Andrew S. Mackie, Adriana Tremoulet, Anitha S. John, Teiji Akagi, Kenichiro Yamamura, Lucy Eun, Alain Fraisse, Nagib Dahdah
{"title":"Transition of care for adolescents and young adults with Kawasaki disease: An international survey","authors":"Andrew S. Mackie, Adriana Tremoulet, Anitha S. John, Teiji Akagi, Kenichiro Yamamura, Lucy Eun, Alain Fraisse, Nagib Dahdah","doi":"10.1016/j.ijcchd.2026.100701","DOIUrl":"10.1016/j.ijcchd.2026.100701","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100701"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551958/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148914581","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}
Marta Gil Molina, Francisco Buendía Fuentes, María Calvo Asensio, Miguel Ángel Arnau Vives, María Rodríguez Serrano, Jaume Aguero, Ana Osa Sáez, Luis Martínez Dolz, Joaquín Rueda Soriano
{"title":"Evolution of CA 125 and NT-proBNP in stable patients with Fontan circulation: longitudinal analysis with sex perspective","authors":"Marta Gil Molina, Francisco Buendía Fuentes, María Calvo Asensio, Miguel Ángel Arnau Vives, María Rodríguez Serrano, Jaume Aguero, Ana Osa Sáez, Luis Martínez Dolz, Joaquín Rueda Soriano","doi":"10.1016/j.ijcchd.2026.100708","DOIUrl":"10.1016/j.ijcchd.2026.100708","url":null,"abstract":"<div><h3>Background</h3><div>Monitoring heart failure in patients with Fontan circulation (FC) remains challenging due to the unique hemodynamic features of this physiology. Conventional heart failure biomarkers are not well validated in this population. We evaluated longitudinal changes in CA125 and NT-proBNP in clinically stable FC patients, with particular attention to sex-based differences.</div></div><div><h3>Methods</h3><div>We performed a retrospective longitudinal study including clinically stable FC outpatients with ≥2 biomarker measurements separated by ≥ 2 months. Temporal trends of CA125 and NT-proBNP were analyzed using repeated measurements and stratified by sex.</div></div><div><h3>Results</h3><div>Sixty-six outpatients were included, with a median of five determinations per patient. The extracardiac conduit was the most frequent Fontan type (72.7%). Baseline CA125 was 22.73 ± 46.26 U/mL and demonstrated a significant upward trend over time (+1.63 U/mL/year; p = 0.008). Baseline NT-proBNP was 165.13 ± 161.62 pg/mL, with a non-significant increase during follow-up (+3.5 pg/mL/year; p = 0.163). Baseline CA125 levels did not differ between sexes. During follow-up, CA125 remained stable in men (0.63 U/mL/year; p = 0.419) but increased significantly in women (2.75 U/mL/year; p < 0.001). NT-proBNP showed no significant sex differences either at baseline or longitudinally.</div></div><div><h3>Conclusions</h3><div>In clinically stable patients with Fontan circulation, CA125 levels increased significantly over time, particularly in women, whereas NT-proBNP levels remained largely stable. Serial biomarker assessment may provide complementary information for long-term clinical monitoring in this population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100708"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148854023","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}
{"title":"Meta-analysis of RAAS inhibition in patients with a systemic right ventricle","authors":"Yousef Baroudi, Bengt Johansson","doi":"10.1016/j.ijcchd.2026.100705","DOIUrl":"10.1016/j.ijcchd.2026.100705","url":null,"abstract":"<div><h3>Introduction</h3><div>Pharmacological therapy has transformed the management of heart failure, yet pivotal trials have primarily focused on acquired left ventricular disease. Evidence for renin-angiotensin-aldosterone system (RAAS) inhibition in systemic right ventricle (sRV) remains scarce, and current practice is largely based on extrapolation from left ventricular studies and expert consensus. This meta-analysis evaluates the efficacy and safety of RAAS blockade in patients with a failing sRV.</div></div><div><h3>Methods</h3><div>A PubMed search identified studies investigating RAAS inhibition in sRV failure. Reported outcomes included changes in NT-pro-BNP, right ventricular ejection fraction (RVEF), and peak oxygen uptake (VO<sub>2</sub>-max). Data were analyzed using conventional statistics and visualized with forest plots.</div></div><div><h3>Results</h3><div>Ten eligible studies were included, comprising a pooled cohort of 325 patients, of which 261 received active treatment. The population was predominantly male (67.1%), with mean cohort ages ranging from 25.2 to 48.5 years. Mean treatment durations spanned 3.5 to 37.2 months. RAAS inhibition was associated with a reduction in NT-pro-BNP (Cohen's d = –0.28, 95% CI –0.49 to –0.07, <em>p</em> = 0.01). However, no improvements in RVEF or VO<sub>2</sub>-max were observed. The therapy was generally well-tolerated. The overall mortality incidence was 5.6 deaths per 1000 person-years in the active treatment groups.