International journal of cardiology. Congenital heart disease最新文献

{"title":"Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease","authors":"Andrea R. Hsu ,&nbsp;Snigdha Karnakoti ,&nbsp;Ahmed T. Abdelhalim ,&nbsp;Jason H. Anderson ,&nbsp;C. Charles Jain ,&nbsp;William R. Miranda ,&nbsp;Elizabeth H. Stephens ,&nbsp;Joseph A. Dearani ,&nbsp;Hartzell V. Schaff ,&nbsp;Heidi M. Connolly ,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100598","DOIUrl":"10.1016/j.ijcchd.2025.100598","url":null,"abstract":"<div><h3>Background</h3><div>Bioprosthetic valves are frequently implanted in adults with congenital heart disease (CHD), but comprehensive data on bioprosthetic valve longevity and outcomes across all valve positions are lacking. The purpose of this study was to determine the incidence and risk factors for bioprosthetic valve dysfunction (BVD) across all valve positions in adults with CHD.</div></div><div><h3>Method</h3><div>BVD was defined as Doppler mean gradient &gt;40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient &gt;10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.</div></div><div><h3>Results</h3><div>There were 1221 adults with CHD that underwent implantation of a bioprosthetic valve (age 39 ± 16 years, males 617 [51 %]). At the time of valve implantation, 755 (62 %), 325 (27 %), 176 (14 %), and 21 (1.7 %) received a bioprosthetic valve in the pulmonary, tricuspid, aortic, and mitral positions, respectively. The 10-year cumulative incidence of BVD was 28 %, 36 %, 43 %, and 68 % for pulmonary, tricuspid, aortic, and mitral bioprosthetic valves, respectively (p &lt; 0.001). Using the pulmonary valve position as the reference group, valve position (tricuspid [HR 1.26, 95 % CI 1.09–1.58), aortic [HR 1.64, 95 % CI 1.22–1.97], mitral [HR 2.01, 95 % CI 0.83–4.05]) were associated with higher risk of BVD. Younger age was associated higher risk of BVD (HR 0.95, 95 % CI 0.86–0.98 per 5-year increment).</div></div><div><h3>Conclusions</h3><div>These data support the existing evidence that left-sided bioprosthetic valves demonstrate reduced durability in young patients and should be included in counseling and clinical decision-making.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100598"},"PeriodicalIF":0.8,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306423","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":"Utilizing optical coherence tomography to explore pulmonary arteries and pulmonary vascular disease in Fontan patients","authors":"François Côté ,&nbsp;Leonaes Brahel Tatchinda Kuete ,&nbsp;Réda Ibrahim ,&nbsp;Jean-Claude Tardif ,&nbsp;Colombe Roy ,&nbsp;François-Pierre Mongeon ,&nbsp;Annie Dore ,&nbsp;Blandine Mondésert ,&nbsp;Paul Khairy ,&nbsp;Marie-A. Chaix","doi":"10.1016/j.ijcchd.2025.100599","DOIUrl":"10.1016/j.ijcchd.2025.100599","url":null,"abstract":"<div><h3>Background</h3><div>The Fontan procedure is the palliative intervention of choice for patients with single ventricle physiology. Pulmonary vascular disease (PVD) is a major contributor to Fontan circulatory failure. Our study utilized optical coherence tomography (OCT) imagery in pulmonary arteries to better characterize PVD in Fontan patients.</div></div><div><h3>Methods</h3><div>In a prospective pilot study, OCT images were acquired during cardiac catheterization in a distal left pulmonary artery (PA) branch (A10). The PA intima-media ratio and wall-vessel ratio (i.e., difference between the external elastic membrane area and the luminal area divided by the external elastic membrane area) were measured off-line.</div></div><div><h3>Results</h3><div>A total of 9 Fontan patients (5 women), median age of 23.0 (21.0,38.0) years, were included. All patients exhibited an intima-to-media ratio above 1.0. The ratio exceeded 2.8 in patients with high pulmonary vascular resistance. No difference is observed between patients for the wall-vessel ratio. No relation between time of completion of Fontan and intima-media ratio is identified.</div></div><div><h3>Conclusion</h3><div>OCT shows promise in evaluating PVD in patients with Fontan palliation. PA remodeling is characterized by intimal hyperplasia with medial regression. Further studies are required to determine the role of OCT in guiding treatment decisions and assessing therapeutic responses.