Sophia Anastasia Mouratoglou , Alexandra Arvanitaki , George Papadopoulos , Rogerio Souza , George Giannakoulas
{"title":"Pulmonary arterial hypertension treatment. A new era","authors":"Sophia Anastasia Mouratoglou , Alexandra Arvanitaki , George Papadopoulos , Rogerio Souza , George Giannakoulas","doi":"10.1016/j.ijcchd.2025.100594","DOIUrl":"10.1016/j.ijcchd.2025.100594","url":null,"abstract":"<div><div>Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100594"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144221932","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}
Amr Moustafa, Ahmed T Abdelhalim, William R Miranda, Heidi M Connolly, Elizabeth H Stephens, Joseph A Dearani, Alexander C Egbe
{"title":"Mechanical valve replacement in adults with Fontan physiology is an effective palliation in selected patients.","authors":"Amr Moustafa, Ahmed T Abdelhalim, William R Miranda, Heidi M Connolly, Elizabeth H Stephens, Joseph A Dearani, Alexander C Egbe","doi":"10.1016/j.ijcchd.2025.100612","DOIUrl":"10.1016/j.ijcchd.2025.100612","url":null,"abstract":"<p><strong>Background: </strong>Valve regurgitation is associated with systemic ventricular dysfunction and mortality in patients with Fontan palliation. Valve repair and valve replacement with bioprostheses have limited durability, and outcomes after valve replacement with mechanical prostheses are poorly defined. The study objectives were to assess: (1) Operative mortality, valve-related complications (stroke, mechanical valve thrombosis, major bleeding complication, prosthetic valve endocarditis, and valve reoperation), and late mortality/transplant. (2) Postoperative changes in ventricular volumes and ejection fraction; (3) Postoperative changes in the clinical indices of disease severity.</p><p><strong>Method: </strong>Retrospective cohort study of adults (age ≥18 years) with Fontan palliation who underwent isolated aortic or atrioventricular valve replacement at Mayo Clinic (2003-2023). Preoperative and postoperative evaluations (clinical evaluation, echocardiogram, biomarkers) were performed 6 months preoperatively and 6-24 months postoperatively, respectively.</p><p><strong>Results: </strong>Overall, 17 patients underwent isolated atrioventricular (N = 9) or aortic valve (N = 8) replacement with mechanical prosthesis (age 27 ± 8 years; 9 [53 %] males). There was no operative mortality. There was postoperative improvement in functional capacity (NYHA class), heart failure indices (NT proBNP), and hepatorenal function (MELD-XI score). There was a postoperative decrease in systemic ventricular volumes, without change in ejection fraction. The 10-year cumulative incidence of valve-related complications and death/transplant was 15 % and 18 %, respectively.</p><p><strong>Conclusions: </strong>Atrioventricular valve and aortic valve replacement with mechanical prostheses was associated with low (zero) operative mortality, low risk of valve-related complications, and clinical improvement. This suggests that valve replacement with mechanical prostheses may be an effective palliation in selected patients and may delay the need for transplant in this population.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100612"},"PeriodicalIF":1.2,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12356454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M Pilati, F A D'Aiello, M Chessa, G Santoro, A Donti, E Mariucci, C Marrone, G Annoni, G Gaio, M Rebonato, E Piccinelli, M Cheli, R Formigari, G Butera
{"title":"Percutaneous implantation of self-expanding pulmonary valves: results from real-life experience of the Venus-P valve Registry of the Italian Society of Pediatric Cardiology (SICPED).","authors":"M Pilati, F A D'Aiello, M Chessa, G Santoro, A Donti, E Mariucci, C Marrone, G Annoni, G Gaio, M Rebonato, E Piccinelli, M Cheli, R Formigari, G Butera","doi":"10.1016/j.ijcchd.2025.100609","DOIUrl":"10.1016/j.ijcchd.2025.100609","url":null,"abstract":"<p><strong>Background: </strong>Self-expandable valves may have a significant impact on the feasibility of transcatheter treatments of RVOT dysfunction. Venus P valve is the first autoexpadable Valve that received CE mark in 2022. Here, we are reporting on a multicenter data collection about patients undergoing Venus P valve implantation in Italy.</p><p><strong>Materials: </strong>Seven italian centers were included in the study. Data were collected from patients treated with a venus P valve between July 2022 and May 2024. Indication for percutaneous valve implantation were in accordance with published guidelines.</p><p><strong>Results: </strong>During the study period, a total of 65 patients were considered eligible to percutaneous implantation of a Venus P valve. Median age at procedure was 35 years (IQR 12-63 years) median weight 63 Kg (IQR 33-103 Kg). Indication for PPVI was severe regurgitation in all subjects but one where ventricular pressure was increased because of stenosis on a homograft conduit and pulmonary arteries which needed previous bilateral stenting. Venus P valve was implanted successfully in 61/65(94 %) patients. Four procedural complications occurred(6 %): 1 artero-venous fistula, 1 pulmonary bleeding, 1 hemothorax and a pulmonary oedema. During follow up (median 13 months,1-20months), all patients experienced a significant improvement of functional class. Neither deaths or re-intervention occurred. CT Scan showed hypoattenuating leaflet thickness (HALT) in 4 patients and one of them experienced endocarditis 12 months after the procedure.