CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Prognostic Value of N-Terminal Pro–B-Type Natriuretic Peptide for Risk Stratification in Adults With Fontan Palliation","authors":"Omar Abozied MBB Ch,&nbsp;William R. Miranda MD,&nbsp;Yogesh V.N. Reddy MBBS,&nbsp;Zeyad Kholeif MBB Ch,&nbsp;Heidi M. Connolly MD,&nbsp;Allan S. Jaffe MD,&nbsp;Alexander C. Egbe MD, MPH, MS","doi":"10.1016/j.cjcpc.2026.03.001","DOIUrl":"10.1016/j.cjcpc.2026.03.001","url":null,"abstract":"<div><h3>Background</h3><div>There are limited data about the prognostic role of N-terminal pro–B-type natriuretic peptide (NT-proBNP) in adults with Fontan palliation. The purpose of this study was to determine the correlates and the prognostic value of serial NT-proBNP assay for risk stratification in adults with Fontan palliation.</div></div><div><h3>Methods</h3><div>This is a retrospective cohort study of adults with Fontan palliation who had ≥1 NT-proBNP measurement in the outpatient clinic (2003-2023). In patients with ≥3 NT-proBNP measurements, serial NT-proBNP was calculated as the mean of the first 3 measurements. The study outcome was death/transplant.</div></div><div><h3>Results</h3><div>Of 369 patients (age 27 ± 8 years; 55% male) with NT-proBNP, 214 (58%) had serial measurements. The baseline level was 217 (82, 562) pg/mL, whereas the serial value was 261 (98, 715) pg/mL. The correlates of higher NT-proBNP levels were older age, atriopulmonary connection, atrial fibrillation, hepatorenal dysfunction, systemic ventricular dilation and systolic dysfunction, and higher Fontan pressures and pulmonary artery wedge pressure. Baseline and serial NT-proBNP levels were associated with death/transplant independent of anatomic indices, comorbidities, and hemodynamic indices. Serial NT-proBNP levels had superior prognostic value to single (baseline) NT-proBNP measurements, echocardiographic indices, cardiac magnetic resonance imaging indices, and cardiac catheterization indices.</div></div><div><h3>Conclusions</h3><div>These findings have important clinical implications in the management of adults with Fontan palliation because NT-proBNP assay is inexpensive, readily available, and noninvasive, and hence ideal for longitudinal assessment. Further studies are required to determine whether NT-proBNP can be used as a surrogate marker to monitor response to therapeutic interventions in this population.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 282-290"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219030","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":"The Fontan Conundrum: Rarity, Complexity, and the Research Gap","authors":"Ashish H. Shah MD, MD-Research (UK),&nbsp;Richard A. Krasuski MD,&nbsp;Jasmine Grewal BSc, MD,&nbsp;Alexander R. Opotowsky MD, MMSc,&nbsp;Sivasubramanian Ramakrishnan MD, DM","doi":"10.1016/j.cjcpc.2026.03.002","DOIUrl":"10.1016/j.cjcpc.2026.03.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 294-298"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219269","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":"Early Outcomes of Harmony Transcatheter Pulmonary Valve Implantation in the Middle East: A Single-Center Experience From Saudi Arabia","authors":"Abdullghany M. Dowaikh MD,&nbsp;Amr A. Arafat PhD,&nbsp;Sameh F. Ahmed MD,&nbsp;Ahmed A. Ismail MD,&nbsp;Atif H. Alsahari MD,&nbsp;Abdulmajeed A. Alotay MD,&nbsp;Tarek S. Momenah MD, FRCPC, FSCAI, FACC","doi":"10.1016/j.cjcpc.2026.05.001","DOIUrl":"10.1016/j.cjcpc.2026.05.001","url":null,"abstract":"<div><h3>Background</h3><div>Significant pulmonary regurgitation (PR) is a common late complication after repair of right ventricular outflow tract (RVOT) lesions, potentially leading to right ventricular failure. The Harmony transcatheter pulmonary valve (TPV) was developed for large, native, or patched RVOTs. Although multicenter studies have shown favorable results, regional single-center data, particularly from the Middle East, are scarce. This study reports our initial single-center experience with Harmony TPV implantation, focusing on procedural success, safety, and 1-year outcomes.