Pub Date : 2025-06-30DOI: 10.1016/j.ijcchd.2025.100607
Zahra Imran , Taimor Mohammed Khan , Saad Ahmed Waqas , Raheel Ahmed , Abdul Mannan Khan Minhas
Purpose
Congenital heart disease (CHD) remains the leading cause of infant mortality due to birth defects in the United States. We analyzed long-term CHD mortality trends across age, sex, and racial groups from 1968 to 2022.
Methods
We extracted CHD mortality data from the CDC WONDER database (1968–2022), using ICD-8/9/10 codes. Age-adjusted mortality rates (AAMRs) and crude mortality rates (CMRs) were calculated per 100,000 population. Joinpoint regression was used to assess temporal trends in mortality, reporting annual percentage changes (APCs) and average APCs (AAPCs) with 95 % confidence intervals (CIs).
Results
From 1968 to 2019, 234,658 CHD-related deaths were recorded. Overall AAMR declined from 3.2 (1968) to 0.8 (2019) per 100,000 (AAPC: −2.7 %; 95 % CI: −2.9 to −2.5). Males consistently had higher AAMRs than females. Racial disparities persisted, with slower declines among Black or African American individuals. Infants under 1 year accounted for 56.0 % of CHD deaths and showed the steepest mortality decline (AAPC: −3.2 %). Mortality rates plateaued in recent years.
Conclusions
CHD mortality in the U.S. has declined markedly over the past five decades, though progress has slowed since 2009. Persistent disparities by race and sex emphasize the need for equitable access to specialized CHD care and ongoing public health efforts.
{"title":"Trends in congenital heart disease mortality in the United States from 1968 to 2022","authors":"Zahra Imran , Taimor Mohammed Khan , Saad Ahmed Waqas , Raheel Ahmed , Abdul Mannan Khan Minhas","doi":"10.1016/j.ijcchd.2025.100607","DOIUrl":"10.1016/j.ijcchd.2025.100607","url":null,"abstract":"<div><h3>Purpose</h3><div>Congenital heart disease (CHD) remains the leading cause of infant mortality due to birth defects in the United States. We analyzed long-term CHD mortality trends across age, sex, and racial groups from 1968 to 2022.</div></div><div><h3>Methods</h3><div>We extracted CHD mortality data from the CDC WONDER database (1968–2022), using ICD-8/9/10 codes. Age-adjusted mortality rates (AAMRs) and crude mortality rates (CMRs) were calculated per 100,000 population. Joinpoint regression was used to assess temporal trends in mortality, reporting annual percentage changes (APCs) and average APCs (AAPCs) with 95 % confidence intervals (CIs).</div></div><div><h3>Results</h3><div>From 1968 to 2019, 234,658 CHD-related deaths were recorded. Overall AAMR declined from 3.2 (1968) to 0.8 (2019) per 100,000 (AAPC: −2.7 %; 95 % CI: −2.9 to −2.5). Males consistently had higher AAMRs than females. Racial disparities persisted, with slower declines among Black or African American individuals. Infants under 1 year accounted for 56.0 % of CHD deaths and showed the steepest mortality decline (AAPC: −3.2 %). Mortality rates plateaued in recent years.</div></div><div><h3>Conclusions</h3><div>CHD mortality in the U.S. has declined markedly over the past five decades, though progress has slowed since 2009. Persistent disparities by race and sex emphasize the need for equitable access to specialized CHD care and ongoing public health efforts.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100607"},"PeriodicalIF":0.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144614325","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}
Pub Date : 2025-06-30DOI: 10.1016/j.ijcchd.2025.100606
Arundhati Sharma , Anubhuti Sharma , Kalyan Chaliki , Marco Antonio Fernandes , Francois Marcotte , Satyajit Reddy
Background
Coarctation of the aorta (CoA) is a relatively rare congenital narrowing of the distal aortic arch to proximal descending thoracic aorta, usually diagnosed in infancy or early childhood with upper extremity hypertension as a typical finding. Late detection in asymptomatic, physically active older adults is rare and can be clinically significant.
Case presentation
We present the case of a 65-year-old lifelong endurance athlete found to have transient severe left ventricular (LV) systolic dysfunction after completing a marathon. Stress testing revealed a hypertensive response, prompting cardiac MRI, which detected a new severe juxta-ductal CoA with collateralization. The patient underwent successful endovascular stenting with improved blood pressure response and stabilization of LV function.
