International journal of cardiology. Congenital heart disease最新文献

{"title":"Role of balloon pulmonary valvuloplasty in symptomatic infants with tetralogy of Fallot awaiting intracardiac repair","authors":"Sanjeev Hanumantacharya Naganur,&nbsp;Abhishek Mallick,&nbsp;Manoj Kumar Rohit,&nbsp;Parag Barwad,&nbsp;Vinothkumar S.P.","doi":"10.1016/j.ijcchd.2025.100621","DOIUrl":"10.1016/j.ijcchd.2025.100621","url":null,"abstract":"<div><h3>Background and aim</h3><div>Mortality risk in tetralogy of Fallot (TOF) is highest in infancy, predominantly due to hypercyanotic spells. Timing of corrective surgery is centre/expertise dependent and surgical shunt procedures carry high mortality risk. We aimed to study the efficacy and feasibility of balloon pulmonary valvuloplasty (BPV) in TOF physiology children, as an alternative to surgical shunt, in those infants who were severely cyanosed (saturation &lt;75 %) or having recurrent spells and who were still awaiting intracardiac repair (ICR). The primary objective was to assess the following parameters pre and post BPV: resting saturation, Z score of pulmonary annuli by 2D Echocardiography, right ventricular outflow tract (RVOT) gradient, hypercyanotic spell frequency. Secondarily we looked at the modified Nakata index (MNI), any procedural complications, requirement of early shunt or ICR (within study period).</div></div><div><h3>Materials and methods</h3><div>This was a single arm interventional study done over a period of one year. Values pre and post intervention were compared using Wilcoxon matched pair signed rank test and Chi-square test for categorical variables as appropriate. Independent groups were compared using Mann Whitney <em>U</em> test. The children were followed up for a period of 6 months post procedure.</div></div><div><h3>Results</h3><div>After enrolling 10 infants, data could be studied in 9 of them. 1 child was lost to follow up. Significant improvement was noted in saturation, size of PA annulus, RVOT gradient, MNI (p value &lt; 0.05 each). 2 patients had access site thrombosis which resolved with heparin. 1 had transient complete heart block which resolved within 24 h but required temporary pacing. There was no mortality and none had more than mild pulmonary regurgitation. Out of 5 children who presented with recurrent spells, 2 were completely free of spells after BPV, 2 had spells with reduced frequency/duration, 1 continued to have spells needing surgical intervention. (p = 0.36). Apart from this child who needed surgery, one other patient remained severely cyanotic and had to undergo a shunt procedure.</div></div><div><h3>Conclusion</h3><div>To conclude, BPV seems like a safe and cost-effective alternative to high-risk surgical shunt palliation in symptomatic TOF infants, in resource constrained regions.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100621"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361642","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":"Long-term outcome after surgical ventricular septal defect closure: Longitudinal follow-up into the fifth decade","authors":"Sahra Ünlütürk ,&nbsp;Robert M. Kauling ,&nbsp;Judith A.A.E. Cuypers ,&nbsp;Annemien E. van den Bosch ,&nbsp;Alexander Hirsch ,&nbsp;Chiara Pelosi ,&nbsp;Daniel J. Bowen ,&nbsp;Raluca G. Chelu ,&nbsp;Ad J.J.C. Bogers ,&nbsp;Willem A. Helbing ,&nbsp;Isabella Kardys ,&nbsp;Jolien W. Roos-Hesselink","doi":"10.1016/j.ijcchd.2025.100624","DOIUrl":"10.1016/j.ijcchd.2025.100624","url":null,"abstract":"<div><h3>Objectives</h3><div>To evaluate survival, clinical outcome and quality of life (QoL) of patients up to 49 years after surgical ventricular septal defect (VSD) closure.</div></div><div><h3>Methods</h3><div>Single-center, longitudinal cohort study evaluating consecutive patients with surgical VSD closure between 1968 and 1980 with extensive cardiac and QoL evaluation every decade.