CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(26)00058-8","DOIUrl":"10.1016/S2772-8129(26)00058-8","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Page A1"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148558998","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 Practice of Extubation Readiness Testing in Pediatric Cardiac Intensive Care Units: A Cross-sectional Study Based on an Electronic Survey","authors":"Gul Sher MD, MBA,&nbsp;Mehdi Hedjazi Moghari PhD,&nbsp;Kamil Abbas BS,&nbsp;Margaret Mathewson MD,&nbsp;Nan Garber MD,&nbsp;Veronica Godsey MD,&nbsp;Jai Parkash Udassi MD","doi":"10.1016/j.cjcpc.2025.10.003","DOIUrl":"10.1016/j.cjcpc.2025.10.003","url":null,"abstract":"<div><h3>Background</h3><div>Prolonged postoperative mechanical ventilation in children with congenital heart disease is associated with increased morbidity. Although early extubation may reduce complications, the risk of extubation failure requires reliable assessment before extubation. This study explores current periextubation practices in pediatric cardiac intensive care units.</div></div><div><h3>Methods</h3><div>A cross-sectional survey was conducted among pediatric cardiac intensive care unit providers globally in a virtual environment, using email.</div></div><div><h3>Results</h3><div>A total of 169 providers responded with a response rate of 16.7% (169 of 1009), predominantly from North America (110 of 134, 82%) and primarily physicians (127 of 167, 76%). Only 22.3% (37 of 166) consistently use a validated sedation scale, whereas 39.8% (66 of 166) did not use one. Tracheal secretions were assessed by 90.4% (151 of 167), with 60% (100 of 166) delaying extubation due to significant secretions. An air leak test was routinely performed by 75.4% (126 of 167), and 78.7% (129 of 164) administered dexamethasone when leak was absent. A spontaneous breathing trial was regularly performed by 43.7% (73 of 167), most using continuous positive airway pressure with pressure support (137 of 166, 82.5%), typically once daily for 30-60 minutes. High-flow nasal cannula was the most commonly used noninvasive support method (147 of 160, 91.9%). Most providers defined extubation failure as reintubation within 24 or 48 hours. Providers were more likely to report a spontaneous breathing trial if they worked in transplant centers or if the patients had single-ventricle physiology.</div></div><div><h3>Conclusions</h3><div>This study highlights notable differences in how extubation readiness is assessed and managed across institutions. Although guidelines exist and multiple collaborative efforts have been undertaken to standardize extubation readiness testing protocols, notable variability remains. The impact of this variability on clinical outcomes warrants further investigation.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 129-135"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343410/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414544","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":"Functional Pulmonary Atresia With Overlapping Dilated, Hypertrophic, and Excessive Trabeculation Cardiomyopathy in a Neonate With a TPM1 Mutation","authors":"Eva Correia PhD,&nbsp;Anaïs Medouni BSc,&nbsp;Robert Tuta DEC,&nbsp;Chloe Wanert MD,&nbsp;Marie-Josée Raboisson MD,&nbsp;Gregor Andelfinger MD","doi":"10.1016/j.cjcpc.2025.11.003","DOIUrl":"10.1016/j.cjcpc.2025.11.003","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 189-191"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343417/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414627","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":"Trigger Criteria for Advance-Care Planning in ACHD: The Time Is Now","authors":"Michelle Keir MD, MSc,&nbsp;Jennifer Ann Sullivan RN, BScN","doi":"10.1016/j.cjcpc.2025.10.007","DOIUrl":"10.1016/j.cjcpc.2025.10.007","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 179-180"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343409/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413818","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":"Physiological-Based Cord Clamping and Early Oxygenation in Newborns With Transposition of the Great Arteries: A Quality Improvement Study Protocol","authors":"Jesse A. Weeda MD,&nbsp;Arjan B. te Pas MD, PhD,&nbsp;Zsuzsanna Nagy MD,&nbsp;Gábor H. Kovács MD,&nbsp;Miklós Szabó MD, PhD,&nbsp;Gergő Leipold MD, PhD,&nbsp;Nariae Baik-Schneditz MD, PhD,&nbsp;Bernhard Schwaberger MD, PhD,&nbsp;Christian Heiring MD, PhD,&nbsp;Emma Louise Malchau Carlsen MD, PhD,&nbsp;Monique C. Haak MD, PhD,&nbsp;Nico A. Blom MD, PhD,&nbsp;Stuart B. Hooper PhD,&nbsp;Janneke Dekker PhD,&nbsp;Roel L.F. van der Palen MD, PhD","doi":"10.1016/j.cjcpc.2025.10.005","DOIUrl":"10.1016/j.cjcpc.2025.10.005","url":null,"abstract":"<div><div>At