</p><p><strong>Conclusions: </strong>Our study shows that Venus P valve implantation is safe and results are stable during a short-to-medium term follow-up. Endocarditis and HALT are inducing some concern and larger studies are needed.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100609"},"PeriodicalIF":1.2,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12355537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Larissa Lloyd, Sasha Ruban, Tanya Badal, Geoff Strange, David S Celermajer, Carissa Bonner
{"title":"Living with congenital heart disease: a qualitative study to explore psychosocial impacts in adulthood.","authors":"Larissa Lloyd, Sasha Ruban, Tanya Badal, Geoff Strange, David S Celermajer, Carissa Bonner","doi":"10.1016/j.ijcchd.2025.100610","DOIUrl":"10.1016/j.ijcchd.2025.100610","url":null,"abstract":"<p><strong>Background: </strong>The survival prospects of adults with congenital heart disease have improved considerably due to improved surgical interventions, resulting in a growing population. This study aims to explore the experiences of adults with congenital heart disease, to identify support needs.</p><p><strong>Methods: </strong>Participants were recruited from the National Australian Congenital Heart Disease Registry using purposive sampling to ensure diversity in terms of gender, rurality and disease complexity. Semi-structured interviews were conducted with adults with congenital heart disease. The interviews were recorded digitally and transcribed verbatim. Framework Analysis was used to ensure rigour in identifying themes.</p><p><strong>Results: </strong>43 interviews were completed (23 males and 20 females; age 20 to 54 years). Three main themes and seven subthemes were identified to explain varying experiences of living with congenital heart disease: I. Lifestyle factors; (i) physical activity, (ii) weight management. II. Practical issues; (iii) employment, (iv) financial stress, (v) education. III. Psychosocial impact; (vi) mental health and resilience, and (vii) social engagement. Participants across a range of disease complexities reported more impact on their lives when they felt inadequately supported by their healthcare providers to address these challenges, such as insufficient education on exercise leading to difficulty performing clinically recommended physical activity.</p><p><strong>Conclusions: </strong>The lived experiences of adults living with congenital heart disease highlight specific areas needing support, which could inform future improvements in their \"whole of life\" care. This could include referral to allied health professionals, education across lifestyle and practical domains, and appropriate peer support networks.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100610"},"PeriodicalIF":1.2,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12355555/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ismael Z. Assi , Michael J. Landzberg , Kristian C. Becker , David Renaud , Fernando Baraona Reyes , David M. Leone , Mark Benson , Miriam Michel , Robert E. Gerszten , Alexander R. Opotowsky
{"title":"Correlation between Olink and SomaScan proteomics platforms in adults with a Fontan circulation","authors":"Ismael Z. Assi , Michael J. Landzberg , Kristian C. Becker , David Renaud , Fernando Baraona Reyes , David M. Leone , Mark Benson , Miriam Michel , Robert E. Gerszten , Alexander R. Opotowsky","doi":"10.1016/j.ijcchd.2025.100584","DOIUrl":"10.1016/j.ijcchd.2025.100584","url":null,"abstract":"<div><h3>Background</h3><div>High-throughput proteomics platforms using aptamers (SomaScan) or proximity extension assay (Olink) provide novel opportunities for improving diagnostic and risk stratification tools in cardiovascular diseases, including understudied congenital heart diseases. The correlation between these proteomics approaches has not yet been studied among individuals with a Fontan circulation.</div></div><div><h3>Objective</h3><div>The correlation of plasma protein measurements between SomaScan and Olink platforms was evaluated in adults with a Fontan circulation.</div></div><div><h3>Methods</h3><div>We measured 491 proteins in plasma of 71 adults with a Fontan circulation using Olink and SomaScan. Missing Olink measurements (0.13%, 47/34,861) were imputed using non-parametric imputation. Spearman's rank correlation coefficient for absolute values of protein expression between platforms was calculated. Protein correlation frequencies were compared to 3 cohorts reported in the literature using Pearson's Chi-squared test of independence.