</div></div><div><h3>Results</h3><div>Of the original cohort of 174 patients, 39 died (22 %), 8 were lost to follow-up and 38 had not participated previously. Survival rate at 49 years follow-up was 77 % and 86 % when excluding early postoperative mortality. Of the 89 eligible survivors, 76 (85 %) were evaluated (59 % male, median age 49 [44–54] years) with a median follow-up of 44 (range 40–49) years after surgery. Event-free survival at 49 years was 50 %, with symptomatic arrhythmias (10 %), pacemaker implantation (8 %) and VSD-related interventions (3 %) being common complications. At last follow-up, 58 % had left atrial dilation, 25 % had aortic regurgitation and 5 patients (7 %) had a residual VSD. Early postoperative arrhythmias predicted mortality. Both left ventricular (LV) and right ventricular ejection fraction remained stable, with only 1 % having an LV ejection fraction below 45 % at last follow-up. Exercise capacity and VO₂max were mildly reduced in 33 % and 49 % of the patients while self-perceived QoL was stable and comparable with the general Dutch population.</div></div><div><h3>Conclusion</h3><div>Half of the patients with surgical VSD closure had an event-free survival at 49 years. Pacemaker implantation was often needed. Early postoperative arrhythmias predicted mortality. QoL was good and remained stable over time.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100624"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361636","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":"Physical fitness in adults with isolated secundum atrial septal defects","authors":"Linda Ashman Kröönström ,&nbsp;Anna-Klara Zetterström ,&nbsp;Mikael Dellborg ,&nbsp;Åsa Cider ,&nbsp;Peter Eriksson ,&nbsp;Emelie Dahlin ,&nbsp;Zacharias Mandalenakis","doi":"10.1016/j.ijcchd.2025.100632","DOIUrl":"10.1016/j.ijcchd.2025.100632","url":null,"abstract":"<div><h3>Background</h3><div>Atrial septal defect (ASD) is one of the most common diagnoses in patients with adult congenital heart disease (ACHD). However, recent studies indicate that ASD is not so benign and may impede physical fitness. We aimed to assess physical fitness in patients with isolated secundum ASD compared with healthy reference values and to evaluate the results according to the absence or presence of repair.</div></div><div><h3>Methods</h3><div>Between April 2009 and March 2025, patients with ACHD performed physical fitness tests comprising five muscular fitness tests and one cardiorespiratory endurance test. Data were stored in a registry at the Physical Therapy Department, Sahlgrenska University Hospital/Östra. In total, 102 adults with isolated secundum ASD were included in this descriptive, register-based cohort study.</div></div><div><h3>Results</h3><div>Both women and men with ASD showed significantly lower isoinertial muscular fitness test results (heel lifts: p &lt; 0.001 and shoulder flexions: p &lt; 0.001) and maximal exercise capacity (p &lt; 0.001) compared with healthy reference values. The shoulder flexion test revealed the lowest percentage of reference values for women and men with ASD (58.3 % and 53.3 %, respectively). Women who had undergone surgical procedures performed the timed-stands test significantly faster when compared with women who had undergone device procedures (13.0 s [10.2, 16.0] vs. 19.1 s [15.1, 26.2]; p = 0.014).</div></div><div><h3>Conclusion</h3><div>Patients with less complex ACHD, such as secundum ASD, may have impaired muscular fitness and cardiorespiratory endurance. Therefore, all patients with ACHD could be offered tests of physical fitness. These tests are necessary when prescribing individualized exercise, as suggested by guidelines.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100632"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578754","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":"Pregnancy outcomes and effects on cardiac function among patients with Tetralogy of Fallot: A single-center cohort study","authors":"Ohad Houri ,&nbsp;Noam Shasho-Peres ,&nbsp;Eran Hadar ,&nbsp;Sharon Orbach Zinger ,&nbsp;Ran Kornowski ,&nbsp;Anat Pardo ,&nbsp;Shimon Kolker ,&nbsp;Shahar Vig ,&nbsp;Asnat Walfisch ,&nbsp;Bar Narkis ,&nbsp;Nili Schamroth Pravda","doi":"10.1016/j.ijcchd.2025.100613","DOIUrl":"10.1016/j.ijcchd.2025.100613","url":null,"abstract":"<div><h3>Background</h3><div>Data on the characteristics and outcomes of pregnancy amongst patients with Tetralogy of Fallot (TOF) is limited. Depending on the hemodynamic status of the patient, the risk of maternal and fetal complications during pregnancy can be considerable.