Conclusion
This case underscores the importance of thorough cardiovascular assessment in older athletes and highlights the impact of lifelong exercise in compensating for congenital defects like CoA.
{"title":"Late diagnosis of severe aortic coarctation in a 65-year-old endurance athlete: Insights into exercise physiology and collateral compensation","authors":"Arundhati Sharma , Anubhuti Sharma , Kalyan Chaliki , Marco Antonio Fernandes , Francois Marcotte , Satyajit Reddy","doi":"10.1016/j.ijcchd.2025.100606","DOIUrl":"10.1016/j.ijcchd.2025.100606","url":null,"abstract":"<div><h3>Background</h3><div>Coarctation of the aorta (CoA) is a relatively rare congenital narrowing of the distal aortic arch to proximal descending thoracic aorta, usually diagnosed in infancy or early childhood with upper extremity hypertension as a typical finding. Late detection in asymptomatic, physically active older adults is rare and can be clinically significant.</div></div><div><h3>Case presentation</h3><div>We present the case of a 65-year-old lifelong endurance athlete found to have transient severe left ventricular (LV) systolic dysfunction after completing a marathon. Stress testing revealed a hypertensive response, prompting cardiac MRI, which detected a new severe juxta-ductal CoA with collateralization. The patient underwent successful endovascular stenting with improved blood pressure response and stabilization of LV function.</div></div><div><h3>Conclusion</h3><div>This case underscores the importance of thorough cardiovascular assessment in older athletes and highlights the impact of lifelong exercise in compensating for congenital defects like CoA.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100606"},"PeriodicalIF":0.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631708","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}
Pub Date : 2025-06-28DOI: 10.1016/j.ijcchd.2025.100605
Providence Akingeneye , David J. Bradley , Joseph Mucumbitsi , Naphtal Nyilimanzi , Yves Mutabandama , Emmanuel K. Rusingiza
Background
Congenital heart disease surgery and catheterization for Rwandan children has historically been accomplished with the assistance of visiting external teams and referrals abroad. However, follow-up information on patient-specific clinical outcomes is lacking.
Methods
This is a retrospective cross-sectional study of clinical outcomes among Rwandan children under 15 years of age at the time of heart surgery operated between 2006 and 2020. Data from two Rwandan teaching hospitals were collected from paper and digital patient charts.
Results
Among the 406 children included in our study, 59.1 % were female; 30.5 % and 28.1 % of the children were underweight and stunted, respectively. Tetralogy of Fallot was the most common cyanotic CHD. Of procedures, VSD repair was the most performed operation and PDA closure represented the majority of therapeutic catheterizations. Postoperatively complications such as pneumonia occurred in 6 %, residual shunt in 17 % and residual mild or greater stenosis in 41 %. The overall mortality was 3.7 %, with sepsis as the leading cause of death. Intensive care unit stay longer than seven days, symptomatic heart failure before surgery, and being underweight were significantly associated with complications. Cardiopulmonary bypass over 60 min was associated with mortality among operated children.
Conclusion
Procedural treatment of CHD in Rwandan children was predominantly accomplished by visiting missions during the study period. Of patients with sufficient clinical follow up data, survival was 96 % and complications occurred in 35 %. Nutritional compromise is a correlate of poor outcome. A centralized cardiac registry might mitigate the observed loss of clinical follow up data in 14 % of children.
