalence and trends of HF due to CHD among age groups and worldwide regions. These results demonstrate the worldwide burden and trends of HF due to CHD.
{"title":"Worldwide prevalence of heart failure due to congenital heart disease: An analysis from the Global Burden of Disease Study 2021","authors":"Ahmed Kheiwa , Inhae Baek , Ian S. Harris , Abdul Mannan Khan Minhas , Dmitry Abramov","doi":"10.1016/j.ijcchd.2024.100552","DOIUrl":"10.1016/j.ijcchd.2024.100552","url":null,"abstract":"<div><h3>Background</h3><div>There are limited data on the prevalence of heart failure (HF) due to congenital heart diseases (CHD).</div></div><div><h3>Methods</h3><div>The Global Burden of Disease (GBD) dataset was used to analyze the disease burden of HF due to CHD between 1990 and 2021. Age-standardized rates (ASR) (crude prevalence rates for age groups) and total percent change for the overall worldwide burden as well as among subgroups based on age and geographic regions were determined.</div></div><div><h3>Results</h3><div>The global prevalence of HF due to CHD in 1990 and 2021 was 2,494,547 (95 % IU 2,054,729 to 3,030,909) and 3,155,991 (95 % IU 2,578,552 to 3,843,062) individuals respectively, with an increase in ASR from 41.02 (33.79–49.79) to 45.33 (37.15–55.17) per 100,000 individuals during that period. HF due to CHD in 2021 was most common in children aged 2–4, crude rate of 248.44 (195.99–302.57), followed by children <1 years of age, crude rate of 142.75 (116.87–174.26), and children aged 5–14, crude rate of 89.55 (62.35–129.61). During the study period, there was an increase in the prevalence of HF due to CHD among most age groups, other than children <1 year of age who had stable rates and individuals >70 who had no reported prevalence. There were geographic differences in the prevalence and trends of HF due to CHD.</div></div><div><h3>Conclusions</h3><div>There are differences in prevalence and trends of HF due to CHD among age groups and worldwide regions. These results demonstrate the worldwide burden and trends of HF due to CHD.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100552"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2024-12-20DOI: 10.1016/j.ijcchd.2024.100560
Covadonga Terol Espinosa de los Monteros , Roel L.F. van der Palen , Jef Van den Eynde , Lukas Rammeloo , Mark G. Hazekamp , Nico A. Blom , Irene M. Kuipers , Arend D.J. ten Harkel
Background
Advanced echocardiographic techniques such as Tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE) can detect more subtle changes in ventricular performance. We aimed to study the ventricular performance in patients with transposition of the great arteries (TGA) at mid-term follow-up after the arterial switch operation (ASO) with advanced echocardiographic techniques. In addition, we sought to discover new clinical phenotypes using unsupervised machine learning.
Methods
Conventional, TDI and STE echocardiographic parameters were prospectively obtained from 124 TGA patients (66.1 % male, age 10.8 ± 5.1 years, 24.2 % with ventricular septal defect) in this observational study. The data was analyzed with conventional statistics and new machine learning techniques.
Results
TGA patients had reduced biventricular systolic (septal s’ Z-score −2.28 ± 1.26; RV s’ Z-score −2.16 ± 0.71; mean left ventricular longitudinal strain Z-score of the LV -2.49 ± 1.68) and RV diastolic performance (RV E/e’ Z-score 2.35 ± 1.70) mid-term after ASO. Unsupervised clustering within the TGA population revealed 3 clusters. Interestingly, cluster 3 defined a group of patients with older age at ASO, the most reduced ventricular performance as well as the highest rates of reoperations and interventions.
Conclusions
Assessment of ventricular performance with TDI and STE 10 years after ASO showed that TGA patients have decreased biventricular systolic and diastolic function, especially at the septal regions. Novel analytical methods such as unsupervised clustering may help identify new clinical phenotypes from multiple variables and may contribute to improved risk stratification.
先进的超声心动图技术，如组织多普勒成像（TDI）和斑点跟踪超声心动图（STE）可以检测到心室功能更细微的变化。我们的目的是利用先进的超声心动图技术研究大动脉转位（TGA）患者在动脉转换手术（ASO）后中期随访时的心室功能。此外，我们试图使用无监督机器学习来发现新的临床表型。方法对124例TGA患者（男性66.1%，年龄10.8±5.1岁，室间隔缺损24.2%）的常规、TDI和STE超声心动图参数进行前瞻性分析。使用传统统计学和新的机器学习技术对数据进行分析。结果stga患者双室收缩（间隔）Z-score（- 2.28±1.26）降低；RV s ' Z-score−2.16±0.71；ASO中期平均左室纵向应变Z-score（-2.49±1.68）和左室舒张性能（RV E/ E ' Z-score 2.35±1.70）。TGA人口中的无监督聚类显示出3个聚类。有趣的是，第3组定义了一组年龄较大的ASO患者，心室功能下降最多，再手术和干预率最高。结论在ASO术后10年用TDI和STE评估心室功能显示TGA患者双心室收缩和舒张功能下降，特别是在间隔区。新的分析方法，如无监督聚类可能有助于从多个变量中识别新的临床表型，并可能有助于改善风险分层。
{"title":"Using machine learning analysis to describe patterns in tissue Doppler and speckle tracking echocardiography in patients with transposition of the great arteries after arterial switch operation","authors":"Covadonga Terol Espinosa de los Monteros , Roel L.F. van der Palen , Jef Van den Eynde , Lukas Rammeloo , Mark G. Hazekamp , Nico A. Blom , Irene M. Kuipers , Arend D.J. ten Harkel","doi":"10.1016/j.ijcchd.2024.100560","DOIUrl":"10.1016/j.ijcchd.2024.100560","url":null,"abstract":"<div><h3>Background</h3><div>Advanced echocardiographic techniques such as Tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE) can detect more subtle changes in ventricular performance. We aimed to study the ventricular performance in patients with transposition of the great arteries (TGA) at mid-term follow-up after the arterial switch operation (ASO) with advanced echocardiographic techniques. In addition, we sought to discover new clinical phenotypes using unsupervised machine learning.</div></div><div><h3>Methods</h3><div>Conventional, TDI and STE echocardiographic parameters were prospectively obtained from 124 TGA patients (66.1 % male, age 10.8 ± 5.1 years, 24.2 % with ventricular septal defect) in this observational study. The data was analyzed with conventional statistics and new machine learning techniques.</div></div><div><h3>Results</h3><div>TGA patients had reduced biventricular systolic (septal s’ Z-score −2.28 ± 1.26; RV s’ Z-score −2.16 ± 0.71; mean left ventricular longitudinal strain Z-score of the LV -2.49 ± 1.68) and RV diastolic performance (RV E/e’ Z-score 2.35 ± 1.70) mid-term after ASO. Unsupervised clustering within the TGA population revealed 3 clusters. Interestingly, cluster 3 defined a group of patients with older age at ASO, the most reduced ventricular performance as well as the highest rates of reoperations and interventions.</div></div><div><h3>Conclusions</h3><div>Assessment of ventricular performance with TDI and STE 10 years after ASO showed that TGA patients have decreased biventricular systolic and diastolic function, especially at the septal regions. Novel analytical methods such as unsupervised clustering may help identify new clinical phenotypes from multiple variables and may contribute to improved risk stratification.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100560"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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