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100599"},"PeriodicalIF":0.8,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306424","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":"A detailed profiling study from the national Australian Congenital Heart Disease registry: rationale and design","authors":"Tanya Badal ,&nbsp;Calum Nicholson ,&nbsp;Julian Ayer ,&nbsp;Michael Cheung ,&nbsp;Leeanne Grigg ,&nbsp;David S. Celermajer ,&nbsp;Geoff Strange","doi":"10.1016/j.ijcchd.2025.100595","DOIUrl":"10.1016/j.ijcchd.2025.100595","url":null,"abstract":"<div><h3>Background</h3><div>Previous research has outlined significant gaps in the care of children and adults with Congenital Heart Disease (CHD). The Australian Study of the Burden and Outcomes of CHD will assess medical, psychological, social and economic issues affecting CHD patients and carers and provide a detailed account of CHD patients’ healthcare utilisation.</div></div><div><h3>Methods</h3><div>A random sample of patients aged ≥5 years, equally split between genders, CHD complexity (mild, moderate, severe) and rural and remote locations (20–25%) was extracted from the National Australian CHD Registry (n = 68,214) from four large participating CHD centres (2 adult and 2 child). Patients and their carers were invited to participate in an in-person study visit for physical assessment and for a series of surveys questioning physical and mental health, neurodevelopment, and quality of life.</div></div><div><h3>Results</h3><div>Of 6,726 contacted patients, 1,911 (28%) consented, with 99.5% of surveys completed online. 1,658 consented participants completed at least one study activity (87% response rate). Proxies/carers completed surveys for 11% of participants (primarily those under age 8 or with intellectual disability). All study activities were completed by 750 respondents (45%). Respondents were older (median age 24 [IQR 12–41 years] vs 18 [IQR 11–35 years], p = 0.005) and had a higher proportion of severe CHD (28%) compared to non-respondents (23%). No significant differences in disease complexity were observed between respondents and non-respondents.</div></div><div><h3>Conclusion</h3><div>This multi-site study of over 1600 CHD patients aims to offer the first comprehensive profiling of the burdens and outcomes faced by Australia's growing CHD population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100595"},"PeriodicalIF":0.8,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144330248","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 arterial hypertension treatment. A new era","authors":"Sophia Anastasia Mouratoglou ,&nbsp;Alexandra Arvanitaki ,&nbsp;George Papadopoulos ,&nbsp;Rogerio Souza ,&nbsp;George Giannakoulas","doi":"10.1016/j.ijcchd.2025.100594","DOIUrl":"10.1016/j.ijcchd.2025.100594","url":null,"abstract":"<div><div>Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100594"},"PeriodicalIF":0.8,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144221932","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":"The PAH patient's perspective","authors":"Daniel A. Jeffery ,&nbsp;Wendy Gin-Sing ,&nbsp;Radwa Bedair ,&nbsp;Luke S. Howard","doi":"10.1016/j.ijcchd.2025.100596","DOIUrl":"10.1016/j.ijcchd.2025.100596","url":null,"abstract":"<div><div>Pulmonary Hypertension is a serious condition which can occur in adults living with congenital heart disease. Due to advances in the care of patients with congenital heart disease, patients are living longer with the condition. Unfortunately, patients living with Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) face a number of different issues directly related to their condition, which range from physical limitations and uncertainty over their diagnosis, which in some cases cause significant depression and anxiety, to impact on their relationships with friends and family, financial challenges and family planning.</div><div>In this article, we interview two patients living with PAH-CHD and discuss the numerous issues that patients may face as a direct result of their condition.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100596"},"PeriodicalIF":0.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144243004","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":"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}