International journal of cardiology. Congenital heart disease最新文献

{"title":"Hepatic cholate clearance as assessed by HepQuant-SHUNT is associated with clinical outcomes in individuals with Fontan circulation","authors":"Yuli Y. Kim ,&nbsp;Daniel Ganger ,&nbsp;Alexis Z. Tomlinson ,&nbsp;Isabella Z. Farkas ,&nbsp;Jack Rychik ,&nbsp;Joanne Imperial ,&nbsp;Michael P. McRae ,&nbsp;Greg T. Everson ,&nbsp;Maarouf A. Hoteit","doi":"10.1016/j.ijcchd.2026.100654","DOIUrl":"10.1016/j.ijcchd.2026.100654","url":null,"abstract":"<div><h3>Background</h3><div>Fontan-associated liver disease [FALD] is universal in individuals with Fontan circulation [FC]. The dual cholate clearance test is a noninvasive, flow-dependent measure of liver function. We aim to explore the association between cholate clearance and clinical outcomes in this population.</div></div><div><h3>Methods</h3><div>Prospective study of adults with FC ≥ 18 years from University of Pennsylvania [Penn] and Northwestern University [NU]. Hepatic clearance of oral d4-cholate and intravenous 13C-cholate were measured from peripheral venous blood and calculated as portal hepatic filtration rate [HFR], systemic HFR, and SHUNT% with elevated SHUNT% being &gt;30 %. Primary outcome was transplant-free survival. Association between SHUNT% and primary outcome was assessed using Kaplan-Meier curves and log-rank test or Cox regression. Secondary outcome was composite of death, transplant, new onset heart failure, ascites, protein losing enteropathy, or hepatocellular carcinoma. Association between SHUNT% and composite outcome was analyzed by logistic regression.</div></div><div><h3>Results</h3><div>Fifty participants (35 Penn, 15 NU) were enrolled. Unadjusted 1-, 3-, and 5-year transplant-free survival was 96 %, 94 %, and 79 %, respectively. Individuals with elevated SHUNT% had significantly lower transplant-free survival at 5-years. The composite outcome was reached in 14/50 (29 %), including death (n = 4), combined heart-liver transplant (n = 4), or both (n = 2). Elevated SHUNT% was associated with 3.9-fold increase odds of composite outcome (95 % CI 1.02–15.3; p = 0.048).</div></div><div><h3>Conclusions</h3><div>An increase in shunting of portal blood into the systemic circulation as quantified by SHUNT% is associated with decreased transplant-free survival in individuals with FC and has excellent discrimination in identifying those at risk for mid-term adverse clinical events.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100654"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146188085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcome of ventricular assist devices in adults with congenital heart disease – A single center case series","authors":"Ahmed Younis ,&nbsp;Zeyad M. Kholeif ,&nbsp;William R. Miranda ,&nbsp;Heidi M. Connolly ,&nbsp;Mauricio T. Villavicencio ,&nbsp;Joseph A. Dearani ,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100648","DOIUrl":"10.1016/j.ijcchd.2025.100648","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100648"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advancing the science and care of pulmonary hypertension, globally","authors":"George Giannakoulas ,&nbsp;Ioana R. Preston ,&nbsp;Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2025.100651","DOIUrl":"10.1016/j.ijcchd.2025.100651","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100651"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147421119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progression of stenosis severity and aortopathy in adult patients with congenital aortic stenosis","authors":"Zoë A. Keuning ,&nbsp;Frederike Meccanici ,&nbsp;Kevin M. Veen ,&nbsp;Bibi Schreurs ,&nbsp;Roland R.J. van Kimmenade ,&nbsp;Joost P. van Melle ,&nbsp;Monique R.M. Jongbloed ,&nbsp;Michiel Voskuil ,&nbsp;Berto J. Bouma ,&nbsp;Famke Sneep ,&nbsp;Jeroen F.A. Simons ,&nbsp;Alexander Hirsch ,&nbsp;Jolien W. Roos-Hesselink ,&nbsp;Annemien E. van den Bosch","doi":"10.1016/j.ijcchd.2025.100646","DOIUrl":"10.1016/j.ijcchd.2025.100646","url":null,"abstract":"<div><h3>Background</h3><div>Congenital aortic stenosis (AS) is a heterogeneous disease. However, repeated data describing disease progression is limited, especially in adults. Therefore, the objective of this study is to investigate progression of peak velocity and aortic dimensions in adult congenital AS patients, identifying markers for fast progression.