Pub Date : 2025-03-01Epub Date: 2024-11-27DOI: 10.1016/j.ijcchd.2024.100554
Austin Angelotti , Maninder Dhesi , Shyam S. Bansal , Elisa A. Bradley
Introduction
Single ventricle congenital heart disease resulting in Fontan palliation has led to improved survival, however, Fontan-associated liver disease (FALD) is ubiquitous in this population by adulthood. While lymphopenia has been associated with the degree of FALD, potential immunologic mechanisms remain unstudied, and were the focus of this study.
Methods
Single-nuclei RNA-seq (snRNA-seq) data from liver samples of adolescent Fontan and control patients were analyzed with specific focus on lymphocytes and natural killer (NK) and T-cell fractions.
Results
Liver samples from Fontan patients demonstrated upregulation of endothelial cells (ECs: 4.2 ± 1.0 vs. 13.6 ± 3.4 %, p = 0.037) and total lymphocytes (0.7 ± 0.1 vs. 3.6 ± 0.7 %, p = 0.007), more specifically in NK and T-cells (NK: 0.29 ± 0.16 vs. 1.40 ± 0.64 %, p = 0.028 and T-cell: 0.28 ± 0.04 vs. 1.80 ± 1.01 %, p = 0.034). Enhanced genes important in T-cell activation and differentiation were demonstrated, as well as those involved in cell-to-cell adhesion and lymphocyte migration. Supporting lymphocyte trafficking, ECs demonstrated amplification of critical chemotactic and lymphocyte recruitment genes. Increased time from Fontan palliation was associated with more dramatic lymphocytic transcriptomic changes.
Conclusions
Hepatic changes in adolescent Fontan patients suggest that T-cells are contributing to the early development and possible progression of FALD.
{"title":"Novel immunologic mechanisms for Fontan-associated liver disease","authors":"Austin Angelotti , Maninder Dhesi , Shyam S. Bansal , Elisa A. Bradley","doi":"10.1016/j.ijcchd.2024.100554","DOIUrl":"10.1016/j.ijcchd.2024.100554","url":null,"abstract":"<div><h3>Introduction</h3><div>Single ventricle congenital heart disease resulting in Fontan palliation has led to improved survival, however, Fontan-associated liver disease (FALD) is ubiquitous in this population by adulthood. While lymphopenia has been associated with the degree of FALD, potential immunologic mechanisms remain unstudied, and were the focus of this study.</div></div><div><h3>Methods</h3><div>Single-nuclei RNA-seq (snRNA-seq) data from liver samples of adolescent Fontan and control patients were analyzed with specific focus on lymphocytes and natural killer (NK) and T-cell fractions.</div></div><div><h3>Results</h3><div>Liver samples from Fontan patients demonstrated upregulation of endothelial cells (ECs: 4.2 ± 1.0 vs. 13.6 ± 3.4 %, p = 0.037) and total lymphocytes (0.7 ± 0.1 vs. 3.6 ± 0.7 %, p = 0.007), more specifically in NK and T-cells (NK: 0.29 ± 0.16 vs. 1.40 ± 0.64 %, p = 0.028 and T-cell: 0.28 ± 0.04 vs. 1.80 ± 1.01 %, p = 0.034). Enhanced genes important in T-cell activation and differentiation were demonstrated, as well as those involved in cell-to-cell adhesion and lymphocyte migration. Supporting lymphocyte trafficking, ECs demonstrated amplification of critical chemotactic and lymphocyte recruitment genes. Increased time from Fontan palliation was associated with more dramatic lymphocytic transcriptomic changes.</div></div><div><h3>Conclusions</h3><div>Hepatic changes in adolescent Fontan patients suggest that T-cells are contributing to the early development and possible progression of FALD.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100554"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2025-01-31DOI: 10.1016/j.ijcchd.2025.100573
Tomas Pulido , Sofia de la Cruz-Perez , Daniela Valencia , Rafael Conde , Adrian Lescano , Nayeli Zayas
Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials. In contrast, in Latin America, registries have shown that PAH patients are younger and have fewer comorbidities, which raises the question of whether Latin American patients present a different phenotype or if we are lagging behind developed countries and whether we will experience a change in epidemiology in the next couple of years. We analyzed these trends in this review.