{"title":"Clinical outcomes of children operated for congenital heart diseases in Rwanda: a 14-year retrospective analysis","authors":"Providence Akingeneye , David J. Bradley , Joseph Mucumbitsi , Naphtal Nyilimanzi , Yves Mutabandama , Emmanuel K. Rusingiza","doi":"10.1016/j.ijcchd.2025.100605","DOIUrl":"10.1016/j.ijcchd.2025.100605","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease surgery and catheterization for Rwandan children has historically been accomplished with the assistance of visiting external teams and referrals abroad. However, follow-up information on patient-specific clinical outcomes is lacking.</div></div><div><h3>Methods</h3><div>This is a retrospective cross-sectional study of clinical outcomes among Rwandan children under 15 years of age at the time of heart surgery operated between 2006 and 2020. Data from two Rwandan teaching hospitals were collected from paper and digital patient charts.</div></div><div><h3>Results</h3><div>Among the 406 children included in our study, 59.1 % were female; 30.5 % and 28.1 % of the children were underweight and stunted, respectively. Tetralogy of Fallot was the most common cyanotic CHD. Of procedures, VSD repair was the most performed operation and PDA closure represented the majority of therapeutic catheterizations. Postoperatively complications such as pneumonia occurred in 6 %, residual shunt in 17 % and residual mild or greater stenosis in 41 %. The overall mortality was 3.7 %, with sepsis as the leading cause of death. Intensive care unit stay longer than seven days, symptomatic heart failure before surgery, and being underweight were significantly associated with complications. Cardiopulmonary bypass over 60 min was associated with mortality among operated children.</div></div><div><h3>Conclusion</h3><div>Procedural treatment of CHD in Rwandan children was predominantly accomplished by visiting missions during the study period. Of patients with sufficient clinical follow up data, survival was 96 % and complications occurred in 35 %. Nutritional compromise is a correlate of poor outcome. A centralized cardiac registry might mitigate the observed loss of clinical follow up data in 14 % of children.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100605"},"PeriodicalIF":0.8,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144656881","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}
Pub Date : 2025-06-26DOI: 10.1016/j.ijcchd.2025.100604
Aso F. Salih , Trifa M. Qadir , Nagib Dahdah
This case report describes a 1.3-year-old infant with an atypical presentation of Kawasaki Disease (KD) following exposure to COVID-19 within the family and positive SARS-CoV-2 IgG antibodies. The clinical course was marked by significant left ventricular (LV) dysfunction, progressive coronary artery involvement, and complicated by severe dermatologic manifestations including Steven-Johnson-like lesions and psoriatic-like eruptions. Despite initial improvement in cardiac function, the infant developed multiple coronary aneurysms and succumbed to sudden cardiac death. This case highlights the diagnostic and therapeutic challenges in managing atypical KD in the context of COVID-19. We describe the case and review the literature related to the associated severe skin lesions.
{"title":"Kawasaki disease with persistent severe psoriatic-like skin manifestations and sudden cardiac death","authors":"Aso F. Salih , Trifa M. Qadir , Nagib Dahdah","doi":"10.1016/j.ijcchd.2025.100604","DOIUrl":"10.1016/j.ijcchd.2025.100604","url":null,"abstract":"<div><div>This case report describes a 1.3-year-old infant with an atypical presentation of Kawasaki Disease (KD) following exposure to COVID-19 within the family and positive SARS-CoV-2 IgG antibodies. The clinical course was marked by significant left ventricular (LV) dysfunction, progressive coronary artery involvement, and complicated by severe dermatologic manifestations including Steven-Johnson-like lesions and psoriatic-like eruptions. Despite initial improvement in cardiac function, the infant developed multiple coronary aneurysms and succumbed to sudden cardiac death. This case highlights the diagnostic and therapeutic challenges in managing atypical KD in the context of COVID-19. We describe the case and review the literature related to the associated severe skin lesions.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100604"},"PeriodicalIF":0.8,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144522286","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}
Pub Date : 2025-06-21DOI: 10.1016/j.ijcchd.2025.100603
Ralph M.L. Neijenhuis , Madelien V. Regeer , Niki L. Walker , Amanda Hunter , Philippine Kiès , Eduard R. Holman , J. Wouter Jukema , Monique R.M. Jongbloed , Gruschen R. Veldtman , Anastasia D. Egorova
Background
Single ventricle patients are at high risk of developing circulatory failure. There is limited evidence for pharmacological treatment. This study assessed the echocardiographic changes in ventricular function during sodium-glucose cotransporter 2 inhibitor (SGLT2i) therapy in patients with single ventricle failure (SVF).
Methods
SVF patients with a baseline transthoracic echocardiogram within six months before starting SGLT2i and at least one echocardiographic examination within twelve months follow-up were included from a real-world international registry of adult congenital heart disease patients on SGLT2i. Mixed models were used to evaluate longitudinal changes in ventricular function and differences between patients with SVF with ≥ moderately reduced systolic function (SVFrEF) and with ≤ mildly reduced function (SVFpEF).
Results
Thirteen patients were included. The median age was 21 [20–42] years, 8 (61.5 %) were female, 10 (76.9 %) had a Fontan circulation, 8 (61.5 %) had SVFrEF, and 5 (38.5 %) SVFpEF at the start of SGLT2i. The mean follow-up was 7.6 ± 3.3 months. End-systolic area decreased significantly in all patients (−1.6 cm2 per month, p = 0.007) in the first 100 days. Fractional area change improved in the first 100 days in SVFrEF patients (3.5 %-point per month, p < 0.001), while SVFpEF patients remained stable. There was a significant improvement in the free wall strain in all patients (−0.3 %-point per month, p = 0.036) but not in global longitudinal strain (p = 0.087). Isovolumic acceleration also improved in the first 100 days (0.5 m/s2 per month, p = 0.010).