birth, major circulatory and pulmonary adaptations are required for a successful foetal-to-neonatal transition. In newborns with transposition of the great arteries (TGA), this transition is often impaired, leading to severe hypoxemia. This may also result in persistent pulmonary hypertension of the newborn (PPHN), worsening hypoxemia, and increasing the risk of urgent invasive interventions after birth. Physiological-based cord clamping (PBCC), delaying cord clamping until after lung aeration and ventilation are established, promotes a more stable circulatory transition and has shown benefits in both preterm and term neonates. PBCC may also provide advantages in infants with congenital heart disease. In TGA, combining PBCC with early supplemental oxygen may reduce the incidence and severity of PPHN, decrease related complications, and minimize the need for invasive interventions. To evaluate the feasibility, safety, and clinical outcomes of the stabilisation approach, we have initiated a stepwise quality improvement initiative comprising 2 sequential studies. Study phase 1, a single-centre study at Leiden University Medical Centre will assess feasibility, protocol adherence, and safety. Study phase 2, an observational cohort study across multiple European centres will evaluate clinical outcomes, focusing on incidence and severity of PPHN and the need for urgent interventions. All TGA newborns will be stabilised with an intact umbilical cord while receiving 2 L/min nasal high flow (fraction of inspired oxygen 1.0) as supplemental oxygen. Cord clamping will occur once the infant is considered stable, defined as a heart rate &gt;100 bpm and preductal SpO₂ &gt;75% with supplemental oxygen. This study will inform guidelines for delivery room management and early preoperative care in TGA newborns.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 136-144"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343422/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414637","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":"De Novo Heart Failure in a 32-Year-Old Man From Congenital Ostial Left Main Coronary Atresia","authors":"Gary Wang MD,&nbsp;Taylor Petropoulos MD,&nbsp;Osami Honjo MD, PhD,&nbsp;Rafael Alonso-Gonzalez MSc, MD,&nbsp;Adriana Luk MD,&nbsp;Filio Billia MD, PhD","doi":"10.1016/j.cjcpc.2025.12.001","DOIUrl":"10.1016/j.cjcpc.2025.12.001","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 185-188"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343408/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414577","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":"Trigger Criteria for Advance Care Planning in Adults With Congenital Heart Disease: A Systematic Review","authors":"Mathias Possner MD,&nbsp;Fabienne Schwitz MD,&nbsp;Niculin Zach,&nbsp;Tanya Karrer MAS,&nbsp;James Tam MD,&nbsp;Erwin Oechslin MD,&nbsp;Isabelle Vonder Muhll MD,&nbsp;Kayla Wolofsky MD,&nbsp;David Birnie MBChB, MD,&nbsp;Luc Beauchesne MD,&nbsp;Andrew S. Mackie MD,&nbsp;Markus Schwerzmann MD","doi":"10.1016/j.cjcpc.2025.09.002","DOIUrl":"10.1016/j.cjcpc.2025.09.002","url":null,"abstract":"<div><h3>Background</h3><div>Adults with congenital heart disease (CHD) face increased risks of premature morbidity and mortality. Advance care planning (ACP) facilitates longitudinal, patient-centered care but remains underused in this population, partly due to the lack of validated trigger criteria to initiate ACP. This study aimed to systematically review the literature to identify proposed triggers to initiate ACP conversations in adults with CHD.</div></div><div><h3>Methods</h3><div>We conducted a systematic search of MEDLINE, Embase, PsycInfo, CINAHL, Web of Science, Scopus, and the Cochrane Library from database inception to July 26, 2024. The search focused on 2 core concepts: CHD and adults, and ACP or palliative care. Two reviewers independently screened articles for inclusion and extracted potential trigger criteria for ACP initiation.</div></div><div><h3>Results</h3><div>Of 4257 articles screened, 21 were included for data extraction. Nearly half (48%) were published between 2020 and 2024, with most (81%) appearing in cardiology journals. ACP was most often recommended during routine follow-up (15 articles, 71%), independent of defect complexity or functional status. Specific interventions (10 articles, 48%) and signs of disease progression (10 articles, 48%) were the second most frequently cited trigger criteria, followed by defect-specific triggers (7 articles, 33%). Symptom- and patient-based triggers were less frequently reported.