</div></div><div><h3>Objectives</h3><div>We aimed to systematically evaluate maternal, obstetric, and fetal outcomes, and assess longitudinal changes in cardiac function among women with TOF using serial imaging.</div></div><div><h3>Methods</h3><div>Pregnant adult patients with TOF between 2014 and 2022 were included. We examined maternal and obstetric outcomes during pregnancy. We assessed serial imaging to evaluate the temporal effects of pregnancy on cardiac function.</div></div><div><h3>Results</h3><div>Our cohort included 50 patients. There were 85 pregnancies overall with 79 live births (92.9 %). The median gestational age at delivery was 38.3 [37.0–39.1] gestational weeks (GW), nine deliveries (11.3 %) occurred before 37 GW, but only one before 34 GW. The median birthweight at delivery was 2850 [2450–3048] grams. The main fetal complication was small for gestational age (n = 16,22 %). There were no deaths in the peripartum period or the year following pregnancy. There were no significant valvular or ventricular functional differences when comparing pre-conception to post-conception echocardiogram data. In the subgroup with availability of MRI data, the Right ventricular end-diastolic volume index increased significantly post-pregnancy (109 ± 37.9 ml/m² vs 117 ± 25.0 ml/m², p &lt; 0.01).</div></div><div><h3>Conclusions</h3><div>Maternal and obstetric complications are rare among patients with TOF. Our initial findings suggest that pregnancy does influence cardiac dimensions on serial long-term imaging follow-up but further, expanded long-term data is needed.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100613"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922015","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":"Prognostic implications of heart failure rehospitalization in adults with CHD & clinical benefits of medical therapy","authors":"Emili P. Rosado Rodriguez,&nbsp;Cynthia S. Crowson,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100640","DOIUrl":"10.1016/j.ijcchd.2025.100640","url":null,"abstract":"<div><h3>Background</h3><div>The risk factors for heart failure (HF) rehospitalization, and the effect of guideline directed medical therapy (GDMT) are poorly understood in adults with congenital heart disease (ACHD). We aimed to describe the outcomes of HF hospitalization and to assess the clinical benefits of GDMT for HF.</div></div><div><h3>Methods</h3><div>We conducted a retrospective cohort study of ACHD hospitalized for HF at Mayo Clinic from 2003 to 2023. Cox proportional hazard models were used to determine the predictors of HF rehospitalization and all-cause mortality, and to assess the association between death and GDMT, as measured by the Heart Failure Collaborative score.</div></div><div><h3>Results</h3><div>Of the 324 patients, 164 were rehospitalized for HF and 149 died. The 10-year cumulative incidence of rehospitalization was 75.8 % (95 % CI 70.1 %–81.5 %), and the 10-year survival rate from mortality was 47.3 % (95 % CI 40.9 %–54.7 %). Chronic kidney disease (HR 1.46, 95 % CI 1.06–2.01, p = 0.021) and Fontan physiology (HR 1.59, 95 % CI 1.02–2.49, p = 0.043) were associated with HF rehospitalization. HF rehospitalization within 1 year was associated with nearly a 3-fold increased risk of mortality (HR 2.88, 95 % CI 2.00–4.15, p &lt; 0.001). The HFC score was associated with a lower risk of all-cause mortality (HR 0.77, 95 % CI 0.62–0.95, p = 0.016) in the subgroup of patients with reduced ejection fraction.</div></div><div><h3>Conclusions</h3><div>Among adults with CHD hospitalized for HF, half of the patients were rehospitalized for HF. Rehospitalization within 1 year of the index HF hospitalization was associated with mortality. The use of GDMT for HF was associated with improved survival in patients with reduced ejection fraction.