</div></div><div><h3>Results</h3><div>Overall, protein correlations between Olink and SomaScan measurements were moderately strong for most proteins, (rho > 0.4 for 57.2%), but with substantial variability (median correlation = 0.457, IQR = 0.538). The distribution of protein correlations was qualitatively similar to published literature in non-Fontan cohorts. Both Olink and SomaScan identified proteins with sex-based differences; both identified differences in myostatin and leptin, but each identified additional nonoverlapping sexually dimorphic proteins (n = 14 Olink, n = 5 SomaScan).</div></div><div><h3>Conclusions</h3><div>In adults with a Fontan circulation, correlations between plasma proteins measured by Olink and SomaScan varied widely, approximately in line with prior reports in other populations. While these tools may be uniquely useful to generate hypotheses, specifically regarding potential molecular mechanisms, more definitive inference requires independent validation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100584"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865112","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}
Raksheeth Agarwal , Louise E. Coats , Ali N. Zaidi
{"title":"Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead","authors":"Raksheeth Agarwal , Louise E. Coats , Ali N. Zaidi","doi":"10.1016/j.ijcchd.2025.100589","DOIUrl":"10.1016/j.ijcchd.2025.100589","url":null,"abstract":"<div><div>Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100589"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143911594","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}
Nunzia Borrelli , Nicola Grimaldi , Flavia Fusco , Antonio Orlando , Michela Palma , Maria Cristina Boccia , Sabrina Bassolino , Anna Iervolino , Berardo Sarubbi
{"title":"Feasibility and effectiveness of telemedicine for adult patients with congenital heart disease: A one-year single-center experience-based study","authors":"Nunzia Borrelli , Nicola Grimaldi , Flavia Fusco , Antonio Orlando , Michela Palma , Maria Cristina Boccia , Sabrina Bassolino , Anna Iervolino , Berardo Sarubbi","doi":"10.1016/j.ijcchd.2025.100582","DOIUrl":"10.1016/j.ijcchd.2025.100582","url":null,"abstract":"<div><h3>Introduction</h3><div>The number of adults with congenital heart disease has significantly increased in recent years. While telemedicine has emerged as a promising approach to improve care delivery and patient outcomes, its use for adults with congenital cardiopathies has not been extensively explored. This study aims to evaluate the feasibility and clinical impact of a telemedicine programme for adults with congenital heart diseases.</div></div><div><h3>Methods</h3><div>This was a single-center, prospective, observational study, carried out between January 2022 and January 2023. A wristwatch, a paired mobile device, and a dedicated hospital workstation were used to telemonitor 25 adult patients with moderate-to-severe congenital heart condition, in II-IV New-York Heart Association class. We assessed changes in hospital admission days and related costs, patient satisfaction, and functional parameters, including 6-min walk test distance, vital signs, and echocardiographic ejection fraction.</div></div><div><h3>Results</h3><div>All 25 patients agreed to participate to the telemedicine program (mean age 38.35 ± 11.33 years, 52 % male). Twenty-three patients completed the program. No significant changes were observed in vital signs, functional class, ventricular ejection fraction, while walking distance significantly improved (404 ± 82 m versus 433 ± 142 m, p = 0.03). Notably, hospital admission days and related costs were significantly reduced during the TM program year compared to the prior year. Patient satisfaction was high. One patient experienced a prolonged hospitalization and death due to the natural progression of their condition.</div></div><div><h3>Conclusion</h3><div>High-risk patients with moderate-severe congenital heart disease can benefit from a device-implemented telemedicine program, which offers tailored, specialized care directly at home, reducing hospital admissions and ensuring stable clinical status.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100582"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935175","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":"Percutaneous repair of biatrial drainage of the right superior vena cava using a covered stent","authors":"Samuel L. Casella , Sangeeta Shah","doi":"10.1016/j.ijcchd.2025.100587","DOIUrl":"10.1016/j.ijcchd.2025.100587","url":null,"abstract":"<div><div>Biatrial drainage of the right superior vena cava (RSVC) is a rare anomaly of the cavo-pulmonary venous confluence which may lead to chronic cyanosis, stroke and cerebral abscess. Historical corrections have been limited to open surgical repair. We describe an adult patient with biatrial drainage of the RSVC with chronic cyanosis who underwent successful percutaneous correction using a covered stent.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100587"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935176","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}
