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}
Pub Date : 2025-03-25DOI: 10.1016/j.ijcchd.2025.100579
Larissa Lloyd , Calum Nicholson , Geoff Strange , Rachael Cordina , David S. Celermajer , Michael M.H. Cheung
Objective
To compare the outcomes for repaired tetralogy of Fallot and Fontan patients who must travel from regional Victoria and interstate, in order to receive specialist congenital heart disease (CHD) surgery and ongoing care, with those of local patients.
Methods
This retrospective study included 332 patients who underwent tetralogy of Fallot (ToF) repair and 159 patients who underwent a Fontan procedure at Royal Children's Hospital (RCH) Melbourne between 2003 and 2017. Data was obtained from the National CHD Registry, linked with National Death Index data, and follow-up data from the Australian and New Zealand Fontan Registry.
Results
Equivalent outcomes were observed between location groups in both cohorts for all of the main outcomes of interest. Repaired ToF subjects were aged 0.76 years (IQR 0.52–3.33) at operation and 10.2 years (IQR 5.46–14.9) at last follow-up, whilst Fontan subjects were aged 4.94 (IQR 4.27–5.66) years at operation and 14.2 years (IQR 11.3–16.4) at last follow-up. Mortality rates were extremely low and did not significantly differ between geographic groups, with 10-year survival in the repaired ToF cohort 98.0 % in the City group, 98.1 % in the Regional group, and 98.8 % in the Interstate group; and 97.8 %, 92.3 %, and 97.5 % in the Fontan cohort, respectively.
Conclusions
In the Australian setting and with adequate planning and local follow-up options, patients travelling from regional areas or interstate for their CHD operations have similar outcomes, out to 21 years, compared to patients living locally.
{"title":"Excellent medium to long term outcomes after cardiac surgery for moderate and complex congenital heart disease, regardless of geographic location","authors":"Larissa Lloyd , Calum Nicholson , Geoff Strange , Rachael Cordina , David S. Celermajer , Michael M.H. Cheung","doi":"10.1016/j.ijcchd.2025.100579","DOIUrl":"10.1016/j.ijcchd.2025.100579","url":null,"abstract":"<div><h3>Objective</h3><div>To compare the outcomes for repaired tetralogy of Fallot and Fontan patients who must travel from regional Victoria and interstate, in order to receive specialist congenital heart disease (CHD) surgery and ongoing care, with those of local patients.</div></div><div><h3>Methods</h3><div>This retrospective study included 332 patients who underwent tetralogy of Fallot (ToF) repair and 159 patients who underwent a Fontan procedure at Royal Children's Hospital (RCH) Melbourne between 2003 and 2017. Data was obtained from the National CHD Registry, linked with National Death Index data, and follow-up data from the Australian and New Zealand Fontan Registry.</div></div><div><h3>Results</h3><div>Equivalent outcomes were observed between location groups in both cohorts for all of the main outcomes of interest. Repaired ToF subjects were aged 0.76 years (IQR 0.52–3.33) at operation and 10.2 years (IQR 5.46–14.9) at last follow-up, whilst Fontan subjects were aged 4.94 (IQR 4.27–5.66) years at operation and 14.2 years (IQR 11.3–16.4) at last follow-up. Mortality rates were extremely low and did not significantly differ between geographic groups, with 10-year survival in the repaired ToF cohort 98.0 % in the City group, 98.1 % in the Regional group, and 98.8 % in the Interstate group; and 97.8 %, 92.3 %, and 97.5 % in the Fontan cohort, respectively.</div></div><div><h3>Conclusions</h3><div>In the Australian setting and with adequate planning and local follow-up options, patients travelling from regional areas or interstate for their CHD operations have similar outcomes, out to 21 years, compared to patients living locally.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100579"},"PeriodicalIF":0.8,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143785302","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 : 2025-03-13DOI: 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":"2025-03-13","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":"2025-03-13","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}
Patients with repaired tetralogy of Fallot (TOF) now live longer. However, dysrhythmia becomes prevalent in adults with repaired TOF, especially atrial tachyarrhythmia.
Objective
To identify the characteristics of patients who develop atrial tachycardia (AT) and the mechanism of the clinical AT and the induced one.
Method
Seventy-seven patients with repaired TOF were enrolled. The patients were divided into two groups (AT and non-AT). Clinical and electrophysiologic data were studied.
Results
The mean age was 34 years. Twenty-three patients had AT (30 %). In AT group, the left ventricular ejection fraction was lower (58 ± 6 vs 62 ± 5; P = 0.011), the right and left atrial area (cm2) was larger (29 ± 13 vs 15 ± 5; P < 0.001, and 19 ± 3 vs 16 ± 4; P < 0.001, respectively), and the right ventricular S′ wave (cm/s) was smaller (8 ± 2 vs 10 ± 3; P = 0.029).