Conclusions
Echocardiographic signals of improved ventricular function were observed in the first year of SGLT2i therapy in patients with SVF.
{"title":"Echocardiographic effects of sodium-glucose cotransporter 2 inhibitors in single ventricle circulatory failure","authors":"Ralph M.L. Neijenhuis , Madelien V. Regeer , Niki L. Walker , Amanda Hunter , Philippine Kiès , Eduard R. Holman , J. Wouter Jukema , Monique R.M. Jongbloed , Gruschen R. Veldtman , Anastasia D. Egorova","doi":"10.1016/j.ijcchd.2025.100603","DOIUrl":"10.1016/j.ijcchd.2025.100603","url":null,"abstract":"<div><h3>Background</h3><div>Single ventricle patients are at high risk of developing circulatory failure. There is limited evidence for pharmacological treatment. This study assessed the echocardiographic changes in ventricular function during sodium-glucose cotransporter 2 inhibitor (SGLT2i) therapy in patients with single ventricle failure (SVF).</div></div><div><h3>Methods</h3><div>SVF patients with a baseline transthoracic echocardiogram within six months before starting SGLT2i and at least one echocardiographic examination within twelve months follow-up were included from a real-world international registry of adult congenital heart disease patients on SGLT2i. Mixed models were used to evaluate longitudinal changes in ventricular function and differences between patients with SVF with ≥ moderately reduced systolic function (SVFrEF) and with ≤ mildly reduced function (SVFpEF).</div></div><div><h3>Results</h3><div>Thirteen patients were included. The median age was 21 [20–42] years, 8 (61.5 %) were female, 10 (76.9 %) had a Fontan circulation, 8 (61.5 %) had SVFrEF, and 5 (38.5 %) SVFpEF at the start of SGLT2i. The mean follow-up was 7.6 ± 3.3 months. End-systolic area decreased significantly in all patients (−1.6 cm<sup>2</sup> per month, p = 0.007) in the first 100 days. Fractional area change improved in the first 100 days in SVFrEF patients (3.5 %-point per month, p < 0.001), while SVFpEF patients remained stable. There was a significant improvement in the free wall strain in all patients (−0.3 %-point per month, p = 0.036) but not in global longitudinal strain (p = 0.087). Isovolumic acceleration also improved in the first 100 days (0.5 m/s<sup>2</sup> per month, p = 0.010).</div></div><div><h3>Conclusions</h3><div>Echocardiographic signals of improved ventricular function were observed in the first year of SGLT2i therapy in patients with SVF.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100603"},"PeriodicalIF":0.8,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144604628","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}
Pub Date : 2025-06-21DOI: 10.1016/j.ijcchd.2025.100602
Allahdad Khan , Tehreem Asghar , Kumail Mustafa Ali , Muhammad Saeed , Saniya Ishtiaq , Fatima Ashfaq , Jamil Nasrallah , Laiba Yumn , Usman Ali Akbar , Peter Collins , Raheel Ahmed
Background
Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.
Method
We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression.
Result
From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.
Conclusion
This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.
{"title":"Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis","authors":"Allahdad Khan , Tehreem Asghar , Kumail Mustafa Ali , Muhammad Saeed , Saniya Ishtiaq , Fatima Ashfaq , Jamil Nasrallah , Laiba Yumn , Usman Ali Akbar , Peter Collins , Raheel Ahmed","doi":"10.1016/j.ijcchd.2025.100602","DOIUrl":"10.1016/j.ijcchd.2025.100602","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.</div></div><div><h3>Method</h3><div>We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression<strong>.</strong></div></div><div><h3>Result</h3><div>From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.</div></div><div><h3>Conclusion</h3><div>This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100602"},"PeriodicalIF":0.8,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144605150","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}
Pub Date : 2025-06-16DOI: 10.1016/j.ijcchd.2025.100601
Linda Ternrud , Bengt Johansson , David Sparv , Zacharias Mandalenakis , Christina Christersson , Liesbet Van Bulck , Philip Moons , Camilla Sandberg , Joanna Hlebowicz
Background
Fatigue is a symptom that has been described among adult patients with congenital heart disease (CHD), but the prevalence and impact of fatigue on the patient's daily life has been poorly studied. This study (i) examines the prevalence of fatigue in patients aged over 40 years with moderately complex or complex congenital heart disease compared to controls and (ii) explores the relationship between fatigue, heart disease complexity, clinical characteristics and self-reported New York Heart Association Functional Class (self-reported NYHA class).
