CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Physician Perception of Fontan Failure, “Acceptable” Hemodynamics, Assessment, and Indications for Intervention—Results of a Multinational Survey","authors":"Ashish H. Shah MD, MD-Res, FRCP ,&nbsp;Evan J. Wiens MD, MSc, FRCPC ,&nbsp;Jonathan Windram MD, FRCP ,&nbsp;Shakeel A. Qureshi MBChB, FRCP, FRCPCH ,&nbsp;Petra Jenkins FRCP ,&nbsp;Isma Rafiq MBBS, MRCP, MD-Res ,&nbsp;Erwin Oechslin MD, FESC, FRCPC, DRCPSC ,&nbsp;Richard A. Krasuski MD","doi":"10.1016/j.cjcpc.2025.02.004","DOIUrl":"10.1016/j.cjcpc.2025.02.004","url":null,"abstract":"<div><h3>Background</h3><div>Patients with complex congenital heart disease unfit for biventricular repair are often palliated with Fontan circulation (FC), which obviates the need for a subpulmonary ventricle. This approach has led to high survival rates, with over 80% of patients expected to live beyond 30 years. Despite increasing patient numbers and existing guidelines, there are no standardized hemodynamic parameters defining Fontan failure, resulting in considerable variability in management practices. This pilot project aimed to assess real-world practices in FC patient management, particularly in surveillance, defining Fontan circulatory failure, and determining treatment thresholds.</div></div><div><h3>Methods</h3><div>A cross-sectional survey with 10 multiple-choice questions and optional free-form responses was distributed globally to congenital heart disease cardiologists through 2 academic centers. Conducted from January to December 2021, the survey targeted clinicians managing adult FC patients.</div></div><div><h3>Results</h3><div>Of 310 invited cardiologists, 170 (55%) participated, including 27% from the United States, 22% from Canada, and 20% from the United Kingdom. Respondents included pediatric (37%), adult congenital (47%), and dual trained adult/pediatric cardiologists (14%), mostly in academic settings (94%). Variability existed in defining FC failure, with markers such as protein-losing enteropathy (74%), fatigue/dyspnea (62%), and elevated Fontan pressure (58%) commonly cited. Responses differed on defining elevated Fontan pressure, with 53% selecting &gt;15 mm Hg, 33% &gt;18 mm Hg, and 14% &gt;20 mm Hg. Ninety-one percent prescribed pulmonary vasodilators, though indications and thresholds varied.</div></div><div><h3>Conclusion</h3><div>Substantial heterogeneity in FC patient management currently exists, underscoring the need for standardized hemodynamic parameters.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 198-202"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896550","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":"Diastolic Function and Left Atrial Mechanics in Children With Marfan and Loeys-Dietz Syndrome","authors":"Nairy Khodabakhshian BSc, PhD(c) ,&nbsp;Alison J. Howell MD ,&nbsp;Wei Hui ,&nbsp;Luc L. Mertens MD, PhD ,&nbsp;Vitor C. Guerra MD, PhD","doi":"10.1016/j.cjcpc.2025.03.002","DOIUrl":"10.1016/j.cjcpc.2025.03.002","url":null,"abstract":"<div><h3>Background</h3><div>Diastolic function in patients with pediatric Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS) remains underexplored. Conventional diastolic assessments in this population can be challenging, and speckle-tracking echocardiography (STE)-derived left atrial (LA) strain measurements offer a novel and sensitive approach.</div></div><div><h3>Methods</h3><div>This retrospective, observational study included 37 patients with MFS, 37 patients with LDS, and 30 age-matched controls. Comprehensive echocardiographic evaluations were performed to assess diastolic function. STE-based LA strain measurements during the reservoir, conduit, and pump phases were obtained and analyzed.</div></div><div><h3>Results</h3><div>Demographics were mostly similar between groups. Patients with MFS and LDS vs controls exhibited lower LA reservoir strain (42.3% vs 32.5% in MFS, <em>P</em> &lt; 0.001; 42.3% vs 34.5% in LDS, <em>P</em> = 0.004) and LA conduit strain (34.2% vs 24.7% in MFS, <em>P</em> &lt; 0.001; 32.5% vs 26.2% in LDS, <em>P</em> = 0.004). LA pump strain remained similar between groups. In addition, conventional diastolic parameters differed significantly between MFS, LDS, and controls.</div></div><div><h3>Conclusions</h3><div>Pediatric patients with MFS and LDS exhibit lower LA reservoir and conduit strains, along with differences in early diastolic parameters, while maintaining preserved LA pump function. These findings suggest subclinical changes in early diastolic function. STE-derived LA strain offers a sensitive, noninvasive method for detecting subtle functional impairments that may precede clinical dysfunction, underscoring its diagnostic potential for early monitoring and management of at-risk patients.