Patients with AT underwent catheter ablation, and 32 AT were ablated. The mechanism of AT was intra-atrial reentrant tachycardia in 14 AT (44 %), cavotricuspid isthmus-dependent in 12 AT (37 %), and focal activity in the remaining 6 AT (19 %). An important finding was that after the first AT was ablated, another AT was induced in 7 patients. The mechanism was focal in about half of them, in contrast to the first ablated AT, where the focal mechanism was the least common. After a median follow-up of 37 months, four patients had AT recurrence.
Conclusion
The patients with AT had biventricular dysfunction and bi-atrial dilatation. Aggressive induction and ablation of the induced AT may reduce the future AT recurrence.
背景：修复法洛四联症（TOF）的患者现在寿命更长。然而，心律失常在修复TOF的成年人中变得普遍，尤其是心房性心动过速。目的探讨心房心动过速（AT）患者的特点及临床和诱发性心房心动过速的发生机制。方法入选77例修复性TOF患者。患者分为AT组和非AT组。研究临床和电生理数据。结果患者平均年龄34岁。23例患者有AT（30%）。AT组左室射血分数较低（58±6 vs 62±5）；P = 0.011)，左右心房面积（cm2）较大(29±13 vs 15±5；P & lt;0.001, 19±3 vs 16±4；P & lt;右心室S波（cm/ S）较小(8±2 vs 10±3；p = 0.029)。AT患者行导管消融，32例AT消融。AT的发生机制为14例（44%）心房再入性心动过速，12例（37%）心房再入性心动过速，其余6例（19%）心房再入性心动过速。一个重要的发现是，在第一个AT消融后，7例患者又诱导了另一个AT。与第一次消融AT相比，其中大约一半的机制是焦点性的，焦点机制是最不常见的。中位随访37个月后，4例AT复发。结论AT患者存在双室功能障碍和双房扩张。积极诱导和消融诱发的AT可能减少未来AT的复发。
{"title":"Atrial tachycardia in patients with repaired tetralogy of Fallot; its characteristics and catheter ablation outcome","authors":"Qasim J. Naeemah , Miyako Igarashi , Noor K. Albakaa , Yuichi Hanaki , Noboru Ichihara , Chihiro Ota , Akira Kimata , Kojiro Ogawa , Naoto Kawamatsu , Tomoko Machino , Yuki Komatsu , Hiro Yamasaki , Akihiko Nogami , Masaki Ieda , Tomoko Ishizu","doi":"10.1016/j.ijcchd.2024.100558","DOIUrl":"10.1016/j.ijcchd.2024.100558","url":null,"abstract":"<div><h3>Background</h3><div>Patients with repaired tetralogy of Fallot (TOF) now live longer. However, dysrhythmia becomes prevalent in adults with repaired TOF, especially atrial tachyarrhythmia.</div></div><div><h3>Objective</h3><div>To identify the characteristics of patients who develop atrial tachycardia (AT) and the mechanism of the clinical AT and the induced one.</div></div><div><h3>Method</h3><div>Seventy-seven patients with repaired TOF were enrolled. The patients were divided into two groups (AT and non-AT). Clinical and electrophysiologic data were studied.</div></div><div><h3>Results</h3><div>The mean age was 34 years. Twenty-three patients had AT (30 %). In AT group, the left ventricular ejection fraction was lower (58 ± 6 vs 62 ± 5; <em>P</em> = 0.011), the right and left atrial area (cm<sup>2</sup>) was larger (29 ± 13 vs 15 ± 5; <em>P</em> < 0.001, and 19 ± 3 vs 16 ± 4; <em>P</em> < 0.001, respectively), and the right ventricular S′ wave (cm/s) was smaller (8 ± 2 vs 10 ± 3; <em>P</em> = 0.029).</div><div>Patients with AT underwent catheter ablation, and 32 AT were ablated. The mechanism of AT was intra-atrial reentrant tachycardia in 14 AT (44 %), cavotricuspid isthmus-dependent in 12 AT (37 %), and focal activity in the remaining 6 AT (19 %). An important finding was that after the first AT was ablated, another AT was induced in 7 patients. The mechanism was focal in about half of them, in contrast to the first ablated AT, where the focal mechanism was the least common. After a median follow-up of 37 months, four patients had AT recurrence.</div></div><div><h3>Conclusion</h3><div>The patients with AT had biventricular dysfunction and bi-atrial dilatation. Aggressive induction and ablation of the induced AT may reduce the future AT recurrence.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100558"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143562048","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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