Annals of Pediatric Cardiology最新文献

{"title":"Embryological determinants of right ventricular morphogenesis in pulmonary atresia: The role of bipartite and tripartite configurations.","authors":"Ghassan Alnaami","doi":"10.4103/apc.apc_288_25","DOIUrl":"10.4103/apc.apc_288_25","url":null,"abstract":"<p><p>Pulmonary atresia (PA) is a complex congenital heart defect with marked morphological and clinical heterogeneity. Central to this variability is the embryological development of the right ventricle (RV) and its subcomponents. This review explores the embryological basis of RV development, emphasizing the bipartite and tripartite configurations and their clinical relevance in PA. Recent advances in human embryology, including high-resolution reconstructions from the Human Developmental Biology Resource and the work of Hikspoors <i>et al.</i>, provide an updated framework for understanding RV morphogenesis. We highlight how the disruption of ventricular partitioning, mural hypertrophy, and proximal and distal outflow malalignment contribute to the phenotypic spectrum of PA. Finally, we examine the impact of RV morphology on surgical strategy and long-term prognosis. Understanding PA as a failure of stage-specific RV morphogenesis provides a developmental basis for its clinical and surgical diversity.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"312-316"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399870/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583339","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":"Hospital course and duration of oxygen supplementation in children with unoperated intracardiac posttricuspid left-to-right shunts: A prospective observational study.","authors":"Nimisha Sharma, Sakshi Sachdeva, Dinesh Kumar, Lokesh Sharma, Munish Guleria, Hema Gupta Mittal, Dheeraj Deo Bhatt","doi":"10.4103/apc.apc_113_25","DOIUrl":"10.4103/apc.apc_113_25","url":null,"abstract":"<p><strong>Background: </strong>Children with left-to-right (L->R) shunts often need hospital admission and oxygen supplementation due to intercurrent respiratory infection and worsening heart failure. The duration of oxygen requirement in these children is often unknown. This study was done to determine the hospital course of children with unoperated posttricuspid L->R shunts admitted to a hospital with a need for supplemental oxygen. The primary objective was to find the proportion of children requiring prolonged oxygen supplementation (>1 month).</p><p><strong>Methods: </strong>In this prospective observational study, 122 children with a median (interquartile range) age of 5 (3-8.75) months, having a hemodynamically significant posttricuspid L->R shunt and a need for supplemental oxygen, were enrolled. N terminal B type natriuretic peptide (NT pro-BNP) and C-reactive protein were measured at baseline, 2-, and 3-weeks postenrollment. Decongestive measures, antibiotics, and supportive measures were given, and patients were followed up during their hospitalization.</p><p><strong>Results: </strong>22.1% patients died, and 12.3% had prolonged oxygen requirement. 65.5% of admissions and 66.6% of deaths occurred between September and December. At the time of enrollment, NT pro-BNP above age-related cut-off was found in < 20% of patients. On multivariate analysis, the need for mechanical ventilation at admission and the need for escalation of mode of oxygen delivery were independently associated with mortality.</p><p><strong>Conclusion: </strong>Children with hemodynamically significant posttricuspid L->R shunts admitted to a hospital with a need for supplemental oxygen have an unfavorable short-term outcome. A substantial proportion of children needing oxygen supplementation succumbed. Seasonal variation in admission and mortality was seen. NT pro-BNP was elevated in a minority of patients. These findings may have important clinical implications in the management.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"296-302"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399888/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583266","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":"How to reduce the risk of inadvertent aortic root puncture during transseptal puncture?","authors":"Jyothi Vijay, Narayanan Namboodiri","doi":"10.4103/apc.apc_47_26","DOIUrl":"10.4103/apc.apc_47_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"359-360"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583309","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":"Healthcare utilization trends for adolescents and young adults with moderate and severe congenital heart disease.","authors":"Victoria H Yatsenko, Sarah A Mathena, Jennifer G Andrews, Brent J Barber, Andrew W Hoyer, Kenneth A Fox, Omar Meziab, Claudia E Guerrero, Scott E Klewer, Michael D Seckeler","doi":"10.4103/apc.apc_63_26","DOIUrl":"10.4103/apc.apc_63_26","url":null,"abstract":"<p><strong>Background: </strong>Improved congenital heart disease (CHD) care allows >90% of patients to live into adulthood. Young adult CHD patients experience challenges managing their cardiac conditions and other comorbidities that may increase healthcare utilization. There are limited data on hospitalizations of adolescents and young adults with CHD, specifically during the transition from pediatric to adult care. We hypothesized increased healthcare utilization for CHD patients transitioning from adolescent to adult care.