Pub Date : 2025-04-28DOI: 10.1016/j.ijcchd.2025.100589
Raksheeth Agarwal , Louise E. Coats , Ali N. Zaidi
Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.
{"title":"Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead","authors":"Raksheeth Agarwal , Louise E. Coats , Ali N. Zaidi","doi":"10.1016/j.ijcchd.2025.100589","DOIUrl":"10.1016/j.ijcchd.2025.100589","url":null,"abstract":"<div><div>Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100589"},"PeriodicalIF":0.8,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143911594","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 : 2025-04-26DOI: 10.1016/j.ijcchd.2025.100587
Samuel L. Casella , Sangeeta Shah
Biatrial drainage of the right superior vena cava (RSVC) is a rare anomaly of the cavo-pulmonary venous confluence which may lead to chronic cyanosis, stroke and cerebral abscess. Historical corrections have been limited to open surgical repair. We describe an adult patient with biatrial drainage of the RSVC with chronic cyanosis who underwent successful percutaneous correction using a covered stent.
{"title":"Percutaneous repair of biatrial drainage of the right superior vena cava using a covered stent","authors":"Samuel L. Casella , Sangeeta Shah","doi":"10.1016/j.ijcchd.2025.100587","DOIUrl":"10.1016/j.ijcchd.2025.100587","url":null,"abstract":"<div><div>Biatrial drainage of the right superior vena cava (RSVC) is a rare anomaly of the cavo-pulmonary venous confluence which may lead to chronic cyanosis, stroke and cerebral abscess. Historical corrections have been limited to open surgical repair. We describe an adult patient with biatrial drainage of the RSVC with chronic cyanosis who underwent successful percutaneous correction using a covered stent.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100587"},"PeriodicalIF":0.8,"publicationDate":"2025-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935176","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 : 2025-04-25DOI: 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":"2025-04-25","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 : 2025-04-15DOI: 10.1016/j.ijcchd.2025.100584
Ismael Z. Assi , Michael J. Landzberg , Kristian C. Becker , David Renaud , Fernando Baraona Reyes , David M. Leone , Mark Benson , Miriam Michel , Robert E. Gerszten , Alexander R. Opotowsky
Background
High-throughput proteomics platforms using aptamers (SomaScan) or proximity extension assay (Olink) provide novel opportunities for improving diagnostic and risk stratification tools in cardiovascular diseases, including understudied congenital heart diseases. The correlation between these proteomics approaches has not yet been studied among individuals with a Fontan circulation.
Objective
The correlation of plasma protein measurements between SomaScan and Olink platforms was evaluated in adults with a Fontan circulation.
Methods
We measured 491 proteins in plasma of 71 adults with a Fontan circulation using Olink and SomaScan. Missing Olink measurements (0.13%, 47/34,861) were imputed using non-parametric imputation. Spearman's rank correlation coefficient for absolute values of protein expression between platforms was calculated. Protein correlation frequencies were compared to 3 cohorts reported in the literature using Pearson's Chi-squared test of independence.
Results
Overall, protein correlations between Olink and SomaScan measurements were moderately strong for most proteins, (rho > 0.4 for 57.2%), but with substantial variability (median correlation = 0.457, IQR = 0.538). The distribution of protein correlations was qualitatively similar to published literature in non-Fontan cohorts. Both Olink and SomaScan identified proteins with sex-based differences; both identified differences in myostatin and leptin, but each identified additional nonoverlapping sexually dimorphic proteins (n = 14 Olink, n = 5 SomaScan).
Conclusions
In adults with a Fontan circulation, correlations between plasma proteins measured by Olink and SomaScan varied widely, approximately in line with prior reports in other populations. While these tools may be uniquely useful to generate hypotheses, specifically regarding potential molecular mechanisms, more definitive inference requires independent validation.