</div></div><div><h3>Conclusion</h3><div>The evidence for RAAS inhibition in patients with sRV is limited but suggests a beneficial reduction in NT-pro-BNP, indicating a hemodynamic effect but without improvements in RVEF or VO<sub>2</sub>-max. When combined with extrapolated data from large left ventricular heart failure trials, these findings support that RAAS inhibition is safe and may be considered in patients with sRV failure.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100705"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13551887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148914586","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}
{"title":"High prevalence of pes planus in children with Fontan circulation","authors":"Lisheng Lin, Junko Shiono, Shinpei Goto, Yusuke Yano, Yuta Tsukagoshi, Yukiko Ban, Masakazu Abe, Hitoshi Horigome","doi":"10.1016/j.ijcchd.2026.100711","DOIUrl":"10.1016/j.ijcchd.2026.100711","url":null,"abstract":"<div><h3>Background</h3><div>The musculoskeletal system is important for maintaining Fontan circulation. However, little is known about the foot morphology in this population. This study aimed to evaluate the prevalence of pes planus and its association with the patients’ physical characteristics after the Fontan procedure.</div></div><div><h3>Methods</h3><div>This single-center, cross-sectional study evaluated patients who underwent the Fontan procedure. Pes planus was assessed via physical examination. Physical measurements, including height, weight, body mass index, and fat-free mass index (FFMI), were collected and compared between patients with and without pes planus. Multivariable logistic regression analysis adjusted for age and sex, and time since the Fontan procedure was performed.</div></div><div><h3>Results</h3><div>Forty-seven patients (median age: 13 years) were included. Pes planus was observed in 27 (57%) patients and was present in all age groups. The patients with pes planus had a significantly lower height SDS (P = 0.011) and tended to have a lower FFMI (P = 0.066). In the adjusted analysis, a lower FFMI showed a trend toward an association with pes planus, although this did not reach significance (OR 0.648, 95% CI 0.38–1.12, P = 0.118).</div></div><div><h3>Conclusions</h3><div>Pes planus is highly prevalent in patients with Fontan circulation and may represent an under-recognized musculoskeletal phenotype. Further studies are required to clarify the etiology and clinical significance.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100711"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148951336","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}
Floor C. van der Meijs, Philippine Kiès, Loes A.M. van Winden, Marloes Duijkers, Veronica R. Janssen, Henk W. Elzevier
{"title":"Dating, disclosure, and romantic relationships pose challenges for adults with congenital heart disease: A mixed-methods study","authors":"Floor C. van der Meijs, Philippine Kiès, Loes A.M. van Winden, Marloes Duijkers, Veronica R. Janssen, Henk W. Elzevier","doi":"10.1016/j.ijcchd.2026.100710","DOIUrl":"10.1016/j.ijcchd.2026.100710","url":null,"abstract":"<div><h3>Background</h3><div>As survival into adulthood has improved, adults with congenital heart disease (CHD) increasingly face psychosocial challenges related to long-term living with their condition. Romantic relationships are a key source of social support, yet the impact of the condition on dating and romantic experiences remains poorly understood.</div></div><div><h3>Objectives</h3><div>To explore dating and romantic relationship experiences in adults with congenital heart disease (CHD), focusing on disclosure and communication, while describing patterns by sex and disease complexity and incorporating partner perspectives.</div></div><div><h3>Methods</h3><div>This national cross-sectional online survey included individuals aged ≥16 years with CHD. Dating- and relationship-related questionnaire items were analyzed using descriptive quantitative and inductive thematic qualitative analyses, including descriptive subgroup analyses by sex and disease complexity. Partner perspectives were included to provide relational context.