Alex J. Katz , Myke Federman , Jeremy P. Moore , Jeannette P. Lin , Kuan-Ting Chen , Nicholas J. Jackson , Jamil Aboulhosn
{"title":"Organization and structure of intensive care management of adult congenital heart disease in the United States","authors":"Alex J. Katz , Myke Federman , Jeremy P. Moore , Jeannette P. Lin , Kuan-Ting Chen , Nicholas J. Jackson , Jamil Aboulhosn","doi":"10.1016/j.ijcchd.2024.100509","DOIUrl":"10.1016/j.ijcchd.2024.100509","url":null,"abstract":"<div><h3>Background</h3><div>The growing number of adults with congenital heart disease has led to the formation of dedicated centers to provide specialized care. There is a paucity of published data on the organization, staffing, and resources available when adult congenital heart disease (ACHD) patients are hospitalized in intensive care settings. This study aims to describe the delivery of intensive care for ACHD patients in the United States and identify key differences between accredited centers or those pending accreditation and non-accredited Adult Congenital Heart Association (ACHA) centers.</div></div><div><h3>Methods</h3><div>A web-based cross-sectional survey targeting centers with specialized ACHD programs was conducted.</div></div><div><h3>Results</h3><div>Data were obtained from 48 of 96 centers (50%). Twenty-seven (56%) were accredited by the ACHA and 8 (17%) were pending accreditation. Surgical ACHD patients were primarily co-managed by cardiologists and intensivists (n = 11, 23%) and treated within an adult cardiac surgical intensive care unit (n = 34, 72%). Compared to non-accredited centers, ACHA accredited centers or those pending accreditation were more likely to offer daily rounds with ACHD providers (30, 86% vs. 6, 46%, p=<0.01), were more likely to support an ACHD fellowship training program (16, 46% vs. 0, 0%, p=<0.01); and were more likely to have durable mechanical support, such as ventricular assist devices (35, 100% vs. 8, 62%, p < 0.01).</div></div><div><h3>Conclusion</h3><div>This survey reports current trends in organization, staffing, and resources available to ACHD patients requiring intensive care. ACHA accredited centers or those pending accreditation were more likely to have dedicated ACHD rounds, fellowship training programs, and access to durable mechanical support.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100509"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140399259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandra Poppe , Muneaki Matsubara , Jonas Palm , Thibault Schaeffer , Takuya Osawa , Carolin Niedermaier , Paul Philipp Heinisch , Nicole Piber , Bettina Ruf , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono
{"title":"Thrombus formation after the Norwood procedure: Incidence, risk factors, and its impact on late outcomes","authors":"Alessandra Poppe , Muneaki Matsubara , Jonas Palm , Thibault Schaeffer , Takuya Osawa , Carolin Niedermaier , Paul Philipp Heinisch , Nicole Piber , Bettina Ruf , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono","doi":"10.1016/j.ijcchd.2025.100575","DOIUrl":"10.1016/j.ijcchd.2025.100575","url":null,"abstract":"<div><h3>Objective</h3><div>Thrombus formation is a feared complication after congenital heart surgery. We aimed to clarify the clinical characteristics associated with thrombus formation after the Norwood procedure.</div></div><div><h3>Methods</h3><div>All neonates who underwent the Norwood procedure between 2001 and 2022 were reviewed. The incidence and location of thrombus were evaluated. Risk factors for thrombus formation and its impact on survival were analyzed.</div></div><div><h3>Results</h3><div>Among 360 patients who were included, thrombus formation was detected in 42 patients (11.7 %) during the postoperative in-hospital period, with a median of 12 (range: 5–30) postoperative days. The most common site of thrombus was the superior vena cava in 9 (2.5 %) patients, followed by the right atrium in 8 (2.2 %). Patients who received a right ventricle to pulmonary artery conduit had a higher incidence of thrombus than those who received a modified Blalock-Taussig-Thomas shunt (16.4 vs. 7.7 %, p = 0.011). Patients with thrombus formation had a longer stay in the intensive care unit (ICU), than those without (median 21 vs. 13 days, p = 0.018). Survival after ICU discharge was lower in patients with thrombus than those without (57, 54, and 54 % vs 73, 71, and 70 % at 2, 4, and 6 years, respectively; p = 0.032). Restrictive atrial septal defect was identified as an independent risk for thrombus (odds ratio: 2.61; p = 0.005).</div></div><div><h3>Conclusions</h3><div>Thrombus formation was observed in 12 % of the patients during the hospital stay after the Norwood procedure and was associated with prolonged recovery and high mortality. A restrictive atrial septal defect was identified as a risk factor for thrombus formation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100575"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865111","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}