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100640"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145623321","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 role of artificial intelligence and mobile health in diagnosis and management of pulmonary arterial hypertension","authors":"Maria Luisa Benesch Vidal ,&nbsp;Alexandra Arvanitaki ,&nbsp;Gerhard-Paul Diller","doi":"10.1016/j.ijcchd.2025.100622","DOIUrl":"10.1016/j.ijcchd.2025.100622","url":null,"abstract":"<div><div>Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by pulmonary vascular remodeling, increased pulmonary vascular resistance, and ultimately right ventricular failure. Despite therapeutic advances, delayed diagnosis and imprecise risk stratification remain key challenges. Artificial intelligence (AI) and machine learning (ML) offer opportunities across the care continuum. This includes early detection from electronic health records, electrocardiography and imaging, automated, standardized interpretation of echocardiography, computed tomography (CT), and cardiac magnetic resonance (CMR) potentially expediting referral and final diagnosis. Deep learning applied to echocardiography achieves expert-level PAH classification and provides automated right-heart quantification while CT/CMR-based models segment the heart and great vessels, quantify lung disease radiomics, and infer hemodynamics, supporting noninvasive triage and prognostication. In future, remote monitoring with wearables and telemedicine, coupled with AI analytics, promises to enable proactive management and potentially reduce hospitalizations. While early studies are promising, translation to practice requires rigorous external validation, prospective studies, bias auditing and seamless integration into guideline-aligned workflows. Overall, AI stands to augment expert clinical judgement by converting high-dimensional, multimodal data into actionable insights. With careful governance and evidence generation, AI has the potential to shorten time-to-diagnosis, refine risk stratification, and personalize therapy in PAH, ultimately improving outcomes for this high-risk population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100622"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361706","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 course of severe congenital aortic valve stenosis in children","authors":"Maximiliaan L. Notenboom ,&nbsp;Sencer Albayrak ,&nbsp;Kevin M. Veen ,&nbsp;Ingrid van Beynum ,&nbsp;Rebecca Swens ,&nbsp;Jolien W. Roos-Hesselink ,&nbsp;Pieter C. van de Woestijne ,&nbsp;Jonathan R.G. Etnel ,&nbsp;Willem A. Helbing ,&nbsp;Johanna J.M. Takkenberg ,&nbsp;Ad J.J.C. Bogers","doi":"10.1016/j.ijcchd.2025.100626","DOIUrl":"10.1016/j.ijcchd.2025.100626","url":null,"abstract":"<div><h3>Background</h3><div>Congenital valvular aortic stenosis (VAS) in children requires lifelong follow-up. An overview of age-specific treatment pathways from first diagnosis of severe VAS is lacking.</div></div><div><h3>Objectives</h3><div>To assess patient journeys and outcomes after severe pediatric VAS diagnosis, by describing its clinical course and treatment trajectories based on a 37-year single-center experience.</div></div><div><h3>Methods</h3><div>Baseline and time-related data of children diagnosed with severe congenital VAS between 1985 and 2022 were retrospectively collected. Time-related death (Kaplan-Meier estimator) and intervention occurrence (Aalen-Johansen estimator) were analyzed.</div></div><div><h3>Results</h3><div>245 children (73.1 % male, median age: 1.2 years (IQR:0.1–7.0)) were diagnosed with severe VAS (53 aged &lt;30 days, 74 between 30d-1y, 84 between 1y-12y, 34 between 12y-18y). Median survival follow-up was 23.3 years (IQR:10.3–31.2) (99.0 % complete). Thirty-five-year incidence of death after diagnosis was 16.2 %(95 %CI:9.7–22.2 %). Of 245 patients, 211 patients (86.1 %) underwent an intervention and 34 (13.9 %) did not undergo an intervention. Of these 34 children, 7 children showed time-related Doppler gradient regression to mild-or-moderate VAS and 17 had stable severe VAS. These 24 children experienced a 6.0 % incidence of death at 30-years after diagnosis. The most common intervention over time (47.5 %) was balloon valvuloplasty, especially in neonates and infants, followed by aortic valve replacement (37.5 %), especially in older children.</div></div><div><h3>Conclusions</h3><div>This study highlights the vast heterogeneity of treatment pathways and outcomes in children diagnosed with severe VAS at different ages. The observed stabilization of severe VAS or regression of the serial peak Doppler gradient to mild-or-moderate VAS without symptoms in 24 children highlights the need for better insight into determinants of disease course.