International journal of cardiology. Congenital heart disease最新文献

{"title":"Evaluation of prosthetic dysfunction in the diagnosis of endocarditis associated to prosthetic pulmonary valve and pulmonary conduit","authors":"Andrés Cano Pérez ,&nbsp;Larraitz Orive Melero ,&nbsp;Jose Félix Larrea Egurbide ,&nbsp;Jagoba Larrazábal López ,&nbsp;Luis Fernández González ,&nbsp;Roberto Blanco Mata ,&nbsp;Josune Arriola Meabe ,&nbsp;Leire Artiñano Mendizábal ,&nbsp;Ane Josune Goikoetxea Agirre ,&nbsp;María José Blanco Vidal ,&nbsp;Javier Ayala Curiel ,&nbsp;Pedro María Montes Orbe","doi":"10.1016/j.ijcchd.2025.100591","DOIUrl":"10.1016/j.ijcchd.2025.100591","url":null,"abstract":"<div><h3>Introduction</h3><div>The number of cases of infective endocarditis associated to prosthetic pulmonary valves and pulmonary conduits (PPVIE) is likely to increase in the future. Transthoracic echocardiography (TTE) presents challenges in visualizing lesions suggestive of endocarditis in these patients. However, TTE may provide additional findings, such as prosthetic dysfunction, which can guide the diagnosis of this condition. The main objective of this study is to analyze prosthetic pulmonary valve dysfunction as an echocardiographic manifestation of PPVIE.</div></div><div><h3>Methods</h3><div>All cases of PPVIE (definite and possible, according to the modified Duke criteria) at Cruces University Hospital (Baracaldo, Spain) between January 2014 and July 2024 were included. Prosthetic dysfunction was defined as a peak transvalvular gradient (PTG) ≥25 mmHg for homografts and ≥40 mmHg for prosthetic pulmonary valves and bovine pulmonary conduits (stenosis) and/or pulmonary regurgitation (PR) moderate or severe. Number of cases of prosthetic dysfunction between the PPVIE episode and prior to the episode were compared. We analyzed the mechanisms of prosthetic dysfunction in the PPVIE episode.</div></div><div><h3>Results</h3><div>14 cases of PPVIE were identified. In cases prior to the PPVIE episode, 42.9 % had prosthetic dysfunction, while in the PPVIE episode, 92.3 % had prosthetic dysfunction. Stenosis was a more frequent cause of valve dysfunction than PR.</div></div><div><h3>Conclusions</h3><div>Prosthetic dysfunction (due to stenosis or regurgitation) is a relevant finding in the diagnosis of PPVIE in cases where lesions suggestive of endocarditis are not visualised. Although not included in the Duke criteria, stenosis is a more frequent mechanism of dysfunction than PR.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100591"},"PeriodicalIF":0.8,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144139013","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":"2025-05-05","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":"Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead","authors":"Raksheeth Agarwal ,&nbsp;Louise E. Coats ,&nbsp;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}

{"title":"Percutaneous repair of biatrial drainage of the right superior vena cava using a covered stent","authors":"Samuel L. Casella ,&nbsp;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}

{"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":"2025-04-25","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}

{"title":"Correlation between Olink and SomaScan proteomics platforms in adults with a Fontan circulation","authors":"Ismael Z. Assi ,&nbsp;Michael J. Landzberg ,&nbsp;Kristian C. Becker ,&nbsp;David Renaud ,&nbsp;Fernando Baraona Reyes ,&nbsp;David M. Leone ,&nbsp;Mark Benson ,&nbsp;Miriam Michel ,&nbsp;Robert E. Gerszten ,&nbsp;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 &gt; 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}

{"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}

{"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}

{"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":"2025-04-08","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}

{"title":"Feasibility and effectiveness of telemedicine for adult patients with congenital heart disease: A one-year single-center experience-based study","authors":"Nunzia Borrelli ,&nbsp;Nicola Grimaldi ,&nbsp;Flavia Fusco ,&nbsp;Antonio Orlando ,&nbsp;Michela Palma ,&nbsp;Maria Cristina Boccia ,&nbsp;Sabrina Bassolino ,&nbsp;Anna Iervolino ,&nbsp;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}