International journal of cardiology. Congenital heart disease最新文献

{"title":"Correlates of left ventricular hypertrophy in adults with coarctation of aorta and role in risk stratification","authors":"Zeyad Kholeif,&nbsp;Ahmed E. Ali,&nbsp;Karim Ali,&nbsp;Marwan Ahmed,&nbsp;Ahmed Bahnasy,&nbsp;Meena Bai,&nbsp;Sara Aboelmaaty,&nbsp;Mohamed Ellabbad,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100642","DOIUrl":"10.1016/j.ijcchd.2025.100642","url":null,"abstract":"<div><h3>Background</h3><div>Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and LV hypertrophy, which is associated with cardiovascular mortality. Therapies that decrease LV pressure overload result in regression of LV hypertrophy and improved survival. The goal of COA intervention is to relieve LV pressure overload, and the guidelines recommend the use of several COA severity indices as indications for intervention. These indices are Doppler mean gradient, systolic blood pressure (SBP), upper-to-lower-extremity SBP (ULE-SBP), aortic isthmus ratio, presence of collateral vessels, and exercise-induced hypertension (EIH). Although these indices are physiologically intuitive, it is unclear how many of these indices are required in order to recommend intervention, and the hierarchical importance of the different indices in patients that have some but not all the indices.</div></div><div><h3>Methods</h3><div>In this cross-sectional study, multivariate linear regression analysis was used to assess the correlation between LV mass index (LVMI) and indices of COA severity.</div></div><div><h3>Results</h3><div>Of the COA indices analyzed in 811 adult with repaired COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β coefficients ±standard error −31.6 ± 13.8, p &lt; 0.001), as compared to Doppler peak gradient (0.79 ± 0.22, p = 0.002) and SBP (0.53 ± 0.21, p = 0.03).</div></div><div><h3>Conclusion</h3><div>Aortic isthmus ratio had the strongest correlation with LVMI, which is a reflection of chronic LV pressure overload. An aortic isthmus ratio ≤0.7 was the optimal cut-off point to detect LV hypertrophy with good sensitivity and specificity. These results can potentially improve and simplify patient selection for COA intervention.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100642"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145748190","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":"Evaluating the effect of renin-angiotension-aldosterone system inhibitors and beta blockers on the progression of bicuspid aortic valve aortopathy in adulthood: A retrospective cohort study","authors":"Nili Schamroth Pravda ,&nbsp;Elchanan Samuel ,&nbsp;Tamir Bental ,&nbsp;Ofir Brem ,&nbsp;Ilan Richter ,&nbsp;Keren Skalsky ,&nbsp;Miri Schamroth Pravda ,&nbsp;Yaron Shapira ,&nbsp;Ran Kornowski ,&nbsp;Guy Witberg","doi":"10.1016/j.ijcchd.2026.100653","DOIUrl":"10.1016/j.ijcchd.2026.100653","url":null,"abstract":"<div><h3>Purpose</h3><div>Patients with a bicuspid aortic valve (BAV) often have an associated aortopathy and increased risk of aortic dissection. This study aimed to assess if the chronic use of renin-angiotensin-aldosterone system inhibitors (RAASi) or beta blockers (BB) may be associated with reducing the progression of aortic dilation in adult BAV patients.</div></div><div><h3>Methods</h3><div>A retrospective cohort study was performed including adult patients with BAV with serial echocardiography over ≥5 years. The cohort was subdivided according to chronic use of RAASi or BB. The primary outcome was a composite of progression of absolute ascending aortic diameter ≥4.5 cm or surgery for ascending aortic replacement.</div></div><div><h3>Results</h3><div>Included were 262 patients with mean age of 54.3 ± 19.5 years and 75.1 % male. The average follow-up time was 10.8 ± 0.8 years. The cumulative incidence of the primary endpoint was 14.6 % at 10-year follow up and 33.3 % at 15-year follow up. While those with RAASi (n = 39) had the primary endpoint numerically less that those without RAASi chronic therapy (12.8 % vs 24.8 %, p = 0.147), on multivariate analysis there was no significant decreased risk for the primary outcome. (HR = 0.89, 95 % CI [0.34–2.86], p = 0.97). Similarly, there was no significant decreased risk of the primary outcome amongst those with chronic beta blocker use following multivariate analysis (HR = 0.96, 95 % CI [0.37–2.51], p = 0.95).