Methods
The Multidimensional Fatigue Inventory (MFI-20) was applied in 166 patients with moderately complex CHD or complex CHD (44 % females, median age 55.3 years, IQR 47.6–64.8) along with 89 controls (43 % female, median age 54.0, IQR 46.0–65.9). MFI-20 measured general fatigue, physical fatigue, mental fatigue, reduced motivation and reduced activity.
Results
Physical fatigue (severe to very severe) was more common in complex CHD compared with moderately complex CHD (25 % vs. 52 %, p = 0.006). Complex CHD was associated with severe to very severe physical fatigue (odds ratio 3.1 (95 % CI 1.1–9.1). Patients with complex CHD had higher levels of self-reported NYHA class than patients with moderately complex CHD. All dimensions of fatigue were positively associated with self-reported NYHA class.
Conclusions
Patients over the age of 40 with complex CHD were three times more likely to report severe to very severe physical fatigue and reported higher levels of self-reported NYHA class than patients with moderately complex CHD. This highlights the importance of considering CHD complexity in clinical practice.
疲劳是先天性心脏病（CHD）成年患者的一种症状，但疲劳的患病率和对患者日常生活的影响研究甚少。本研究(i)与对照组相比，研究了40岁以上患有中度复杂或复杂先天性心脏病的患者的疲劳患病率；（ii）探讨了疲劳、心脏病复杂性、临床特征和自我报告的纽约心脏协会功能等级（NYHA等级）之间的关系。方法采用多维疲劳量表（MFI-20）对166例中、复杂冠心病患者（女性占44%，中位年龄55.3岁，IQR 47.6 ~ 64.8）和89例对照组（女性占43%，中位年龄54.0岁，IQR 46.0 ~ 65.9）进行分析。MFI-20测量一般疲劳、身体疲劳、精神疲劳、动力减少和活动减少。结果与中度复杂冠心病相比，重度至极重度躯体疲劳在复杂冠心病中更为常见（25% vs. 52%, p = 0.006）。复杂冠心病与严重到非常严重的身体疲劳相关（优势比3.1 (95% CI 1.1-9.1)）。复杂冠心病患者自我报告的NYHA等级水平高于中度复杂冠心病患者。疲劳的所有维度都与自我报告的NYHA等级呈正相关。结论：40岁以上的复杂冠心病患者报告重度至极重度身体疲劳的可能性是中度复杂冠心病患者的3倍，自述NYHA等级水平高于中度复杂冠心病患者。这突出了在临床实践中考虑冠心病复杂性的重要性。
{"title":"Fatigue in adults with congenital heart disease aged over 40 years","authors":"Linda Ternrud , Bengt Johansson , David Sparv , Zacharias Mandalenakis , Christina Christersson , Liesbet Van Bulck , Philip Moons , Camilla Sandberg , Joanna Hlebowicz","doi":"10.1016/j.ijcchd.2025.100601","DOIUrl":"10.1016/j.ijcchd.2025.100601","url":null,"abstract":"<div><h3>Background</h3><div>Fatigue is a symptom that has been described among adult patients with congenital heart disease (CHD)<strong>,</strong> but the prevalence and impact of fatigue on the patient's daily life has been poorly studied. This study (i) examines the prevalence of fatigue in patients aged over 40 years with moderately complex or complex congenital heart disease compared to controls and (ii) explores the relationship between fatigue, heart disease complexity, clinical characteristics and self-reported New York Heart Association Functional Class (self-reported NYHA class).</div></div><div><h3>Methods</h3><div>The Multidimensional Fatigue Inventory (MFI-20) was applied in 166 patients with moderately complex CHD or complex CHD (44 % females, median age 55.3 years, IQR 47.6–64.8) along with 89 controls (43 % female, median age 54.0, IQR 46.0–65.9). MFI-20 measured general fatigue, physical fatigue, mental fatigue, reduced motivation and reduced activity.</div></div><div><h3>Results</h3><div>Physical fatigue (severe to very severe) was more common in complex CHD compared with moderately complex CHD (25 % <em>vs.</em> 52 %, <em>p</em> = 0.006). Complex CHD was associated with severe to very severe physical fatigue (odds ratio 3.1 (95 % CI 1.1–9.1). Patients with complex CHD had higher levels of self-reported NYHA class than patients with moderately complex CHD. All dimensions of fatigue were positively associated with self-reported NYHA class.</div></div><div><h3>Conclusions</h3><div>Patients over the age of 40 with complex CHD were three times more likely to report severe to very severe physical fatigue and reported higher levels of self-reported NYHA class than patients with moderately complex CHD. This highlights the importance of considering CHD complexity in clinical practice.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100601"},"PeriodicalIF":0.8,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144605151","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}
Pub Date : 2025-06-16DOI: 10.1016/j.ijcchd.2025.100600
Naomi Nakagawa, Yusuke Shigemitsu, Kengo Okamoto
A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.