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 179-188"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896577","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":"Complex Considerations for a Complex Disease: Identifying Poor Prognostic Factors in Ebstein’s Anomaly","authors":"Yalin Lin MDCM, MSc ,&nbsp;Varsha Thakur MD, MSc, MBA","doi":"10.1016/j.cjcpc.2025.05.006","DOIUrl":"10.1016/j.cjcpc.2025.05.006","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 213-216"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896551","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":"Painting of 3D Models of Congenital Heart Disease as an Educational Tool for Pediatric Residents","authors":"Beshoy Botros MD ,&nbsp;Jared A. Sheridan MD, FRCPC ,&nbsp;George Slim MD, FRCPC ,&nbsp;Jessica L. Foulds MD, FRCPC ,&nbsp;Marisha McClean MBBS, DM (Paeds) ,&nbsp;Carolina A. Escudero MD, MSc, FRCPC","doi":"10.1016/j.cjcpc.2025.03.003","DOIUrl":"10.1016/j.cjcpc.2025.03.003","url":null,"abstract":"<div><h3>Background</h3><div>Three-dimensional (3D) models of congenital heart diseases have been used to teach cardiac anatomy to learners. Our team sought to determine whether the painting of 3D models could help pediatric residents better understand both the anatomy and physiology of complex congenital heart diseases.</div></div><div><h3>Methods</h3><div>This was a prospective assessment of pediatric resident perceptions regarding a novel teaching method on the 3 stages of palliation for hypoplastic left heart syndrome. After attending a didactic session about the topic, they were provided with 3D models representing each stage. They were guided in painting them to represent the presumed oxygen saturations of each model’s chambers. Questionnaires were used to assess the self-perceived understanding of the anatomy, pathophysiology, and management from before to after the session using 5-point Likert scales. Statistical analysis was performed using a paired samples <em>t</em> test.</div></div><div><h3>Results</h3><div>There were 36 pediatric residents from 2 institutions. There was an increase in the mean self-perceived understanding of the anatomy (2.14 vs 3.94, <em>P</em> &lt; 0.001), pathophysiology (2.14 vs 3.72, <em>P</em> &lt; 0.001), and management of single ventricle palliation (2.19 vs 3.78, <em>P</em> &lt; 0.001) before and after the session. All 36 participants enjoyed the session and wanted to participate in future sessions.</div></div><div><h3>Conclusion</h3><div>Painting of 3D models increased knowledge acquisition among pediatric residents.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 218-225"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896573","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":"Pulmonary Artery Remodelling Assessed by Four-Dimensional Flow Magnetic Resonance Imaging in Pulmonary Arterial Hypertension and Atrial Septal Defect","authors":"Estibaliz Valdeolmillos MD ,&nbsp;Emmanuelle Fournier MD ,&nbsp;Hichem Sakhi MD ,&nbsp;Paul Vignaud MD ,&nbsp;Marion Audié MD, MSc ,&nbsp;Marc-Antoine Isorni MD ,&nbsp;Bastien Provost MD ,&nbsp;Florence Lecerf PhD student ,&nbsp;Clément Batteux MD, PhD ,&nbsp;Grégoire Albenque MD ,&nbsp;Olivier Sitbon MD, PhD ,&nbsp;David Montani MD, PhD ,&nbsp;Xavier Jais MD, PhD ,&nbsp;Laurent Savale MD, PhD ,&nbsp;Marc Humbert MD, PhD ,&nbsp;Arshid Azarine MD ,&nbsp;Sébastien Hascoët MD, PhD, FESC","doi":"10.1016/j.cjcpc.2025.03.004","DOIUrl":"10.1016/j.cjcpc.2025.03.004","url":null,"abstract":"<div><h3>Background</h3><div>Pulmonary artery (PA) stiffness plays a significant role on right ventricular (RV) function, as evidenced in pulmonary arterial hypertension (PAH). In patients with atrial septal defect–related PAH (ASD-PAH), prognosis is dependent on RV function. We aimed to characterize PA remodelling and its relationship with RV function using 4-dimensional flow magnetic resonance imaging (4D flow MRI) in patients with ASD-PAH.</div></div><div><h3>Methods</h3><div>We prospectively included 24 adults with ASD-PAH. They underwent 4D flow MRI and right heart catheterization within 24-48 hours. Remodelling of PA was analysed (stiffness, compliance, distensibility, elastance, and pulsatility) and PA vortex was evaluated. RV adaptation was measured by RV ejection fraction and RV-PA coupling.</div></div><div><h3>Results</h3><div>The median age was 50 [41-55] years, and the median mean PA pressure and pulmonary vascular resistance were 50 [39-58] mm Hg and 8.0 [6.8-11.3] WU, respectively. Fourteen patients (58.3%) had RV dysfunction (RV ejection fraction &lt;45%). All presented PA dilation (median main PA diameter: 42 [37-48] mm) and an increase in PA stiffness (10 [7.4-15.3]), whereas compliance and distensibility were decreased (2.1 [1.8-4.1] mm²/mm Hg and 0.19 [0.13-0.29] %/mm Hg). No correlation was found between PA dilation and indexes of PA remodelling. PA remodelling parameters were more severely impaired in patients with preserved RV function, with an increased stiffness (<em>P</em> = 0.01) and a decreased compliance and distensibility (<em>P</em> = 0.02).