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100626"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361707","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":"Patient-specific mixed reality for venus P-valve implantation: A novel approach to procedural planning","authors":"Angelo Fabio d’Aiello ,&nbsp;Francesca Bevilacqua ,&nbsp;Angelo Micheletti ,&nbsp;Diana Gabriela Negura ,&nbsp;Giulia Pasqualin ,&nbsp;Luca Giugno ,&nbsp;Luciane Piazza ,&nbsp;Laura Schianchi ,&nbsp;Sara Boveri ,&nbsp;Alessandro Giamberti ,&nbsp;Massimo Chessa","doi":"10.1016/j.ijcchd.2025.100614","DOIUrl":"10.1016/j.ijcchd.2025.100614","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Survival rates for patients with congenital heart disease (CHD) have improved, but complications like pulmonary regurgitation (PR) often require re-interventions. Transcatheter pulmonary valve implantation (TPVI) with self-expandable valves, such as the Venus P-Valve, has broadened treatment options. Accurate procedural planning, particularly valve sizing, remains a significant challenge. Mixed Reality (MxR) technology enables a patient-specific approach that enhances procedural planning accuracy.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Aim&lt;/h3&gt;&lt;div&gt;To evaluate the use of MxR in planning Venus-P valve implantation.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Materials and methods&lt;/h3&gt;&lt;div&gt;This study included patients undergoing Venus P-Valve implantation with holographic models generated from CT data using ARTICOR® software from January 2023 to June 2024. Two independent operators used these models for procedural planning. Concordance between operators was assessed. Predictions were compared with implanted valve dimensions to evaluate concordance.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Of 29 eligible patients, 26 underwent successful Venus-P valve implantation. Concordance between the operators reached 60 % (n = 15/26) for valve diameter and over 88 % (n = 23/26) for valve length. Holographic models achieved 96 % (n = 25/26) concordance in predicting valve length, type of the approach (92 %) and 50 % (n = 13/26) concordance for diameter.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Discussion&lt;/h3&gt;&lt;div&gt;Holographic models enhanced procedural planning, enabling better visualization and collaborative decision-making. While highly effective for valve length predictions, and type of the approach limitations in predicting valve diameter highlight the need for improved methods, such as computational modelling or machine learning.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;Patient-specific holographic models are promising tools for TPVI planning. Advancements in technology and interdisciplinary collaboration are critical to overcoming current limitations and advancing procedural planning and related outcomes in interventional cardiology.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Condensed abstract&lt;/h3&gt;&lt;div&gt;This study assesses the use of mixed reality (MxR) technology for procedural planning in transcatheter pulmonary valve implantation (TPVI) with the Venus P-Valve. Holographic models were created from CT data to aid in valve sizing and implantation strategies, with two operators comparing measurements for valve diameter and length. Among 26 patients who underwent successful implantation, concordance between operators was 60 % for valve diameter and 88 % for valve length. Predictions using holographic models showed 96 % concordance for implanted valve length, type of the approach (92 %) and 50 % concordance for valve diameter. These results highlight the potential of MxR for improving TPVI planning, though the limited accuracy for valve diameter suggests a need for further advancements, such as computational modeling or ","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100614"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106151","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":"Mortality rates in adults with severe congenital heart disease: insights from the National Congenital Heart Disease Registry of