International journal of cardiology. Congenital heart disease最新文献

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Physical exercise for pulmonary arterial hypertension diagnosis and therapy 体育锻炼对肺动脉高压的诊断和治疗
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2025-01-04 DOI: 10.1016/j.ijcchd.2025.100565
Myo Lwin , Abigail Masding , Colm McCabe
{"title":"Physical exercise for pulmonary arterial hypertension diagnosis and therapy","authors":"Myo Lwin , Abigail Masding , Colm McCabe","doi":"10.1016/j.ijcchd.2025.100565","DOIUrl":"10.1016/j.ijcchd.2025.100565","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100565"},"PeriodicalIF":0.8,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143452995","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}
引用次数: 0
Cardiac rehabilitation in ACHD: Further investment is now due ACHD的心脏康复:现在需要进一步的投资。
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-31 DOI: 10.1016/j.ijcchd.2024.100564
Ioannis Kasouridis , Heather Probert , Michael A. Gatzoulis
{"title":"Cardiac rehabilitation in ACHD: Further investment is now due","authors":"Ioannis Kasouridis , Heather Probert , Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2024.100564","DOIUrl":"10.1016/j.ijcchd.2024.100564","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100564"},"PeriodicalIF":0.8,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11772920/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143070034","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}
引用次数: 0
Toronto ACHD program: A 65 year legacy 多伦多ACHD项目:65年的历史。
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-30 DOI: 10.1016/j.ijcchd.2024.100563
J.M. Colman , W.G. Williams , C.K. Silversides , L. Harris , L. Benson , J. Heggie , R. Alonso-Gonzalez , E. Oechslin
{"title":"Toronto ACHD program: A 65 year legacy","authors":"J.M. Colman ,&nbsp;W.G. Williams ,&nbsp;C.K. Silversides ,&nbsp;L. Harris ,&nbsp;L. Benson ,&nbsp;J. Heggie ,&nbsp;R. Alonso-Gonzalez ,&nbsp;E. Oechslin","doi":"10.1016/j.ijcchd.2024.100563","DOIUrl":"10.1016/j.ijcchd.2024.100563","url":null,"abstract":"<div><div>The Toronto Adult Congenital Heart Disease (ACHD) Program at the University Health Network, University of Toronto, began in 1959. It traces its origins to a Paul Wood protégé, Dr. John Evans, and to a long-standing and supportive relationship with Hospital for Sick Children (SickKids), located just across the street. Over the decades, the program has grown to become a major center for training and research in ACHD and one of the largest clinical programs for ACHD care globally. This paper recounts the 65-year history of the program, including some of its key individuals, challenges, milestones, innovations, discoveries, and future aspirations.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100563"},"PeriodicalIF":0.8,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11783385/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143082376","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}
引用次数: 0
Ventricular arrhythmias and the role of antitachycardia pacing in patients with electrical heart disease and hypertrophic cardiomyopathy 心电性疾病和肥厚性心肌病患者室性心律失常和抗心动过速起搏的作用
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-28 DOI: 10.1016/j.ijcchd.2024.100562
Verena Kantenwein , Herribert Pavaci , Bernhard Haller , Marta Telishevska , Lena Friedrich , Maximilian Walgenbach , Carsten Lennerz , Christof Kolb
{"title":"Ventricular arrhythmias and the role of antitachycardia pacing in patients with electrical heart disease and hypertrophic cardiomyopathy","authors":"Verena Kantenwein ,&nbsp;Herribert Pavaci ,&nbsp;Bernhard Haller ,&nbsp;Marta Telishevska ,&nbsp;Lena Friedrich ,&nbsp;Maximilian Walgenbach ,&nbsp;Carsten Lennerz ,&nbsp;Christof Kolb","doi":"10.1016/j.ijcchd.2024.100562","DOIUrl":"10.1016/j.ijcchd.2024.100562","url":null,"abstract":"<div><h3>Background</h3><div>Whether it is reasonable to program ATP in patients with electrical heart disease (EHD) or hypertrophic cardiomyopathy (HCM) is not thoroughly clarified. Aim of the study was to define the types of ventricular arrhythmias and evaluate the safety and efficacy of ATP activation in these patients.</div></div><div><h3>Methods and results</h3><div>A total of 154 patients (53.9 % male, 64.9 % secondary prevention) with EHD or HCM, who had an implanted cardioverter defibrillator (ICD) with ATP activated, were included in this retrospective analysis; comprising a median of 65.0 months of follow-up. In 39/154 (25.3 %) patients appropriate ICD therapy was delivered during the follow-up. Patients with HCM had a significantly higher incidence rate of monomorphic VTs than patients with EHD (0.21 versus 0.01 per month, 0 &lt; 0.001). ATP terminated monomorphic VT with an efficacy of 88,2 % in 94.9 % of the occurring episodes. The incidence rate per month of torsade de pointes (TdP) tachycardia and VF was significantly higher in patients with EHD versus HCM (0.04 vs. 0.001, p=&lt;0.001; 0.06 vs. 0.007, p=&lt;0.001). The termination of TdP tachycardia and VF was associated with ATP in 14.0 % and 0 % (ATP efficacy of 28.3 % and 0 % respectively). The implantation for secondary prevention was associated with the occurrence of appropriate ICD therapy during the follow-up period (OR 3.94 [95%CI 1.53–10.14], p = 0.005).