{"title":"Pulmonary arterial hypertension in Latin America. The age and comorbidity paradox","authors":"Tomas Pulido , Sofia de la Cruz-Perez , Daniela Valencia , Rafael Conde , Adrian Lescano , Nayeli Zayas","doi":"10.1016/j.ijcchd.2025.100573","DOIUrl":"10.1016/j.ijcchd.2025.100573","url":null,"abstract":"<div><div>Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials. In contrast, in Latin America, registries have shown that PAH patients are younger and have fewer comorbidities, which raises the question of whether Latin American patients present a different phenotype or if we are lagging behind developed countries and whether we will experience a change in epidemiology in the next couple of years. We analyzed these trends in this review.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100573"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143452996","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2025-01-29DOI: 10.1016/j.ijcchd.2025.100570
Mariana Sousa Paiva , Jorge Ferreira , Rui Anjos , Michael A. Gatzoulis
Advances in medical care have significantly extended the lifespan of patients with congenital heart disease (CHD), allowing most to survive into adulthood. However, they continue to face significant cardiovascular morbidity, particularly atrial arrhythmias (AA), heart failure, and thromboembolic (TE) events. TE events in adult CHD patients arise from various factors, including AA, intracardiac repairs, cyanotic CHD, Fontan palliation, pregnancy, and mechanical heart valves (MHV). As randomized clinical trials are lacking, most current guidelines rely on observational data and expert opinions, leading to inherent variability. While vitamin K antagonists are the only option for patients with MHV and significant mitral stenosis, direct oral anticoagulants appear to be a reasonable choice for other indications. In the presence of AA, complex conditions alone may justify anticoagulation, whereas thromboembolic and haemorrhagic risks should be evaluated individually for simpler lesions. This review summarizes the available evidence and makes relevant recommendations regarding thromboprophylaxis in ACHD patients, focusing on indications, risk scores, and therapies.
{"title":"Thromboprophylaxis and adult congenital heart disease: The latest on indications, risk scoring and therapy","authors":"Mariana Sousa Paiva , Jorge Ferreira , Rui Anjos , Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2025.100570","DOIUrl":"10.1016/j.ijcchd.2025.100570","url":null,"abstract":"<div><div>Advances in medical care have significantly extended the lifespan of patients with congenital heart disease (CHD), allowing most to survive into adulthood. However, they continue to face significant cardiovascular morbidity, particularly atrial arrhythmias (AA), heart failure, and thromboembolic (TE) events. TE events in adult CHD patients arise from various factors, including AA, intracardiac repairs, cyanotic CHD, Fontan palliation, pregnancy, and mechanical heart valves (MHV). As randomized clinical trials are lacking, most current guidelines rely on observational data and expert opinions, leading to inherent variability. While vitamin K antagonists are the only option for patients with MHV and significant mitral stenosis, direct oral anticoagulants appear to be a reasonable choice for other indications. In the presence of AA, complex conditions alone may justify anticoagulation, whereas thromboembolic and haemorrhagic risks should be evaluated individually for simpler lesions. This review summarizes the available evidence and makes relevant recommendations regarding thromboprophylaxis in ACHD patients, focusing on indications, risk scores, and therapies.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100570"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143452993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2025-01-30DOI: 10.1016/j.ijcchd.2025.100571
Giulia Guglielmi , Konstantinos Dimopoulos , S. John Wort
Pulmonary Arterial Hypertension (PAH) is a complex and progressive disease characterized by elevated pulmonary vascular resistance and right heart failure. Current therapies primarily focus on pulmonary vasodilation; however, novel approaches that target the underlying pathophysiological mechanisms—such as TGF-β signalling, epigenetic alterations, growth factors, inflammation, and extracellular matrix remodelling—are promising alternatives for improving treatment outcomes. This is a review of recent advances in the development of innovative therapeutic strategies for PAH.