</div></div><div><h3>Methods</h3><div>We conducted a retrospective analysis of 10 consecutive patients who underwent Harmony TPV implantation at our institution. Data were collected on baseline characteristics, procedural details, and outcomes. Procedural success, complications, and hemodynamic performance were assessed.</div></div><div><h3>Results</h3><div>The median age of our population was 15.5 years, and 70% of patients were male. The most common underlying diagnosis was post–tetralogy of Fallot repair (50%). All patients had severe PR at baseline. Technical success was achieved in all patients. There was no mortality. Minor periprocedural complications occurred in 4 patients, including minor femoral vein avulsion, fever, ecchymosis, and frequent premature ventricular contractions, all managed conservatively. At 1-year follow-up, freedom from death, moderate/severe PR, mean RVOT gradient &gt;40 mm Hg, or reintervention was 100%. No endocarditis, valve thrombosis, or stent fracture was observed.</div></div><div><h3>Conclusions</h3><div>This single-center experience from the Middle East demonstrates that Harmony TPV implantation is a feasible and safe procedure with excellent early clinical and echocardiographic outcomes. These findings are consistent with previously published international series and support the adoption of this technology in specialized centers for managing severe PR in patients with complex RVOT anatomy.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 335-340"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219078","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":"Assessment of the Variability of an Echo-Doppler Method for Measurement of Aortic Pulse Wave Velocity","authors":"Raffaele J. Massarotto MSc,&nbsp;Timothy J. Bradley MBChB,&nbsp;James E. Potts PhD,&nbsp;Kandice Mah MD,&nbsp;George G. Sandor MBChB,&nbsp;Anita T. Cote PhD","doi":"10.1016/j.cjcpc.2026.05.002","DOIUrl":"10.1016/j.cjcpc.2026.05.002","url":null,"abstract":"<div><h3>Background</h3><div>Pulse wave velocity (PWV) is an indirect measure of elastic arterial stiffness. Although various methods have been established to measure PWV, the Echo-Doppler method for measuring thoracic aortic PWV (a-PWV) is a highly available and cost-effective technique within the cardiology clinic. The purpose of this study was to assess the variability of a-PWV using the Echo-Doppler method and identify the optimal number of measurements providing the least variability of a-PWV in paediatric patients and healthy controls.</div></div><div><h3>Methods</h3><div>Eighteen patients with a dilated proximal aorta and 21 healthy controls with a normal-sized proximal aorta (10-18 years) were evaluated. Imaging was performed in 4 studies across 2 visits. Variability comparisons were performed for short-term, medium-term, intra-reviewer, inter-reviewer, and inter-sonographer variability and analysed via intraclass correlation coefficient (ICC) and Bland-Altman plots.</div></div><div><h3>Results</h3><div>The ICCs were highest for 10 beats. The combined group ICCs for the variability comparisons for 10 beats were short-term (0.743), medium-term (0.723) intra-reviewer (0.978), inter-reviewer (0.729), and inter-sonographer (0.823). Bland-Altman plots showed good agreement for all variability comparisons.</div></div><div><h3>Conclusions</h3><div>This study has established that the measurement over 10 beats produces the lowest variability and is recommended when assessing a-PWV in both patients with a dilated proximal aorta and controls. Further, reproducibility remains high, even when accounting for sources of measurement variation, such as differences between sonographers, reviewers, and study visits.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 351-358"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219081","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":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(26)00130-2","DOIUrl":"10.1016/S2772-8129(26)00130-2","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219250","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":"In Memoriam: Dominique Vervoort, MD, MPH, MBA (1994-2026)","authors":"Maral Ouzounian MD, PhD,&nbsp;Anna Dare MBChB, PhD,&nbsp;Terrence M. Yau MD, MSc,&nbsp;Stephen E. Fremes MD, MSc","doi":"10.1016/j.cjcpc.2026.04.004","DOIUrl":"10.1016/j.cjcpc.2026.04.