</div></div><div><h3>Results</h3><div>Among 109 CHD patients (69% women, 54% aged 26-40 years, 87% with moderate to severe disease), 21% reported dating difficulties, 40% difficulties disclosing their condition to dates, and 14% relationship dissolution attributed to CHD. Relationship challenges mainly concerned family planning (27%) and limitations in shared activities (25%). Qualitative findings, supplemented by five partner perspectives (n = 5), suggested fear of rejection during early disclosure and the importance of open communication. Descriptively, women and patients with moderate to severe disease reported more difficulties.</div></div><div><h3>Conclusions</h3><div>This mixed-methods study provides novel exploratory insight into the relational impact of CHD. Disclosure and communication appear particularly challenging during relationship establishment, whereas physical limitations contribute to challenges within established relationships. These findings support integrating relational support into routine CHD care.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100710"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148951326","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}
{"title":"Right ventricular function and exercise capacity in adults with congenital heart disease: Methodological caveats that temper the clinical message","authors":"Murtaza Kamal","doi":"10.1016/j.ijcchd.2026.100706","DOIUrl":"10.1016/j.ijcchd.2026.100706","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100706"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13560021/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148946188","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}
{"title":"Feasibility and safety of a standardized low-dose thrombolytic therapy for right-sided mechanical prosthetic valve thrombosis in adult congenital heart disease","authors":"Naoto Shingu, Tomoyasu Suenaga, Ayako Ishikita, Soshun Asakawa, Masahiko Umei, Akiko Nishizaki, Takamori Kakino, Ichiro Sakamoto, Eiko Terashi, Kenichiro Yamamura, Tomoki Ushijima, Kunihiko Joo, Yusuke Ando, Hiromichi Sonoda, Akira Shiose, Kohtaro Abe","doi":"10.1016/j.ijcchd.2026.100707","DOIUrl":"10.1016/j.ijcchd.2026.100707","url":null,"abstract":"<div><h3>Background</h3><div>Mechanical valve thrombosis is a serious complication in adults with congenital heart disease (ACHD). Although low-dose thrombolytic therapy has emerged as a potential alternative to surgery, evidence in ACHD patients with right-sided mechanical valve thrombosis remains limited. We evaluated the feasibility and safety of a standardized low-dose thrombolytic protocol in this population.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed consecutive ACHD patients with right-sided mechanical valve thrombosis treated at our institution between 2022 and 2025. Diagnosis was based on fluoroscopic, echocardiographic, and computed tomography findings. All patients received a standardized low-dose alteplase regimen (25 mg over 6 h). Treatment efficacy was assessed primarily by restoration of prosthetic leaflet mobility on fluoroscopy.</div></div><div><h3>Results</h3><div>Five patients were included: four with mechanical pulmonary valves and one with a mechanical tricuspid valve. Thrombosis occurred a median of 2.3 years after valve replacement, and all patients had subtherapeutic anticoagulation (international normalized ratio <2.0) during the preceding month. At presentation, four patients were in New York Heart Association functional class II and one in class III. Following a median of 5 days of thrombolytic therapy, complete restoration of prosthetic leaflet mobility was achieved in all patients. No major bleeding or thromboembolic complications occurred. During a median follow-up of 0.7 years, no recurrent valve thrombosis was observed.</div></div><div><h3>Conclusions</h3><div>Standardized low-dose thrombolytic therapy was safe and feasible in this case series and may provide a non-surgical treatment option for ACHD patients with right-sided mechanical valve thrombosis.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100707"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148951334","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}
Andrea Elizabeth Pisesky, Colm Breatnach, Farid Foroutan, Christopher Parshuram, Alejandro Floh
{"title":"Cytokine trajectories after cardiopulmonary bypass in children: a systematic review and quantitative synthesis","authors":"Andrea Elizabeth Pisesky, Colm Breatnach, Farid Foroutan, Christopher Parshuram, Alejandro Floh","doi":"10.1016/j.ijcchd.2026.100698","DOIUrl":"10.1016/j.ijcchd.2026.100698","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"25 ","pages":"Article 100698"},"PeriodicalIF":1.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148854022","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}