</div></div><div><h3>Conclusion</h3><div>Ventricular tachycardias in patients with HCM are primarily monomorphic and can be effectively terminated with ATP. In patients with EHD, TdP tachycardias and VF occur more frequently and are preferentially terminated by ICD shock.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100562"},"PeriodicalIF":0.8,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143463454","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}
引用次数: 0
Coarctation of the aorta and accelerated atherosclerosis: A contemporary review on the burden of atherosclerotic cardiovascular disease 主动脉缩窄和动脉粥样硬化加速:动脉粥样硬化性心血管疾病负担的当代回顾
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-25 DOI: 10.1016/j.ijcchd.2024.100561
Melissa G.Y. Lee , Phillip S. Naimo , Anoop N. Koshy , Edward Buratto , William M. Wilson , Leeanne E. Grigg , Subodh B. Joshi , Katherine M. English
{"title":"Coarctation of the aorta and accelerated atherosclerosis: A contemporary review on the burden of atherosclerotic cardiovascular disease","authors":"Melissa G.Y. Lee ,&nbsp;Phillip S. Naimo ,&nbsp;Anoop N. Koshy ,&nbsp;Edward Buratto ,&nbsp;William M. Wilson ,&nbsp;Leeanne E. Grigg ,&nbsp;Subodh B. Joshi ,&nbsp;Katherine M. English","doi":"10.1016/j.ijcchd.2024.100561","DOIUrl":"10.1016/j.ijcchd.2024.100561","url":null,"abstract":"<div><div>Coarctation of the aorta (CoA) is one of the most common types of congenital heart disease. Unfortunately, there is a high prevalence of hypertension and late cardiovascular mortality in patients with CoA despite successful repair. The growing impact of acquired cardiovascular disease remains a significant concern as the adult congenital heart disease population continues to rapidly expand and age. This review aims to explore (1) the determinants of vascular health and atherosclerosis including endothelial dysfunction and vascular wall abnormalities, (2) the prevalence of atherosclerosis and associated sequelae in repaired CoA including coronary artery disease, coronary artery calcium, aortic calcium, stroke, and peripheral artery disease, and (3) the contributing factors specific to CoA. This review aims to guide optimization of long-term cardiovascular health to ultimately reduce mortality and morbidity in this young high-risk population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100561"},"PeriodicalIF":0.8,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163931","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}
引用次数: 0
Using machine learning analysis to describe patterns in tissue Doppler and speckle tracking echocardiography in patients with transposition of the great arteries after arterial switch operation 应用机器学习分析描述大动脉转位手术后组织多普勒和斑点跟踪超声心动图的模式
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-20 DOI: 10.1016/j.ijcchd.2024.100560
Covadonga Terol Espinosa de los Monteros , Roel L.F. van der Palen , Jef Van den Eynde , Lukas Rammeloo , Mark G. Hazekamp , Nico A. Blom , Irene M. Kuipers , Arend D.J. ten Harkel
{"title":"Using machine learning analysis to describe patterns in tissue Doppler and speckle tracking echocardiography in patients with transposition of the great arteries after arterial switch operation","authors":"Covadonga Terol Espinosa de los Monteros ,&nbsp;Roel L.F. van der Palen ,&nbsp;Jef Van den Eynde ,&nbsp;Lukas Rammeloo ,&nbsp;Mark G. Hazekamp ,&nbsp;Nico A. Blom ,&nbsp;Irene M. Kuipers ,&nbsp;Arend D.J. ten Harkel","doi":"10.1016/j.ijcchd.2024.100560","DOIUrl":"10.1016/j.ijcchd.2024.100560","url":null,"abstract":"<div><h3>Background</h3><div>Advanced echocardiographic techniques such as Tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE) can detect more subtle changes in ventricular performance. We aimed to study the ventricular performance in patients with transposition of the great arteries (TGA) at mid-term follow-up after the arterial switch operation (ASO) with advanced echocardiographic techniques. In addition, we sought to discover new clinical phenotypes using unsupervised machine learning.</div></div><div><h3>Methods</h3><div>Conventional, TDI and STE echocardiographic parameters were prospectively obtained from 124 TGA patients (66.1 % male, age 10.8 ± 5.1 years, 24.2 % with ventricular septal defect) in this observational study. The data was analyzed with conventional statistics and new machine learning techniques.