{"title":"Correlation between Olink and SomaScan proteomics platforms in adults with a Fontan circulation","authors":"Ismael Z. Assi , Michael J. Landzberg , Kristian C. Becker , David Renaud , Fernando Baraona Reyes , David M. Leone , Mark Benson , Miriam Michel , Robert E. Gerszten , Alexander R. Opotowsky","doi":"10.1016/j.ijcchd.2025.100584","DOIUrl":"10.1016/j.ijcchd.2025.100584","url":null,"abstract":"<div><h3>Background</h3><div>High-throughput proteomics platforms using aptamers (SomaScan) or proximity extension assay (Olink) provide novel opportunities for improving diagnostic and risk stratification tools in cardiovascular diseases, including understudied congenital heart diseases. The correlation between these proteomics approaches has not yet been studied among individuals with a Fontan circulation.</div></div><div><h3>Objective</h3><div>The correlation of plasma protein measurements between SomaScan and Olink platforms was evaluated in adults with a Fontan circulation.</div></div><div><h3>Methods</h3><div>We measured 491 proteins in plasma of 71 adults with a Fontan circulation using Olink and SomaScan. Missing Olink measurements (0.13%, 47/34,861) were imputed using non-parametric imputation. Spearman's rank correlation coefficient for absolute values of protein expression between platforms was calculated. Protein correlation frequencies were compared to 3 cohorts reported in the literature using Pearson's Chi-squared test of independence.</div></div><div><h3>Results</h3><div>Overall, protein correlations between Olink and SomaScan measurements were moderately strong for most proteins, (rho > 0.4 for 57.2%), but with substantial variability (median correlation = 0.457, IQR = 0.538). The distribution of protein correlations was qualitatively similar to published literature in non-Fontan cohorts. Both Olink and SomaScan identified proteins with sex-based differences; both identified differences in myostatin and leptin, but each identified additional nonoverlapping sexually dimorphic proteins (n = 14 Olink, n = 5 SomaScan).</div></div><div><h3>Conclusions</h3><div>In adults with a Fontan circulation, correlations between plasma proteins measured by Olink and SomaScan varied widely, approximately in line with prior reports in other populations. While these tools may be uniquely useful to generate hypotheses, specifically regarding potential molecular mechanisms, more definitive inference requires independent validation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100584"},"PeriodicalIF":0.8,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865112","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 : 2025-04-15DOI: 10.1016/j.ijcchd.2025.100586
Ghazwan Butrous
Pulmonary hypertension is a serious condition characterised by elevated blood pressure in the pulmonary arteries, caused by various aetiologies and via different pathological processes. Over the past seventy years, our understanding and management of this disorder have greatly improved, resulting in increased diagnosis and effective clinical management. Current epidemiological estimates are challenged by the increased awareness of this condition and the changing definitions and classification systems. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) has also shown temporal changes in the epidemiology of pulmonary hypertension over the last thirty years, contributing to regional variations in prevalence and incidence.
This review explores the complexities of global and regional variations in different types of pulmonary hypertension reported through many registries, databases and regional studies. Although these tools can help estimate prevalence and incidences, they may also underestimate the actual number of cases due to the continuously changing understanding of the condition and increase awareness globally. Therefore, continued research, international collaboration, and standardised data collection are essential for achieving a more accurate global view of pulmonary hypertension and developing effective management strategies for this serious condition that significantly impacts general health.
{"title":"Pulmonary hypertension aetiologies in different parts of the world","authors":"Ghazwan Butrous","doi":"10.1016/j.ijcchd.2025.100586","DOIUrl":"10.1016/j.ijcchd.2025.100586","url":null,"abstract":"<div><div>Pulmonary hypertension is a serious condition characterised by elevated blood pressure in the pulmonary arteries, caused by various aetiologies and via different pathological processes. Over the past seventy years, our understanding and management of this disorder have greatly improved, resulting in increased diagnosis and effective clinical management. Current epidemiological estimates are challenged by the increased awareness of this condition and the changing definitions and classification systems. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) has also shown temporal changes in the epidemiology of pulmonary hypertension over the last thirty years, contributing to regional variations in prevalence and incidence.</div><div>This review explores the complexities of global and regional variations in different types of pulmonary hypertension reported through many registries, databases and regional studies. Although these tools can help estimate prevalence and incidences, they may also underestimate the actual number of cases due to the continuously changing understanding of the condition and increase awareness globally. Therefore, continued research, international collaboration, and standardised data collection are essential for achieving a more accurate global view of pulmonary hypertension and developing effective management strategies for this serious condition that significantly impacts general health.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100586"},"PeriodicalIF":0.8,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865113","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 : 2025-04-08DOI: 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":"2025-04-08","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 : 2025-04-07DOI: 10.1016/j.ijcchd.2025.100582
Nunzia Borrelli , Nicola Grimaldi , Flavia Fusco , Antonio Orlando , Michela Palma , Maria Cristina Boccia , Sabrina Bassolino , Anna Iervolino , Berardo Sarubbi
Introduction
The number of adults with congenital heart disease has significantly increased in recent years. While telemedicine has emerged as a promising approach to improve care delivery and patient outcomes, its use for adults with congenital cardiopathies has not been extensively explored. This study aims to evaluate the feasibility and clinical impact of a telemedicine programme for adults with congenital heart diseases.