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 279-281"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219262","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":"Acute Kawasaki Disease With Concomitant Adenovirus Infection","authors":"Brian W. McCrindle MD, MPH,&nbsp;Rayan Rahman,&nbsp;Dominic Gangemi,&nbsp;Pedrom Farid BMSc,&nbsp;Nina Butris MSc,&nbsp;Nita Chahal PhD,&nbsp;Sunita O’Shea MA,&nbsp;Tanveer Collins MD,&nbsp;Kyle Runeckles MSc,&nbsp;Vitor Guerra MD,&nbsp;Cedric Manlhiot PhD","doi":"10.1016/j.cjcpc.2026.05.004","DOIUrl":"10.1016/j.cjcpc.2026.05.004","url":null,"abstract":"<div><h3>Background</h3><div>Adenovirus infection is the most common condition in the differential diagnosis of acute Kawasaki disease (KD), but it may also occur as a concomitant infection, the relevance of which has been incompletely defined.</div></div><div><h3>Methods</h3><div>A prospectively maintained, single-center database of all presenting patients with acute KD was queried. Patients with laboratory evidence of adenovirus infection were compared with patients with no laboratory evidence of any concomitant infection of any type. We sought to determine differences in demographics, clinical and laboratory features, treatment received, and coronary artery (CA) outcomes.</div></div><div><h3>Results</h3><div>From 1997 to 2023, a total of 1442 patients who had no laboratory evidence of concomitant infection and 39 patients positive for adenovirus were compared. There were no differences regarding demographics and few differences regarding clinical features at presentation, with 72% in each group meeting guideline criteria for a diagnosis of complete KD. Patients positive for adenovirus were less likely to have rash (69% vs 88%; <em>P</em> &lt; 0.001), were more likely to have respiratory (61% vs 20%; <em>P</em> &lt; 0.001) and gastrointestinal symptoms (47% vs 27%; <em>P</em> = 0.007), and had less severe laboratory abnormalities. Adenovirus positivity was not associated with an increased risk of resistance to intravenous immunoglobulin. CA involvement was not observed in any patient in the adenovirus group (<em>P</em> = 0.049).</div></div><div><h3>Conclusions</h3><div>The presence of positive evidence of adenovirus in the setting of acute KD does not appear to confound the diagnosis of KD, the response to which should be prompt initiation of immunomodulatory treatment. CA outcomes appear to be excellent.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 326-331"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219076","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":"Systematic Review and Meta-regression of Longitudinal Outcomes of Pulmonary Valve Replacement in Tetralogy of Fallot","authors":"Louis-Olivier Roy MSc,&nbsp;Laurence Watelle MD, MSc,&nbsp;Pier-Ann Champagne MD,&nbsp;Samuel Blais MD, PhD,&nbsp;Frédéric Dallaire MD, PhD","doi":"10.1016/j.cjcpc.2026.03.004","DOIUrl":"10.1016/j.cjcpc.2026.03.004","url":null,"abstract":"<div><h3>Background</h3><div>Despite excellent survival after tetralogy of Fallot repair, pulmonary regurgitation (PR) is common. Pulmonary valve replacement (PVR) is frequently performed, but its long-term efficacy remains uncertain. We conducted a meta-regression to assess the impact of PVR on key cardiac outcomes over time.</div></div><div><h3>Methods</h3><div>We included studies reporting PR, right ventricular (RV) and left ventricular volumes, QRS duration, RV ejection fraction, peak VO₂, or New York Heart Association class before and after PVR in patients with tetralogy of Fallot. A multilevel random-effects meta-regression was performed using time since PVR as a covariate. Cubic splines were used to explore temporal trends and meta-analysis to quantify the impact of PVR over time.