</div></div><div><h3>Methods</h3><div>Adult patients (aged 18–55 years) with a native aortic valve and at least mild AS at baseline registered in the Dutch CONCOR registry between 2001 and 2019 from all six tertiary expert centers for congenital heart disease were included. Patients with severe aortic regurgitation at baseline or no available echocardiograms during follow-up were excluded. Data on ascending aortic dimensions and peak velocity changes over time were analyzed using mixed models until death, aortic valve replacement or March 1, 2023.</div></div><div><h3>Results</h3><div>402 patients (63 % male) were included with a median age of 26 [IQR 19–41] years and peak velocity of 3.1 [IQR 2.7–3.6] m/s. Median follow-up time was 6.8 [IQR 3.8–11.6] years. Peak velocity significantly progressed over time with 0.06 ± 0.10 m/s/year (p &lt; 0.001), independent of baseline velocity. Older age and concentric left ventricular (LV) remodeling were associated with faster progression. Mean progression of ascending aortic dimension was 0.4 ± 0.5 mm/year (p &lt; 0.001), with faster progression in younger patients (p = 0.002). No correlation between aortic growth rate and stenosis severity progression was seen (r = 0.001, p = 0.98).</div></div><div><h3>Conclusion</h3><div>Overall disease progression was slow in adult congenital AS patients, and independent of baseline peak velocity. Progression of stenosis should be monitored more carefully in older patients and patients with signs of concentric LV remodeling.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100646"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Strength and resistance training in adult congenital heart disease: A guide to exercise prescription in the clinic","authors":"Calum Dolan ,&nbsp;Gemma Brown ,&nbsp;Elaine Muirhead ,&nbsp;Lorna Swan","doi":"10.1016/j.ijcchd.2025.100650","DOIUrl":"10.1016/j.ijcchd.2025.100650","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100650"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145939397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical worsening in adult congenital heart disease and heart failure: A single-centre, observational study","authors":"Thibault Bourgeois ,&nbsp;Juliette Hubert ,&nbsp;Pieter De Meester ,&nbsp;Thibault Petit ,&nbsp;Els Troost ,&nbsp;Lucas Van Aelst ,&nbsp;Filip Rega ,&nbsp;Philip Moons ,&nbsp;Werner Budts ,&nbsp;Alexander Van De Bruaene","doi":"10.1016/j.ijcchd.2026.100656","DOIUrl":"10.1016/j.ijcchd.2026.100656","url":null,"abstract":"<div><h3>Background</h3><div>Data on contemporary treatment approaches and reliable markers of clinical worsening in adult patients with congenital heart disease and heart failure (ACHD-HF) are scarce. This study aimed at evaluating (1) medical therapy within a contemporary ACHD-HF cohort and (2) the incidence of various (composite) endpoints across anatomical and pathophysiological subgroups.</div></div><div><h3>Methods</h3><div>Retrospective study including ACHD-HF patients (≥16 years) under active follow-up at a tertiary care center, monitored until last visit, death, ventricular assist device (VAD) implantation, or heart transplant (HTX). Medical therapy was documented at time of inclusion and final follow-up. Clinical endpoints were evaluated, after excluding patients with early events (&lt;1 week of inclusion) or without follow-up. Endpoints included HF hospitalization, increase of diuretic treatment, NYHA class progression, all-cause mortality, HTX and VAD placement.</div></div><div><h3>Results</h3><div>Of the 256 ACHD-HF patients (mean age 50 ± 17 years; 52 % male), 56 were excluded for the final analysis. Two hundred patients were followed for a median of 30 (IQR 22–36) months. Changes in medical therapy included: increased use of mineralocorticoid receptor antagonists (p = 0.038), sodium-glucose transport protein 2 inhibitors (p = 0.002) and a trend towards increased use of angiotensin receptor-neprilysin inhibitors (p = 0.070). Furthermore, whereas HF hospitalization (p &lt; 0.001) and progression of NYHA (p = 0.016) were associated with death, whereas HTX or VAD implant and escalation of diuretic therapy (p = 0.961) were not.