{"title":"GORE® CARDIOFORM ASD occluder thrombus in a patient of rheumatoid arthritis occurred three days after deployment","authors":"Naomi Nakagawa, Yusuke Shigemitsu, Kengo Okamoto","doi":"10.1016/j.ijcchd.2025.100600","DOIUrl":"10.1016/j.ijcchd.2025.100600","url":null,"abstract":"<div><div>A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100600"},"PeriodicalIF":0.8,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631709","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}
Pub Date : 2025-06-03DOI: 10.1016/j.ijcchd.2025.100598
Andrea R. Hsu , Snigdha Karnakoti , Ahmed T. Abdelhalim , Jason H. Anderson , C. Charles Jain , William R. Miranda , Elizabeth H. Stephens , Joseph A. Dearani , Hartzell V. Schaff , Heidi M. Connolly , Alexander C. Egbe
Background
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.
Method
BVD was defined as Doppler mean gradient >40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient >10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.
Results
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 < 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).
Conclusions
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.
成人先天性心脏病（CHD）患者经常植入生物瓣膜，但缺乏关于所有瓣膜位置的生物瓣膜寿命和预后的综合数据。本研究的目的是确定成人冠心病患者所有瓣膜位置生物瓣膜功能障碍（BVD）的发生率和危险因素。方法将dbvd定义为主动脉或肺生物假体的多普勒平均梯度为40 mmHg和/或严重假体/假体周围反流，二尖瓣或三尖瓣生物假体的多普勒平均梯度为10 mmHg和/或严重假体/假体周围反流。结果1221例成人冠心病患者行生物瓣膜植入术（年龄39±16岁，男性617例[51%]）。在瓣膜植入时，分别有755例（62%）、325例（27%）、176例（14%）和21例（1.7%）在肺动脉、三尖瓣、主动脉和二尖瓣位置植入了生物瓣膜。肺动脉瓣、三尖瓣、主动脉瓣和二尖瓣生物瓣膜10年累计BVD发生率分别为28%、36%、43%和68% (p <；0.001)。以肺动脉瓣位置为参照组，瓣膜位置（三尖瓣[HR 1.26, 95% CI 1.09-1.58]、主动脉瓣[HR 1.64, 95% CI 1.22-1.97]、二尖瓣[HR 2.01, 95% CI 0.83-4.05]）与BVD的高风险相关。年龄越小，BVD风险越高（每5年增加危险度0.95,95% CI 0.86-0.98）。结论这些数据支持了现有的证据，即左侧生物假体瓣膜在年轻患者中表现出耐久性降低，应纳入咨询和临床决策。
{"title":"Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease","authors":"Andrea R. Hsu , Snigdha Karnakoti , Ahmed T. Abdelhalim , Jason H. Anderson , C. Charles Jain , William R. Miranda , Elizabeth H. Stephens , Joseph A. Dearani , Hartzell V. Schaff , Heidi M. Connolly , 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 >40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient >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 < 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}
Pub Date : 2025-06-03DOI: 10.1016/j.ijcchd.2025.100599
François Côté , Leonaes Brahel Tatchinda Kuete , Réda Ibrahim , Jean-Claude Tardif , Colombe Roy , François-Pierre Mongeon , Annie Dore , Blandine Mondésert , Paul Khairy , Marie-A. Chaix
Background
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.
Methods
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.
Results
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.
Conclusion
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.
{"title":"Utilizing optical coherence tomography to explore pulmonary arteries and pulmonary vascular disease in Fontan patients","authors":"François Côté , Leonaes Brahel Tatchinda Kuete , Réda Ibrahim , Jean-Claude Tardif , Colombe Roy , François-Pierre Mongeon , Annie Dore , Blandine Mondésert , Paul Khairy , 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}
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