</div></div><div><h3>Conclusions</h3><div>Our findings suggest that the chronic use of RAASi or BB was not associated with a blunted progression of BAV aortopathy in the adult population. These results highlight the need for larger, randomised studies to validate these observations and further explore potential preventative strategies in this population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100653"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146188089","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":"Last of the Eisenmengers: A Tribute","authors":"Craig Broberg","doi":"10.1016/j.ijcchd.2025.100645","DOIUrl":"10.1016/j.ijcchd.2025.100645","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100645"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145799049","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":"Right ventricular outflow tract stenting for obstructive lesions in adult congenital heart disease","authors":"Miriam G. Zuniga Salcedo ,&nbsp;Edgar García Cruz ,&nbsp;José Antonio García Montes ,&nbsp;Jorge Sanchez Nieto ,&nbsp;Stephanie Angulo Cruzado ,&nbsp;Edgar Francisco Carrizales Sepúlveda ,&nbsp;Ramiro Flores Ramírez","doi":"10.1016/j.ijcchd.2026.100660","DOIUrl":"10.1016/j.ijcchd.2026.100660","url":null,"abstract":"<div><div>Currently, the use of stents in the right ventricular outflow tract (RVOT) has been described as alternative for palliation in neonates with Tetralogy of Fallot (ToF) and critical infundibular stenosis, who are not candidates for total correction in a single stage. However, there is limited evidence of the benefit of this intervention in other clinical scenarios, and just a few reports in adults.</div><div>A retrospective study was conducted on 23 patients with significant RVOT obstruction who underwent RVOT stent placement and were not considered candidates for surgery. Patients 17 years old at the time of the procedure at our institution were included.</div><div>Median age at the time of procedure was 24 years (IQR 17-49). 34.8% had univentricular heart physiology. The most frequent diagnosis was ToF (43.5%). 56.5% had at least one previous systemic-pulmonary shunt. In 69.5% of the cases the clinical context that led to stent placement was due to an emergency, primarily thrombosis of a systemic-pulmonary shunt (43.5%). RVOT stent implantation was successful in 100% of the cases. There were 2 procedure-related deaths (8.7%). The median stent patency time was 18.7 months (IQR 0-112 months).</div><div>This is the first study that demonstrates the feasibility of RVOT stenting as a palliative method in adult patients with congenital heart disease in emergency settings. In addition to showing the possibility of its use in univentricular hearts, it demonstrates the long-term durability of RVOT stents as a palliative method, something never previously reported.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100660"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147379900","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":"Two Italian fellows remember Richard Van Praagh","authors":"Bruno Marino,&nbsp;Angelo Restivo","doi":"10.1016/j.ijcchd.2025.100647","DOIUrl":"10.1016/j.ijcchd.2025.100647","url":null,"abstract":"<div><h3>Methods</h3><div>two italian fellows remember its figure of scientist, teacher and friend.</div></div><div><h3>Results</h3><div>in 1964 Dr Van Praagh anticipated the new diagnostic approach to congenital heart disease and in the following years he published more than 300 articles clarifying what was not clear in the past. He was a fascinating pearson and teacher.</div></div><div><h3>Conclusion</h3><div>Richard Van Praagh was an example of pioneer, scientist and man and his contribution changed the knowledge of congenital heart disease.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100647"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147421118","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The utility of 4D intracardiac echocardiography in transcatheter pulmonary valve replacement in adult congenital heart disease patients","authors":"Eihab Ghantous ,&nbsp;Hans Gao ,&nbsp;Carlos Sisniega ,&nbsp;Angela Li ,&nbsp;Jamil Aboulhosn","doi":"10.1016/j.ijcchd.2026.100652","DOIUrl":"10.1016/j.ijcchd.2026.100652","url":null,"abstract":"<div><h3>Background</h3><div>Intracardiac echocardiography (ICE) has become a critical imaging modality in adult congenital heart disease (ACHD) interventions, offering high-resolution intracardiac imaging without the need for intubation. In transcatheter pulmonary valve replacement (TCPVR), ICE may overcome limitations of traditional imaging, particularly in visualizing the right ventricular outflow tract (RVOT) and pulmonary valve.</div></div><div><h3>Objectives</h3><div>This study aimed to evaluate the feasibility, safety, and clinical utility of four-dimensional (4D) ICE in ACHD patients undergoing TCPVR.</div></div><div><h3>Methods</h3><div>Beginning in February 2023, we prospectively enrolled all patients undergoing TCPVR at a tertiary ACHD center. Patients underwent preprocedural imaging and clinical evaluation. 4D ICE was used pre- and post-valve implantation to assess cardiac anatomy and valve function. Patients without valve implantation or 4D ICE imaging were excluded.</div></div><div><h3>Results</h3><div>Of 55 referred patients, 45 underwent successful TCPVR with 4D ICE. The mean age was 42.6 ± 13.6 years, and 51 % were male. 4D ICE confirmed procedural indications, provided comprehensive anatomic and functional assessment, and detected post-implantation regurgitation in 35.6 % of cases, significantly more than angiography (11.1 %) or transthoracic echocardiography (4.4 %). Incidental but clinically relevant findings were identified in 33.3 % of patients, influencing management in 15.6 %. No ICE-related complications occurred.