</div></div><div><h3>Conclusions</h3><div>This study characterizes current ACHD-HF therapy and identifies NYHA progression and HF hospitalization as potential markers of clinical worsening as they relate to death, HTX, and VAD implantation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100656"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146188088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burden of diffuse myocardial fibrosis assessed by cardiac magnetic resonance in repaired tetralogy of Fallot: A systematic review and meta-analysis","authors":"José Carlos P. Secco ,&nbsp;Eduardo Schaustz ,&nbsp;Adriana M. Innocenzi ,&nbsp;Fernanda.P. Fernandes ,&nbsp;Emiliano Medei ,&nbsp;Andréa Silvestre-Sousa ,&nbsp;Gabriel C. Camargo ,&nbsp;Ronir R. Luiz ,&nbsp;Jessica P.L. Moreira ,&nbsp;Carlos A.S. Magliano ,&nbsp;Renata Moll-Bernardes ,&nbsp;Mariana Póvoa-Corrêa","doi":"10.1016/j.ijcchd.2025.100631","DOIUrl":"10.1016/j.ijcchd.2025.100631","url":null,"abstract":"<div><h3>Background</h3><div>The assessment of myocardial fibrosis by cardiac magnetic resonance (CMR) in patients with repaired tetralogy of Fallot (rTOF) is not routinely performed, primarily due to technical challenges in measuring right ventricular native T1 and extracellular volume (ECV). Additionally, although native T1 can be assessed without contrast, offering broader applicability, its role in evaluating myocardial fibrosis in rTOF patients remains unclear.</div></div><div><h3>Objectives</h3><div>The aim of this systematic review and meta-analysis was to evaluate interstitial fibrosis in both the right and left ventricles using native T1 and ECV quantification in patients with rTOF.</div></div><div><h3>Methods</h3><div>This systematic review was conducted according to the 2020 PRISMA guidelines and was registered in PROSPERO. The PubMed, Scopus, and Embase databases were searched to identify studies in which patients with rTOF underwent CMR with native T1 and ECV assessment. Random-effect models were used to estimate pooled effect sizes.</div></div><div><h3>Results</h3><div>The sample comprised 14 studies that included 893 patients with rTOF and 284 controls. LV native T1 values did not differ significantly between groups, except in the studies with a 3.0 T scanner (<em>d</em> = 3.52; <em>p</em> &lt; 0.01). RV values were significantly elevated in patients with rTOF (<em>d</em> = 1.36, <em>p</em> &lt; 0.01). The left ventricle and right ventricle ECVs were significantly higher in these patients than in controls (<em>d</em> = 0.95 and 1.11, respectively; both <em>p</em> &lt; 0.01).</div></div><div><h3>Conclusion</h3><div>Despite successful surgical repair, patients with rTOF frequently exhibit evidence of myocardial fibrosis, a marker of adverse remodeling that may progress long after anatomical correction.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100631"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578753","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital heart disease in men of reproductive age: Nationwide cohort studies of prevalence, male fertility, and birth outcomes in offspring","authors":"Marit Sandberg ,&nbsp;Nina Øyen ,&nbsp;Tatiana Fomina ,&nbsp;Ferenc Macsali ,&nbsp;Gottfried Greve ,&nbsp;Elisabeth Leirgul","doi":"10.1016/j.ijcchd.2025.100637","DOIUrl":"10.1016/j.ijcchd.2025.100637","url":null,"abstract":"<div><h3>Background</h3><div>The potential for men with congenital heart disease (CHD) to father children and the impact of paternal CHD on offspring birth outcomes are not well understood.</div></div><div><h3>Methods</h3><div>Using Norwegian nationwide registries from 1994 to 2014, we identified two study populations: 1 829 877 men aged 18–50 years and 1 207 410 newborns. First, we reported the time trends of CHD in men and paternal CHD in newborns. Second, we compared the rate of becoming fathers in men with and without CHD. Third, we compared birth outcomes in offspring with and without paternal CHD.</div></div><div><h3>Results</h3><div>Between 1994 and 2014, the prevalence of CHD in men of reproductive age increased from 22.7 to 32.2 per 10 000, while the prevalence of paternal CHD in newborns increased from 21.6 to 26.9 per 10 000. Men with mild CHD had a similar rate of becoming fathers compared to men without CHD (rate ratio 0.97, 95 % confidence interval 0.90–1.05), but men with moderate/severe CHD had a lower rate (rate ratio 0.78, 95 % confidence interval 0.70–87). Newborns with paternal CHD had no increased risk of preterm birth or being small for gestational age, compared to newborns without paternal CHD.</div></div><div><h3>Conclusion</h3><div>The prevalence of CHD in men and paternal CHD in newborns was increasing. Moderate/severe CHD in men was associated with a lower rate of becoming fathers compared to the general male population. Paternal CHD was not associated with an increased risk of newborns being preterm or small for gestational age.