</div></div><div><h3>Conclusions</h3><div>This review highlights a wide range of proposed triggers and a lack of consensus on when to initiate ACP in adults with CHD. The identified triggers can inform clinical practice and serve as a foundation for developing standardized criteria. However, findings are limited by the heterogeneity among the included studies.</div></div><div><h3>Registration</h3><div>PROSPERO CRD42024597771.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 3","pages":"Pages 169-178"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343426/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414632","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":"Long-term Outcomes in Anomalous Left Coronary Artery From the Pulmonary Artery Syndrome: A Multicenter Half-Century Experience","authors":"Sean D. Smith MD ,&nbsp;Eiad Habib MBBS ,&nbsp;Tala B. Shahin MD ,&nbsp;Amani Elshaer MBBS ,&nbsp;Claire Yee PhD ,&nbsp;Elaina A. Blickenstaff PhD ,&nbsp;Francois Marcotte MD ,&nbsp;Alexander Egbe MD ,&nbsp;Heidi M. Connolly MD ,&nbsp;Joseph Dearani MD ,&nbsp;Hartzell V. Schaff MD ,&nbsp;David S. Majdalany MD","doi":"10.1016/j.cjcpc.2025.07.005","DOIUrl":"10.1016/j.cjcpc.2025.07.005","url":null,"abstract":"<div><h3>Background</h3><div>Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a congenital anomaly that presents in infancy or rarely in adulthood and generally requires surgical correction. Data on long-term sequelae are minimal.</div></div><div><h3>Methods</h3><div>All patients with ALCAPA seen within our hospital system from 1965 to 2022 were reviewed. Patients with other structural heart diseases were excluded.</div></div><div><h3>Results</h3><div>Thirty-one patients were identified, with 9 patients (29.0%) being diagnosed as adults. The cohort was divided into pediatric (&lt;18 years) and adult (≥18 years) subgroups. Heart failure was the most common presentation in pediatric patients (36.4%), whereas dyspnea and chest pain were more common in adults (44.4% and 33.3%, respectively). All patients underwent surgical repair. Overall survival was 93.4% at 1 year and 5 years (95% confidence interval [CI]: 85-100) and 83.6% at 10 years (95% CI: 69.8-100). The need for reintervention was 0% at 1 year, 15.8% at 5 years (95% CI: 0-31), and 29.8% at 10 years (95% CI: 3.4-49). Preoperative ejection fraction (left ventricular ejection fraction [LVEF]) was 51% for pediatric patients and 43% for adults; follow-up LVEF was 61% among pediatric patients and 55% among adults. Significant mitral valve regurgitation (MR) was noted on the preoperative echocardiogram in 30.8% of pediatric patients and 42.9% of adults. The prevalence of MR decreased to 11.8% in pediatric patients, but it was still 40% in adults at long-term follow-up.</div></div><div><h3>Conclusion</h3><div>ALCAPA has variable presentations in childhood and adulthood. Overall survival after surgery is excellent. LVEF improved throughout the cohort and MR improved in pediatric patients. Reintervention rates increased over time.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 57-64"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727131","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":"Use of and Experience With Wearable Biosensors in Congenital Heart Disease: A Survey Study","authors":"Animesh Tandon MD, MS ,&nbsp;Paige Ewing MPH ,&nbsp;Mark Abboud BA ,&nbsp;Colin K. Drummond PhD, MBA ,&nbsp;Abigail C. Demianczyk PhD ,&nbsp;Sabahat Hizlan BA, BS ,&nbsp;Bradley S. Marino MD, MPP, MSCE, MBA ,&nbsp;Rashmi Rao MD ,&nbsp;Jennifer N. Avari Silva MD ,&nbsp;MaryBeth Mercer MPH","doi":"10.1016/j.cjcpc.2025.10.002","DOIUrl":"10.1016/j.cjcpc.2025.10.002","url":null,"abstract":"<div><div>Wearable biosensors (“wearables”) are increasingly used by consumers to monitor their health, but their use in congenital heart disease is understudied. This survey study aimed to define wearable use in congenital heart disease. We found that patients and parents tended to buy wearables because they expected good utility, that they were generally happy with the experience of using the wearables, and that those who did not purchase wearables did not expect to get useful data. These findings suggest that utility may be a key driver of patient uptake of wearables.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Pages 75-79"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727135","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":"Partial Anomalous Pulmonary Venous Connection in Turner Syndrome: The Challenge of the Right Follow-up","authors":"Estelle Tenisch MD ,&nbsp;Tobias Rutz MD","doi":"10.1016/j.cjcpc.2025.10.009","DOIUrl":"10.1016/j.cjcpc.2025.10.009","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"5 2","pages":"Page 81"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147727061","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}