</div></div><div><h3>Conclusions</h3><div>4D ICE is a feasible, safe, and clinically valuable imaging tool during TCPVR in ACHD patients. It enhances procedural guidance, detects early valve-related complications, and identifies unexpected findings that affect management. Its integration into structural heart interventions may significantly improve outcomes, especially as technology continues to evolve.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100652"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978174","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":"Point of care INR monitoring in cyanotic congenital heart disease-related compensatory erythrocytosis","authors":"Phidias Rueter ,&nbsp;Freda Passam ,&nbsp;David Celermajer ,&nbsp;David Baker ,&nbsp;Cristina Ma Biason ,&nbsp;Danielle Osborn ,&nbsp;Rachael Cordina","doi":"10.1016/j.ijcchd.2026.100655","DOIUrl":"10.1016/j.ijcchd.2026.100655","url":null,"abstract":"<div><h3>Introduction</h3><div>People with cyanotic congenital heart disease (CHD) frequently require anticoagulation with warfarin. Point of care (POC) devices offer significant benefits such as patient-managed warfarin and rapid testing, however, there is limited experience with the use of POC in adult patients with CHD and high haematocrit.</div></div><div><h3>Methods</h3><div>This exploratory prospective cohort study recruited people between September 2021 and December 2023. Inclusion criteria were cyanotic CHD and oxygen saturations &lt;93% and a Hct ≥0.51. Exclusion criteria were pregnancy, antiplatelet agents, recent thromboembolic event or major medical illness. A Coaguchek XS (Roche Diagnostics) was used.</div></div><div><h3>Results</h3><div>Five individuals were recruited to the warfarin group (Hct 0.54, 0.52–0.58, IQR 0.04) and five to the control group (Hct 0.55, 0.51–0.60, IQR 0.07). Difference in INR between methods was 0.1 (−0.3-0.2, IQR 0.2) in the warfarin and −0.1 (−0.3 – 0.1, IQR 0.2) in the cyanosis-control arms. Bland Altman analysis demonstrated no proportional bias. No eligible participants had Hct ≥0.60. One potential participant had Hct of 0.73 but POC testing was unrecordable. Correlation between POC and laboratory INR was r = 0.98, p &lt; 0.01 (95 % CI 0.98–1.22) for the whole cohort, r = 1.0, p &lt; 0.01 (95 % CI 0.75–1.50) in the warfarin group and r = 0.87, p = 0.06 (95 %CI -0.23-3.10) in the control group.</div></div><div><h3>Conclusion</h3><div>POC INR testing may be an accurate alternative for individuals with compensatory erythrocytosis and Hct up to 0.60.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100655"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146188084","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":"Biventricular remodeling and function after Cone repair of Ebstein Anomaly: A review","authors":"Mimi X. Deng ,&nbsp;Alison J. Howell ,&nbsp;Osami Honjo ,&nbsp;Mark K. Friedberg","doi":"10.1016/j.ijcchd.2025.100649","DOIUrl":"10.1016/j.ijcchd.2025.100649","url":null,"abstract":"<div><div>The Cone reconstruction is the prevailing repair strategy for Ebstein anomaly (EA), providing near-anatomical restoration of the tricuspid valve (TV) and reliable reduction of tricuspid regurgitation (TR). Most of the risk of Cone repair is seen in the early postoperative period from dehiscence, with very low rates of mortality and reintervention thereafter. By correcting TV dysfunction and abnormal right ventricle (RV) geometry, ventricular reverse remodeling has been demonstrated through decreased right atrial and atrialized RV size, along with improved antegrade pulmonary flow and consequent left ventricular filling. Early postoperative RV dysfunction is common due to increased afterload upon addressing TR, with suggestion of delayed recovery. Cone repair augments left ventricular preload and preserves left ventricular function. The contrasting effects of ventricular plication and superior repositioning of TV during the Cone repair has led to discrepant reports in the trajectory of RV remodeling, which is further confounded by the lack of standardization in imaging measurements and timing of surveillance. Consistency in imaging protocol and lengthening follow-up will hopefully provide a more robust understanding about the evolution of the postoperative RV. Improvement in functional status and aerobic capacity can also be appreciated post-Cone repair, particularly in highly symptomatic patients. Innovation to promote remodeling of the Ebsteinoid heart include stem cell therapy at the time of surgical repair, which has shown promise in phase I clinical trial. Lastly, the goal of biventricular repair through Cone reconstruction can be facilitated by Starnes palliation to stabilize critical presentations, a paradigm shift that is increasingly adopted.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"23 ","pages":"Article 100649"},"PeriodicalIF":1.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145939396","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":"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}