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100637"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of a mental health screening program for adults with congenital heart disease","authors":"Alice Zhang ,&nbsp;Matthew W. Buelow ,&nbsp;Cheryl L. Brosig ,&nbsp;Amber Craig ,&nbsp;Lawrence A. Miller ,&nbsp;Jane M. Sowinski ,&nbsp;Patricia M. Stoll ,&nbsp;Kristen Andersen ,&nbsp;Scott B. Cohen","doi":"10.1016/j.ijcchd.2025.100623","DOIUrl":"10.1016/j.ijcchd.2025.100623","url":null,"abstract":"<div><h3>Background</h3><div>Adult congenital heart disease (ACHD) patients face a significant burden of mental health challenges related to their primary cardiac disease.</div></div><div><h3>Objectives</h3><div>This study characterizes the outcomes of mental health follow up, the prevalence and associated risk factors for mental health concerns in our single center ACHD population.</div></div><div><h3>Methods</h3><div>The Wisconsin Adult Congenital Heart Disease (WAtCH) program began an outpatient mental health screening program in May 2021 using the Patient Health Questionnaire-4 (PHQ-4). Through a retrospective chart review of ACHD patients seen from May 3, 2021, to May 5, 2022, we report on outcomes of a mental health referral process for ACHD patients with positive mental health screens. We also examined associations between mental health screens and patient demographic and clinical variables.</div></div><div><h3>Results</h3><div>Among 778 ACHD patient encounters (from 681 patients), there were 213 (27.4 %) positive mental health screens. Of 157 patient encounters with a positive screen and a recorded mental health disposition, 55 (35 %) reported no preexisting mental health provider. Patients at 21 of these encounters accepted a mental health referral, resulting in 3 completed appointments with an in-house mental health specialist. Younger age, female gender, minority race, great complexity congenital heart disease (CHD), were independently associated with a positive screen.</div></div><div><h3>Conclusions</h3><div>We found limited established mental health care and a low rate of mental health follow up in our ACHD population with a positive mental health screen. Several demographic and disease related factors are related to a greater risk of mental health concerns in ACHD patients.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100623"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in the congenital heart disease spectrum at China national children's medical center: a review of 10-year data","authors":"Jia Zheng,&nbsp;Zhiqiang Li,&nbsp;Song Bai,&nbsp;Jian Guo,&nbsp;Zhenjiang Song,&nbsp;Qilin Li,&nbsp;Nan Ding,&nbsp;Yaobin Zhu,&nbsp;Fan Fan,&nbsp;Zhangke Guo,&nbsp;Hanlu Yi","doi":"10.1016/j.ijcchd.2025.100633","DOIUrl":"10.1016/j.ijcchd.2025.100633","url":null,"abstract":"<div><h3>Background</h3><div>With the decline in the birth rate and simultaneous advancements in medical technology in China, research on how birth population changes have affected congenital heart disease (CHD) surgery in the last 10 years has increased.</div></div><div><h3>Method</h3><div>We investigated the medical data of all patients diagnosed with CHD treated at the surgical department at our hospital from January 2013 to December 2022, including their age and the type of surgery. We classified patients into two groups based on their age: less than 1 year (younger) and greater than 1 year (older). We defined patent ductus arteriosus, atrial septal defect, and ventricular septal defect as simple CHDs and others as complex diseases. Vascular ring diseases, including double aortic arch, pulmonary artery sling, and aberrant left subclavian artery with diverticulum, were considered special conditions.</div></div><div><h3>Result</h3><div>We found that 6440 patients underwent surgery at our hospital in the period mentioned. The number of surgeries conducted annually increased significantly from 609 in 2016 to 941 in 2019 but gradually declined since 2020. The proportion of simple CHD cases increased annually, whereas the proportion of complex CHD cases declined. The proportion of patients with vascular ring disease gradually increased. The proportion of older children increased, whereas that of younger children decreased each year. All changes were statistically significant.</div></div><div><h3>Conclusions</h3><div>Owing to advancements in medical technology, economic factors, change of birth rate and abortion rate, the number of surgeries for CHDs has decreased significantly. It is challenging to predict future trends. Nevertheless, this phenomenon is specific to China. Policymakers, hospitals, and surgeons must introduce appropriate adjustments in response to this change.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100633"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578758","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}