</div></div><div><h3>Results</h3><div>Sixty-nine studies were included. Compared with pre-PVR values, PVR yielded substantial absolute reductions in PR fraction and RV end-systolic and end-diastolic volume. Left ventricular end-diastolic volume increased slightly. New York Heart Association class also improved. The median follow-up was 14.4 months (interquartile range: 10.3-31.1 months) with only 1 study extending beyond 10 years. Estimated annual changes after PVR were minimal for all parameters and negligible for key parameters such as PR fraction (0.36%, 95% confidence interval [CI]: –0.05% to 0.77%), RV end-systolic volume (–0.58 mL/m², 95% CI: –1.62 mL/m² to 0.45 mL/m²), and RV end-diastolic volume (–0.49 mL/m², 95% CI: –0.72 mL/m² to 1.72 mL/m²).</div></div><div><h3>Conclusions</h3><div>The long-term impact of PVR remains uncertain. Although short-term improvements are clear, insufficient long-term data preclude firm conclusions on sustained benefits.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 299-315"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219075","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":"Exercise Limitations in Fontan Circulation: A Systematic Review and Meta-analysis of Peak Oxygen Uptake","authors":"Nathan R. Weeldreyer PhD,&nbsp;Amanda Shewman BKin,&nbsp;Marae Waldron BKin,&nbsp;Dean Hewitt MSc,&nbsp;Veronika Schmid MSc,&nbsp;Christopher Weinkauf BKin,&nbsp;Joseph Pagano MD,&nbsp;Corey R. Tomczak PhD,&nbsp;David Niederseer MD, PhD,&nbsp;Jan Vontobel MD,&nbsp;Siddhartha S. Angadi PhD,&nbsp;Stephen J. Foulkes PhD,&nbsp;Mark J. Haykowsky PhD","doi":"10.1016/j.cjcpc.2026.04.003","DOIUrl":"10.1016/j.cjcpc.2026.04.003","url":null,"abstract":"<div><h3>Background</h3><div>The degree to which individuals with Fontan circulation (FC) experience exercise limitations and the underlying physiological mechanisms remains unclear. Thus, we sought to quantify the magnitude of peak oxygen consumption (<span><math><mrow><mover><mi>V</mi><mo>˙</mo></mover></mrow></math></span>O₂peak) impairment and its determinants in individuals with FC and age-matched healthy controls (CON).</div></div><div><h3>Methods</h3><div>PubMed, Web of Science, and Scopus were searched from January 1980 through July 2025. Studies were included if they reported <span><math><mrow><mover><mi>V</mi><mo>˙</mo></mover></mrow></math></span>O₂peak or its Fick determinants derived from a cardiopulmonary exercise test and compared FC with CON. Random-effects models were used for pooled data analysis, and meta-regressions were used to explore key moderators of age and time after Fontan surgery.</div></div><div><h3>Results</h3><div>Pooled analysis demonstrated significantly reduced <span><math><mrow><mover><mi>V</mi><mo>˙</mo></mover></mrow></math></span>O₂peak in FC vs CON (19 studies, n = 893; weighted mean difference: 17.2 mL/kg/min, 95% confidence interval: –20.9 to –14.3 mL/kg/min). Meta-regressions revealed that age significantly moderated the weighted mean difference for relative <span><math><mrow><mover><mi>V</mi><mo>˙</mo></mover></mrow></math></span>O₂peak (β = –5.77 mL/kg/min; 95% confidence interval: –11.27 to –0.27; <em>P</em> = 0.041). Peak exercise oxygen pulse, heart rate, systolic blood pressure, and arterial oxygen saturation were significantly lower in FC compared with CON, whereas peak exercise stroke volume did not differ significantly between the groups.</div></div><div><h3>Conclusions</h3><div>Exercise capacity is markedly impaired in individuals with FC due to central and peripheral limitations, and the magnitude of this decline accelerates with time after surgery. These findings highlight the importance of early identification and targeted (exercise) intervention to mitigate long-term functional decline.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 316-325"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219123","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":"Adenovirus and Kawasaki Disease: A Diagnostic Challenge","authors":"Nadine F. Choueiter MD","doi":"10.1016/j.cjcpc.2026.06.003","DOIUrl":"10.1016/j.cjcpc.2026.06.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 5","pages":"Pages 332-334"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219077","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}