</div></div><div><h3>Results</h3><div>TGA patients had reduced biventricular systolic (septal s’ Z-score −2.28 ± 1.26; RV s’ Z-score −2.16 ± 0.71; mean left ventricular longitudinal strain Z-score of the LV -2.49 ± 1.68) and RV diastolic performance (RV E/e’ Z-score 2.35 ± 1.70) mid-term after ASO. Unsupervised clustering within the TGA population revealed 3 clusters. Interestingly, cluster 3 defined a group of patients with older age at ASO, the most reduced ventricular performance as well as the highest rates of reoperations and interventions.</div></div><div><h3>Conclusions</h3><div>Assessment of ventricular performance with TDI and STE 10 years after ASO showed that TGA patients have decreased biventricular systolic and diastolic function, especially at the septal regions. Novel analytical methods such as unsupervised clustering may help identify new clinical phenotypes from multiple variables and may contribute to improved risk stratification.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100560"},"PeriodicalIF":0.8,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163933","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}
引用次数: 0
Effect of pregnancy on bioprosthetic structural valve degeneration 妊娠对生物假体结构瓣膜变性的影响
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-20 DOI: 10.1016/j.ijcchd.2024.100559
Joshua J. Price , Kalani Ruiz , Jaewon Lim , Yuli Kim , Jonathan Buber
{"title":"Effect of pregnancy on bioprosthetic structural valve degeneration","authors":"Joshua J. Price ,&nbsp;Kalani Ruiz ,&nbsp;Jaewon Lim ,&nbsp;Yuli Kim ,&nbsp;Jonathan Buber","doi":"10.1016/j.ijcchd.2024.100559","DOIUrl":"10.1016/j.ijcchd.2024.100559","url":null,"abstract":"<div><h3>Background</h3><div>Despite increasing frequency of pregnancies among patients with cardiac conditions, including the presence of prosthetic heart valves, data on the effects of physiological changes during pregnancy on the function of bioprosthetic valves remains scarce and shows conflicting results.</div></div><div><h3>Objectives</h3><div>This study was aimed to determine the effect of pregnancy on the rate of bioprosthetic structural valve degeneration.</div></div><div><h3>Methods</h3><div>We designed a retrospective matched cohort study of patients seen between June 2018 and February 2023. All pregnant patients with bioprosthetic valves were matched to non-pregnant controls with prior valve replacement based on bioprosthetic valve location, time since valve implantation, age of the patient at valve implantation and time between baseline and follow up echocardiograms. Echocardiograms of pregnant patients were evaluated for bioprosthetic structural valve degeneration grade based on a dedicated scale before and after pregnancy. Non-pregnant controls had echocardiogram scoring of structural valve degeneration over a similar time period.</div></div><div><h3>Results</h3><div>Thirty four pregnant patients with bioprosthetic valves in the pulmonary, aortic and mitral positions were matched with 71 non-pregnant controls with identical bioprosthetic valves locations. Over a median follow up period of 13.5 months that included the gestational period, 18 (53 %) pregnant patients had an increase in structural valve degeneration score as compared to 17 (26 %) of the non-pregnant patients in median follow up of 13.7 months (OR 3.87, p = 0.004). On multivariable analysis, pregnancy was the only variable associated with increased structural valve degeneration score.</div></div><div><h3>Conclusions</h3><div>Our results suggest that pregnancy is associated with increased bioprosthetic structural valve degeneration.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100559"},"PeriodicalIF":0.8,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163934","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}
引用次数: 0
The Ahmanson/UCLA adult congenital heart disease center, at UCLA, los angeles, USA: The history of a leading ACHD institution and its vision for the future 位于美国洛杉矶加州大学洛杉矶分校的Ahmanson/UCLA成人先天性心脏病中心:一家领先的先天性心脏病机构的历史及其对未来的展望
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-11 DOI: 10.1016/j.ijcchd.2024.100556
Jamil Aboulhosn
{"title":"The Ahmanson/UCLA adult congenital heart disease center, at UCLA, los angeles, USA: The history of a leading ACHD institution and its vision for the future","authors":"Jamil Aboulhosn","doi":"10.1016/j.ijcchd.2024.100556","DOIUrl":"10.1016/j.ijcchd.2024.100556","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100556"},"PeriodicalIF":0.8,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143463453","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}
引用次数: 0
Congenital heart disease-related mortality during the first year of life: The peruvian experience 出生第一年与先天性心脏病相关的死亡率:秘鲁经验
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-12-10 DOI: 10.1016/j.ijcchd.2024.100557
Jose Arriola-Montenegro , Joel Coronado-Quispe , Juan Carlos Mego , Odalis Luis-Ybáñez , Astrid Tauma-Arrué , Samantha Chavez-Saldivar , Javier E. Sierra-Pagan , Miguel Pinto-Salinas , Rafael Marquez , Miguel Arboleda , Ivan Niño de Guzman , Luis Vera , Carlos Alvarez , Katia Bravo-Jaimes