The first section of this paper explores approaches targeting TGF-β signalling, both acting directly on receptors through drugs like Sotatercept and exogenous BMP9, and indirectly, inhibiting the degradation of key receptors, such as BMPR2. Subsequent sections describe treatments that target epigenetic regulators, e.g. poly (ADP-ribose) polymerase-1 (PARP-1) inhibitors and direct BRD4 antagonists, tyrosine kinase inhibitors (Seralutinib), and therapies aimed at inflammation, such as IL-6 inhibitors, CD-20 inhibitors, and monoclonal antibodies that prevent macrophage migration. Finally, strategies that target the serotonin pathway, and other metabolic and hormonal pathways are described.
This review includes both preclinical and clinical trial data that support efficacy, safety and the future potential of such therapies. Collectively, these therapeutic approaches can be valuable in treating PAH by targeting multiple aspects of its pathogenesis, potentially resulting in improved clinical outcomes for patients affected by this debilitating, life-limiting condition.
{"title":"New therapies in pulmonary arterial hypertension: Recent insights","authors":"Giulia Guglielmi , Konstantinos Dimopoulos , S. John Wort","doi":"10.1016/j.ijcchd.2025.100571","DOIUrl":"10.1016/j.ijcchd.2025.100571","url":null,"abstract":"<div><div>Pulmonary Arterial Hypertension (PAH) is a complex and progressive disease characterized by elevated pulmonary vascular resistance and right heart failure. Current therapies primarily focus on pulmonary vasodilation; however, novel approaches that target the underlying pathophysiological mechanisms—such as TGF-β signalling, epigenetic alterations, growth factors, inflammation, and extracellular matrix remodelling—are promising alternatives for improving treatment outcomes. This is a review of recent advances in the development of innovative therapeutic strategies for PAH.</div><div>The first section of this paper explores approaches targeting TGF-β signalling, both acting directly on receptors through drugs like Sotatercept and exogenous BMP9, and indirectly, inhibiting the degradation of key receptors, such as BMPR2. Subsequent sections describe treatments that target epigenetic regulators, e.g. poly (ADP-ribose) polymerase-1 (PARP-1) inhibitors and direct BRD4 antagonists, tyrosine kinase inhibitors (Seralutinib), and therapies aimed at inflammation, such as IL-6 inhibitors, CD-20 inhibitors, and monoclonal antibodies that prevent macrophage migration. Finally, strategies that target the serotonin pathway, and other metabolic and hormonal pathways are described.</div><div>This review includes both preclinical and clinical trial data that support efficacy, safety and the future potential of such therapies. Collectively, these therapeutic approaches can be valuable in treating PAH by targeting multiple aspects of its pathogenesis, potentially resulting in improved clinical outcomes for patients affected by this debilitating, life-limiting condition.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100571"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2024-11-20DOI: 10.1016/j.ijcchd.2024.100553
Josu Erquicia Peralt , Larraitz Orive Melero , Jagoba Larrazabal López , José Félix Larrea Egurbide , Eugenia García Fernández , Luis Fernández González , Roberto Blanco Mata , Josune Arriola Meabe
Introduction
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that in most cases manifests in the first months of life, being a well-studied entity of myocardial ischaemia in young patients. It has been reported that up to 90 % of infants die without treatment in the first year, although late diagnosis is increasingly reported in adults.
Case summary
We present the case of a 23-year-old woman of Moroccan origin with no medical history of interest, who was admitted to the cardiac intensive care unit after an episode of sustained ventricular tachycardia (VT) that required electrical cardioversion. On admission, the electrocardiogram and blood test were normal, but the transthoracic echocardiogram showed moderate left ventricular systolic dysfunction and severe mitral regurgitation of rheumatic aetiology which was later confirmed with a transesophageal echocardiogram. In view of the arrhythmic event presented, a complete study was performed with cardiac magnetic resonance imaging (MRI) which showed no pathological enhancements. Nevertheless, coronary artery angiography revealed an anomalous origin of the left coronary artery in the pulmonary artery which was confirmed by cardiac computed tomography (CT). Corrective surgery was performed with direct translocation of the left coronary artery to the aorta and mitral valve replacement.
