International journal of cardiology. Congenital heart disease最新文献

{"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":"2025-06-01","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}

{"title":"Reflections on transfemoral TAVI in dextrocardia with severe aortic regurgitation: Navigating anatomical complexity, off-label innovation, and future directions","authors":"Pandula Athauda arachchi ,&nbsp;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}

{"title":"Excellent medium to long term outcomes after cardiac surgery for moderate and complex congenital heart disease, regardless of geographic location","authors":"Larissa Lloyd ,&nbsp;Calum Nicholson ,&nbsp;Geoff Strange ,&nbsp;Rachael Cordina ,&nbsp;David S. Celermajer ,&nbsp;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":"2025-06-01","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}

{"title":"Impact of smoking on cardiovascular health: Mechanisms, epidemiology and specific concerns regarding congenital heart disease","authors":"Mishary Alhindal ,&nbsp;Jood Janahi ,&nbsp;Emanuela C. D'Angelo ,&nbsp;Veronica Lisignoli ,&nbsp;Rosalinda Palmieri ,&nbsp;Antonella Cutrì ,&nbsp;Gianfranco Butera ,&nbsp;Michael A. Gatzoulis ,&nbsp;Claudia Montanaro","doi":"10.1016/j.ijcchd.2025.100581","DOIUrl":"10.1016/j.ijcchd.2025.100581","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100581"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143776653","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}

{"title":"Physical activity and weight are important predictors of health related quality of life in adults with congenital heart disease","authors":"Constance G. Weismann ,&nbsp;Frishta Jafar ,&nbsp;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 &lt; 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 &lt; 0.001), male sex (OR 1.58, p &lt; 0.001), mild-moderate CHD (OR 1.59, p &lt; 0.001) and being of normal/overweight (OR 1.44, p &lt; 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}

{"title":"Overcoming borders: International cooperation in re-use and re-interpretation of omics data in Fontan circulation","authors":"Miriam Michel ,&nbsp;David Renaud ,&nbsp;Benjamin Kelly ,&nbsp;Ismael Z. Assi ,&nbsp;Liming Pei ,&nbsp;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}

{"title":"Temporal change in the age at time of death in adults with congenital heart disease","authors":"Zeyad Kholeif,&nbsp;Omar Abozied,&nbsp;Ahmed T. Abdelhalim,&nbsp;Sara ElZalabany,&nbsp;Amr Moustafa,&nbsp;Ali Ali,&nbsp;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 &lt; 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 &lt; 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 &lt; 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}

{"title":"The impact of tricuspid regurgitation on timing pulmonary valve replacement in ACHD patients after tetralogy of Fallot repair","authors":"Gianluca Brancaccio ,&nbsp;Veronica Bordonaro ,&nbsp;Matteo Trezzi ,&nbsp;Marcello Chinali ,&nbsp;Delia Fugallo ,&nbsp;Carolina D'Anna ,&nbsp;Veronica Lisignoli ,&nbsp;Marin Verrengia ,&nbsp;Fabio Miraldi ,&nbsp;Gianfranco Butera ,&nbsp;Roberta Iacobelli ,&nbsp;Benedetta Leonardi ,&nbsp;Claudia Montanaro ,&nbsp;Aurelio Secinaro ,&nbsp;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}

{"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 ,&nbsp;Madelien V. Regeer ,&nbsp;Adrianus P. Wijnmaalen ,&nbsp;Monique R.M. Jongbloed ,&nbsp;Mark G. Hazekamp ,&nbsp;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 &gt;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}

{"title":"Posttraumatic stress disorder symptoms in adults with congenital heart disease (ACHD) during the COVID-19 pandemic in Norway","authors":"Grimholt T.K. ,&nbsp;Gjesdal O. ,&nbsp;Bonsaksen T. ,&nbsp;Heir T. ,&nbsp;Ekeberg O. ,&nbsp;Schou Bredal I. ,&nbsp;Skogstad L. ,&nbsp;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}