Methods
This was a single-center, prospective, observational study, carried out between January 2022 and January 2023. A wristwatch, a paired mobile device, and a dedicated hospital workstation were used to telemonitor 25 adult patients with moderate-to-severe congenital heart condition, in II-IV New-York Heart Association class. We assessed changes in hospital admission days and related costs, patient satisfaction, and functional parameters, including 6-min walk test distance, vital signs, and echocardiographic ejection fraction.
Results
All 25 patients agreed to participate to the telemedicine program (mean age 38.35 ± 11.33 years, 52 % male). Twenty-three patients completed the program. No significant changes were observed in vital signs, functional class, ventricular ejection fraction, while walking distance significantly improved (404 ± 82 m versus 433 ± 142 m, p = 0.03). Notably, hospital admission days and related costs were significantly reduced during the TM program year compared to the prior year. Patient satisfaction was high. One patient experienced a prolonged hospitalization and death due to the natural progression of their condition.
Conclusion
High-risk patients with moderate-severe congenital heart disease can benefit from a device-implemented telemedicine program, which offers tailored, specialized care directly at home, reducing hospital admissions and ensuring stable clinical status.
近年来，患有先天性心脏病的成年人数量明显增加。虽然远程医疗已经成为一种有希望改善护理服务和患者预后的方法，但其在成人先天性心脏病患者中的应用尚未得到广泛探索。本研究旨在评估成人先天性心脏病远程医疗方案的可行性和临床影响。方法：这是一项单中心、前瞻性、观察性研究，于2022年1月至2023年1月进行。在纽约心脏协会II-IV级，使用腕表、配对移动设备和专用医院工作站对25例中度至重度先天性心脏病成年患者进行远程监测。我们评估了住院天数和相关费用、患者满意度和功能参数的变化，包括6分钟步行测试距离、生命体征和超声心动图射血分数。结果25例患者均同意参加远程医疗项目，平均年龄38.35±11.33岁，男性占52%。23名患者完成了该项目。生命体征、功能分级、心室射血分数无明显变化，步行距离明显改善（404±82 m vs 433±142 m, p = 0.03）。值得注意的是，与前一年相比，住院天数和相关费用在TM计划年度显着减少。患者满意度高。一名患者因病情的自然进展而长期住院并死亡。结论中重度先天性心脏病高危患者可从设备实施的远程医疗项目中获益，该项目可直接在家中提供量身定制的专科护理，减少住院率，确保临床状态稳定。
{"title":"Feasibility and effectiveness of telemedicine for adult patients with congenital heart disease: A one-year single-center experience-based study","authors":"Nunzia Borrelli , Nicola Grimaldi , Flavia Fusco , Antonio Orlando , Michela Palma , Maria Cristina Boccia , Sabrina Bassolino , Anna Iervolino , Berardo Sarubbi","doi":"10.1016/j.ijcchd.2025.100582","DOIUrl":"10.1016/j.ijcchd.2025.100582","url":null,"abstract":"<div><h3>Introduction</h3><div>The number of adults with congenital heart disease has significantly increased in recent years. While telemedicine has emerged as a promising approach to improve care delivery and patient outcomes, its use for adults with congenital cardiopathies has not been extensively explored. This study aims to evaluate the feasibility and clinical impact of a telemedicine programme for adults with congenital heart diseases.</div></div><div><h3>Methods</h3><div>This was a single-center, prospective, observational study, carried out between January 2022 and January 2023. A wristwatch, a paired mobile device, and a dedicated hospital workstation were used to telemonitor 25 adult patients with moderate-to-severe congenital heart condition, in II-IV New-York Heart Association class. We assessed changes in hospital admission days and related costs, patient satisfaction, and functional parameters, including 6-min walk test distance, vital signs, and echocardiographic ejection fraction.</div></div><div><h3>Results</h3><div>All 25 patients agreed to participate to the telemedicine program (mean age 38.35 ± 11.33 years, 52 % male). Twenty-three patients completed the program. No significant changes were observed in vital signs, functional class, ventricular ejection fraction, while walking distance significantly improved (404 ± 82 m versus 433 ± 142 m, p = 0.03). Notably, hospital admission days and related costs were significantly reduced during the TM program year compared to the prior year. Patient satisfaction was high. One patient experienced a prolonged hospitalization and death due to the natural progression of their condition.</div></div><div><h3>Conclusion</h3><div>High-risk patients with moderate-severe congenital heart disease can benefit from a device-implemented telemedicine program, which offers tailored, specialized care directly at home, reducing hospital admissions and ensuring stable clinical status.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100582"},"PeriodicalIF":0.8,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935175","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 : 2025-04-03DOI: 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":"2025-04-03","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}
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