Discussion
In ALCAPA, the coronary steal phenomenon produced from the pulmonary artery to the coronary circulation causes myocardial ischaemia and involves a left-to-right shunt. Myocardial ischaemia is cause of angina, systolic dysfunction, mitral regurgitation and sudden cardiac death in children and young adults.
{"title":"Concurrent finding of rheumatic severe mitral regurgitation and anomalous left coronary artery from the pulmonary artery (ALCAPA) in a 23-year-old patient consulting for ventricular tachycardia: A case report and a review of the literature","authors":"Josu Erquicia Peralt , Larraitz Orive Melero , Jagoba Larrazabal López , José Félix Larrea Egurbide , Eugenia García Fernández , Luis Fernández González , Roberto Blanco Mata , Josune Arriola Meabe","doi":"10.1016/j.ijcchd.2024.100553","DOIUrl":"10.1016/j.ijcchd.2024.100553","url":null,"abstract":"<div><h3>Introduction</h3><div>Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that in most cases manifests in the first months of life, being a well-studied entity of myocardial ischaemia in young patients. It has been reported that up to 90 % of infants die without treatment in the first year, although late diagnosis is increasingly reported in adults.</div></div><div><h3>Case summary</h3><div>We present the case of a 23-year-old woman of Moroccan origin with no medical history of interest, who was admitted to the cardiac intensive care unit after an episode of sustained ventricular tachycardia (VT) that required electrical cardioversion. On admission, the electrocardiogram and blood test were normal, but the transthoracic echocardiogram showed moderate left ventricular systolic dysfunction and severe mitral regurgitation of rheumatic aetiology which was later confirmed with a transesophageal echocardiogram. In view of the arrhythmic event presented, a complete study was performed with cardiac magnetic resonance imaging (MRI) which showed no pathological enhancements. Nevertheless, coronary artery angiography revealed an anomalous origin of the left coronary artery in the pulmonary artery which was confirmed by cardiac computed tomography (CT). Corrective surgery was performed with direct translocation of the left coronary artery to the aorta and mitral valve replacement.</div></div><div><h3>Discussion</h3><div>In ALCAPA, the coronary steal phenomenon produced from the pulmonary artery to the coronary circulation causes myocardial ischaemia and involves a left-to-right shunt. Myocardial ischaemia is cause of angina, systolic dysfunction, mitral regurgitation and sudden cardiac death in children and young adults.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100553"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142759308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2024-11-14DOI: 10.1016/j.ijcchd.2024.100551
Junfang Shi , Baiqiang Wang , Weida Lu , Xiao Meng
Background
To report one young man suffered from retinal artery occlusion (RAO) due to patent foramen ovale (PFO).
Objective
The aim of this study was to assess the mechanism for RAO in a patient with a PFO.
Method
A 35-year-old male who developed RAO was studied. He received transoesophageal echocardiography and a right-to-left shunt across of a PFO was found. Then, the patient was scheduled for percutaneous PFO closure. An optical coherence tomography (OCT) catheter was positioned into the PFO tunnel and the microstructure of PFO was observed. We found that there were several white thrombi in the tunnel; the PFO was successfully closed. At 10-month follow-up, the patient recovered well and had no new retinal artery occlusion or stroke.
Conclusions
PFO was the site of thrombus formation in our case of RAO as confirmed by OCT. PFO should be considered in young patients diagnosed with RAO with no other risk factors.