{"title":"Congenital heart disease-related mortality during the first year of life: The peruvian experience","authors":"Jose Arriola-Montenegro ,&nbsp;Joel Coronado-Quispe ,&nbsp;Juan Carlos Mego ,&nbsp;Odalis Luis-Ybáñez ,&nbsp;Astrid Tauma-Arrué ,&nbsp;Samantha Chavez-Saldivar ,&nbsp;Javier E. Sierra-Pagan ,&nbsp;Miguel Pinto-Salinas ,&nbsp;Rafael Marquez ,&nbsp;Miguel Arboleda ,&nbsp;Ivan Niño de Guzman ,&nbsp;Luis Vera ,&nbsp;Carlos Alvarez ,&nbsp;Katia Bravo-Jaimes","doi":"10.1016/j.ijcchd.2024.100557","DOIUrl":"10.1016/j.ijcchd.2024.100557","url":null,"abstract":"<div><h3>Introduction</h3><div>Congenital heart disease (CHD) affects 1 % of all live births globally, with critical CHD (CCHD) requiring early intervention to avoid neonatal mortality. To date, CHD-related mortality within the first year of life and its associated factors have not been studied in Peru.</div></div><div><h3>Methods</h3><div>This is an observational secondary analysis using the Peruvian National Computerized System of Deaths, data from 2017 to 2021. CHD-related mortality was assessed by sex, age, altitude level, region, insurance type, healthcare institution, contributing factors, and CHD complexity. Geographic patterns were visualized using heatmaps, trends were analyzed through linear regression and survival analyses were performed using Kaplan Meier curves and log-rank tests.</div></div><div><h3>Results</h3><div>Peru experienced 70.6 CHD-related deaths per 100,000 live births. Mortality was significantly higher in males, most CHD-related deaths took place at a median age of 1 month. Regions like Huancavelica and Junín, located in the Andes, exhibited the highest mortality rates. Others significant related factors were receiving care at healthcare institution operated by Ministry of Health (MINSA) and complex CHDs. A non-significant increase in CHD-related deaths and mortality rates was noted, particularly affecting regions such as La Libertad and Lima.</div></div><div><h3>Conclusion</h3><div>Peru faces a critical public health crisis concerning CHD-related mortality among infants, especially those living in the Andes and receiving care at healthcare institutions operated by MINSA. A National Congenital Heart Disease Program is essential to ensure equitable access to specialized care, potentially saving numerous young lives.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100557"},"PeriodicalIF":0.8,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143463455","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}
引用次数: 0
Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch 左心发育不全综合征(HLHS)的年龄变化:评估包括新主动脉/主动脉弓在内的HLHS的年轻人
IF 0.8
International journal of cardiology. Congenital heart disease Pub Date : 2024-11-29 DOI: 10.1016/j.ijcchd.2024.100555
Polona Kačar , Pietro Paolo Tamborrino , Giulia Iannaccone , Gianfranco Butera , Margarita Brida , Katja Prokšelj , Michael A. Gatzoulis , Claudia Montanaro
{"title":"Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch","authors":"Polona Kačar ,&nbsp;Pietro Paolo Tamborrino ,&nbsp;Giulia Iannaccone ,&nbsp;Gianfranco Butera ,&nbsp;Margarita Brida ,&nbsp;Katja Prokšelj ,&nbsp;Michael A. Gatzoulis ,&nbsp;Claudia Montanaro","doi":"10.1016/j.ijcchd.2024.100555","DOIUrl":"10.1016/j.ijcchd.2024.100555","url":null,"abstract":"<div><div>Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects (CHD), characterized by a hypoplastic left ventricle (LV), dominant right ventricle (RV) and small left-sided heart structures. The introduction of the Norwood operation has significantly improved outcomes, with 5-year survival reported up to 65 %. Despite these advances, post-operative morbidity and mortality remain high, and the long-term complications in adult survivors represent a challenge. The number of HLHS patients with Fontan circulation is expected to double in the next 20 years, leading to a growing population requiring specialized care from adult congenital heart disease (ACHD) teams.</div><div>This article reviews current management strategies for HLHS, outlines potential long-term complications, and highlights existing knowledge gaps. Specific considerations in this population include the assessment of the neo-aorta and aortic arch, and systemic RV dysfunction in the setting of a Fontan circulation. The proposed surveillance strategy emphasizes the need for vigilant monitoring and timely intervention to treat the complications unique to this population, ensuring better outcomes for HLHS patients reaching adulthood.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100555"},"PeriodicalIF":0.8,"publicationDate":"2024-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163419","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}
引用次数: 0
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