</div></div><div><h3>Conclusions</h3><div>PA stiffness parameters characterized by 4D flow MRI were impaired in the population with ASD-PAH, and PA remodelling appears to be influenced by volumetric overload from the left-to-right shunt through the ASD.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 189-197"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896622","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 Emotional Heart of Telehealth: Rethinking Psychosocial Care for Congenital Heart Disease","authors":"Alina Yang","doi":"10.1016/j.cjcpc.2025.06.002","DOIUrl":"10.1016/j.cjcpc.2025.06.002","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Page 217"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896552","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":"A Novel Puncture Method Combining Intracardiac Echocardiography and a 3D Mapping System for Catheter Ablation After a Lateral Tunnel Fontan Operation","authors":"Kota Nagaoka MD ,&nbsp;Taisuke Nabeshima MD ,&nbsp;Hitoshi Mori MD, PhD ,&nbsp;Takuro Kojima MD, PhD ,&nbsp;Ritsushi Kato MD, PhD ,&nbsp;Naokata Sumitomo MD, PhD","doi":"10.1016/j.cjcpc.2025.02.003","DOIUrl":"10.1016/j.cjcpc.2025.02.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 231-234"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896575","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 and Imaging Predictors of Poor Prognosis in Ebstein Anomaly","authors":"Nabil Dib MD, PhD ,&nbsp;Laura Vidales MD ,&nbsp;Juva Benseba MD ,&nbsp;Paul Khairy MD, PhD ,&nbsp;François-Pierre Mongeon MD, SM ,&nbsp;Annie Dore MD ,&nbsp;Nancy Poirier MD ,&nbsp;Blandine Mondésert MD ,&nbsp;Stéphanie Tan MD ,&nbsp;Marie-A. Chaix MD, PhD","doi":"10.1016/j.cjcpc.2025.04.002","DOIUrl":"10.1016/j.cjcpc.2025.04.002","url":null,"abstract":"<div><div>Ebstein anomaly is a rare congenital heart condition characterized by the partial or complete absence of tricuspid valve delamination, a variable degree of “atrialization” of the right ventricle, and a redundant anterior leaflet with fenestrations. Tricuspid valve repair or replacement is often necessary to address tricuspid valve regurgitation, yet the timing remains controversial. Early intervention risks multiple reoperations, whereas disease progression may cancel the benefits of a delayed intervention. Clinical, biological, imaging, and electrocardiographic markers of poor prognosis may help determine when to intervene. Transthoracic echocardiography and cardiac magnetic resonance imaging are imaging modalities of choice to detect prognostic markers in the context of severe tricuspid regurgitation. This review summarizes established and emerging prognostic factors that may help to individualize the timing and planning of surgery for patients with Ebstein anomaly.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 203-212"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896623","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":"Capturing Physiological Data in Children With Heart Failure Using Wearable Digital Technology: Lessons From Pilot Project","authors":"David M. Peng MD ,&nbsp;Jonathan B. Edelson MD ,&nbsp;Harishwara R. Gureddygari MS ,&nbsp;Toni Duganiero MPA ,&nbsp;Aine Lynch MBBCh ,&nbsp;Melissa McQueen BS ,&nbsp;Joseph Hillenburg ,&nbsp;E. Kevin Hall MD ,&nbsp;Lauren Smyth MHA ,&nbsp;David N. Rosenthal MD ,&nbsp;Angela Lorts MD","doi":"10.1016/j.cjcpc.2025.03.007","DOIUrl":"10.1016/j.cjcpc.2025.03.007","url":null,"abstract":"<div><div>We conducted a proof-of-concept pilot project to evaluate the feasibility of collecting and describing physiological and activity data from pediatric patients with heart failure using an Apple Watch and a specially designed app. Five patients (12-17 years old) with significant heart failure, including 2 patients with left ventricular assist device, were enrolled. Heart rate and activity data from each patient were obtained and presented along with corresponding clinical status and trajectory. We believe that sharing these early, detailed data can help generate hypotheses and spur further study of this growing technology and its applicability in pediatric conditions including, but not limited to, heart failure.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 226-230"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896574","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":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(25)00082-X","DOIUrl":"10.1016/S2772-8129(25)00082-X","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144896576","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}