{"title":"Patent foramen ovale-induced retinal artery occlusion: Evidence for in situ Thromobosis","authors":"Junfang Shi , Baiqiang Wang , Weida Lu , Xiao Meng","doi":"10.1016/j.ijcchd.2024.100551","DOIUrl":"10.1016/j.ijcchd.2024.100551","url":null,"abstract":"<div><h3>Background</h3><div>To report one young man suffered from retinal artery occlusion (RAO) due to patent foramen ovale (PFO).</div></div><div><h3>Objective</h3><div>The aim of this study was to assess the mechanism for RAO in a patient with a PFO.</div></div><div><h3>Method</h3><div>A 35-year-old male who developed RAO was studied. He received transoesophageal echocardiography and a right-to-left shunt across of a PFO was found. Then, the patient was scheduled for percutaneous PFO closure. An optical coherence tomography (OCT) catheter was positioned into the PFO tunnel and the microstructure of PFO was observed. We found that there were several white thrombi in the tunnel; the PFO was successfully closed. At 10-month follow-up, the patient recovered well and had no new retinal artery occlusion or stroke.</div></div><div><h3>Conclusions</h3><div>PFO was the site of thrombus formation in our case of RAO as confirmed by OCT. PFO should be considered in young patients diagnosed with RAO with no other risk factors.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100551"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2025-01-07DOI: 10.1016/j.ijcchd.2025.100566
Gurleen Wander , Claudia Montanaro , Prithvi Dixit , Daryl Dob , Mark R. Johnson , Roshni R. Patel
Pulmonary hypertension (PH) is a term used to describe a complex heterogenous group of conditions defined by a mean pulmonary arterial pressure of more than 20 mmHg at rest on right-heart catheterization. PH in pregnancy is associated with high rates of maternal morbidity and mortality and poor fetal outcomes. Currently, pregnancy in these women is classified as modified WHO class IV (pregnancy contraindicated). More recent data suggest that the prognosis varies with the underlying aetiology as well as with PH severity. Consequently, management during pregnancy must be individualised, with patients cared for in a tertiary unit as part of an experienced multidisciplinary team. In this article, we will discuss the importance of preconception counselling, the impact of the haemodynamic changes induced by pregnancy, the maternal and fetal risks of pregnancy in women with PH, and how these can be minimised by close antenatal, intrapartum and post-partum care and the development of individualised pregnancy plans.
{"title":"Pregnancy and pulmonary artery hypertension: Management challenges","authors":"Gurleen Wander , Claudia Montanaro , Prithvi Dixit , Daryl Dob , Mark R. Johnson , Roshni R. Patel","doi":"10.1016/j.ijcchd.2025.100566","DOIUrl":"10.1016/j.ijcchd.2025.100566","url":null,"abstract":"<div><div>Pulmonary hypertension (PH) is a term used to describe a complex heterogenous group of conditions defined by a mean pulmonary arterial pressure of more than 20 mmHg at rest on right-heart catheterization. PH in pregnancy is associated with high rates of maternal morbidity and mortality and poor fetal outcomes. Currently, pregnancy in these women is classified as modified WHO class IV (pregnancy contraindicated). More recent data suggest that the prognosis varies with the underlying aetiology as well as with PH severity. Consequently, management during pregnancy must be individualised, with patients cared for in a tertiary unit as part of an experienced multidisciplinary team. In this article, we will discuss the importance of preconception counselling, the impact of the haemodynamic changes induced by pregnancy, the maternal and fetal risks of pregnancy in women with PH, and how these can be minimised by close antenatal, intrapartum and post-partum care and the development of individualised pregnancy plans.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100566"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143452997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01Epub Date: 2024-12-30DOI: 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
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.
{"title":"Toronto ACHD program: A 65 year legacy","authors":"J.M. Colman , W.G. Williams , C.K. Silversides , L. Harris , L. Benson , J. Heggie , R. Alonso-Gonzalez , 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":"2025-03-01","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}
Pub Date : 2025-03-01Epub Date: 2024-12-10DOI: 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
Introduction
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.
Methods
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.
Results
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.
Conclusion
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.
{"title":"Congenital heart disease-related mortality during the first year of life: The peruvian experience","authors":"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","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":"2025-03-01","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}
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