Australia","authors":"Jason Chami MD ,&nbsp;Calum Nicholson ,&nbsp;David Baker ,&nbsp;Rachael Cordina ,&nbsp;Geoff Strange ,&nbsp;David S. Celermajer","doi":"10.1016/j.ijcchd.2025.100629","DOIUrl":"10.1016/j.ijcchd.2025.100629","url":null,"abstract":"<div><h3>Background</h3><div>Survival into adulthood with congenital heart disease (CHD) has improved, but premature mortality remains common. Long-term, multi-centre data are scarce. We used a validated complexity algorithm to select “severe CHD” patients from the National CHD Registry of Australia, quantifying mortality by ventricular physiology, residential remoteness and treating-centre location.</div></div><div><h3>Methods</h3><div>From 70185 CHD patient records, we identified 6557 patients classified as “severe” by 2020 European Society of Cardiology guidelines, born from 1980 onwards and alive at 16 years old. We found 2118 single-ventricle patients, 3674 two-ventricle and 765 with insufficient information for classification. Residence was grouped into Major Cities, Inner Regional, and Outer Regional/Remote. Vital status follow-up was to December 31, 2023. Kaplan–Meier curves were used to compare survival.</div></div><div><h3>Results</h3><div>Overall, 25-year survival was 96.9 % and 40-year survival was 89.0 %. Survival differed by physiology: single-ventricle survival was 96.4 % at 25 years and 88.2 % at 40 years, while two-ventricle survival was better, at 97.6 % at 25 years and 90.8 % at 40 years (p = 0.0015). Patients managed in Sydney/Melbourne fared better than those treated elsewhere, particularly after age 35 (25-year survival ∼97 % for both; 40-year survival 89.9 % vs 81.9 %; p = 0.027). Survival did not, however, vary with residential remoteness.</div></div><div><h3>Conclusion</h3><div>Adults with severe lesions, particularly single-ventricle physiology, remain at high risk of premature mortality. Patients managed in Sydney/Melbourne had better survival, possibly reflecting the influence of long-established specialist ACHD programs, although residual confounding cannot be excluded. Urban-rural outcome parity suggests that Australia's centralised care model mitigates geographical barriers.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100629"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578755","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 rudimentary left ventricle does not impact on right ventricular size and function in hypoplastic left heart syndrome during serial follow up after Fontan completion","authors":"Abigail Burleigh ,&nbsp;Dominik Daniel Gabbert ,&nbsp;Yujiro Ide ,&nbsp;Anselm Uebing ,&nbsp;Inga Voges","doi":"10.1016/j.ijcchd.2025.100627","DOIUrl":"10.1016/j.ijcchd.2025.100627","url":null,"abstract":"<div><h3>Background</h3><div>Previous studies in patients with hypoplastic left heart syndrome (HLHS) suggested that a larger left ventricle (LV) might negatively impact right ventricular (RV) function. This study aimed to analyse the impact of the presence of an LV remnant on RV size and function during serial follow up.</div></div><div><h3>Methods</h3><div>Serial cardiovascular magnetic resonance (CMR) examinations after completion of the total cavopulmonary connection were retrospectively analysed. Patients were divided into those with and those without a rudimentary LV. RV and LV end diastolic and end systolic volumes as well as stroke volume, ejection fraction (RVEF, LVEF) and end diastolic mass were measured.</div></div><div><h3>Results</h3><div>90 HLHS patients (female: 26) who had at least two CMR examinations were included. 51 patients had three examinations. 56 patients had an LV remnant, 34 did not. RV volumes and mass indexed to body surface area as well as RVEF did not differ significantly between both groups. LV volumes showed no association with RV volumes and RVEF.</div></div><div><h3>Conclusion</h3><div>Analysis of serial CMR examination suggests that the presence of an LV remnant does not have a major impact on RV size and function during longer-term follow-up. Future studies might focus on regional RV function.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100627"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361644","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}