</p><p><strong>Materials and methods: </strong>Retrospective review of the Vizient^® Clinical Data Base (national, administrative database) for admissions from October 2019 to December 2023, aged 10-29 years with International Classification of Diseases-10 codes for moderate or severe CHD, stratified into age categories. Data included demographics, hospital outcomes, insurance status, admission through the emergency department (ED), and costs. Comparisons were made between the age groups.</p><p><strong>Results: </strong>There were 16,284 admissions during the study period. As age increased, the proportion of female admissions rose, with a higher prevalence of white admissions and a lower prevalence of Hispanic admissions. There was a decline in public insurance coverage, while rates of uninsured status and ED admissions increased with age. Mortality rates were highest at 15-19 years and 25-29 years.</p><p><strong>Conclusions: </strong>Despite increased admissions from the ED, complications, and mortality, healthcare costs declined between the younger and older age groups. There were higher rates of uninsured status and ED admissions for hospitalized young adults with CHD transitioning from pediatric to adult care. However, overall hospital costs decreased. More studies are needed to guide improvements in care during this at-risk time.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"278-281"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399902/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583329","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":"Trans-2,3-enoyl-CoA reductase-like-related catecholaminergic polymorphic ventricular tachycardia in a child: Clinical course and management outcomes.","authors":"E K Kulbachinskaya, A A Pushkov, V V Bereznitskaya, I S Zhanin, K V Savostyanov, I A Kovalev","doi":"10.4103/apc.apc_66_26","DOIUrl":"10.4103/apc.apc_66_26","url":null,"abstract":"<p><p>Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but potentially fatal inherited arrhythmia. We report a 7-year-old child with CPVT associated with a homozygous trans-2,3-enoyl-CoA reductase-like c.730 + 1G>C variant. To our knowledge, this variant has not been previously reported in the indexed peer-reviewed literature. Prophylactic implantation of an implantable cardioverter defibrillator before symptom onset proved lifesaving during a subsequent ventricular fibrillation episode despite beta-blocker therapy.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"333-336"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399883/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583435","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":"First palliation for the fragile: Short-term outcomes of ductal stenting versus surgical shunt in small infants with restricted pulmonary blood flow.","authors":"Olfi Lelya, Alesia Prillya Mauna, Michael Sugiyanto, Sisca Natalia Siagian, Yovi Kurniawati, Oktavia Lilyasari, Aditya Agita Sembiring, Damba Dwisepto Aulia Sakti, Indriwanto Sakidjan Atmosudigdo, Rissa Ummy Setiani, Radityo Prakoso","doi":"10.4103/apc.apc_42_26","DOIUrl":"10.4103/apc.apc_42_26","url":null,"abstract":"<p><strong>Background: </strong>In congenital heart disease (CHD) with restricted pulmonary blood flow, infants require urgent palliation, either with a modified Blalock-Taussig-Thomas (m-BTT) shunt or ductal stenting. However, evidence in infants weighing under 5 kg remains limited. This study aims to compare short-term outcomes after the two approaches in infants weighing <5 kg.</p><p><strong>Methods: </strong>This retrospective cohort study included infants weighing under 5 kg diagnosed with CHD and restricted pulmonary blood flow who underwent either ductal stenting or m-BTT shunt between 2018 and 2023 at the National Cardiovascular Centre Harapan Kita. Outcome assessment included mortality, reintervention, rehospitalization, complications, intensive care unit (ICU) stay, and total hospital duration within 30 days.</p><p><strong>Results: </strong>After the 1:1 propensity score matching, we analyzed 106 infants, comprising 53 infants (mean weight: 3525.7 ± 682.0 g) who underwent ductal stenting and 53 infants (mean: weight 3700.9 ± 649.0 g) who received m-BTT shunt surgery. Mortality (18.9% vs. 22.6%; <i>P</i> = 0.63), reintervention (1.9% vs. 7.5%; <i>P</i> = 0.36), and complication (54.7% vs. 71.7%; <i>P</i> = 0.07) rates were not significantly different between the two groups within 30 days. However, rehospitalization rates (3.8% vs. 15.1%; <i>P</i> = 0.046) were significantly smaller in the ductal stenting group. Furthermore, ductal stenting was associated with significantly shorter durations of both in total hospitalization (8 [1-42] vs. 12 [0-75] days; <i>P</i> < 0.001) and in intensive care stay (2 [0-19] vs. 6 [1-37] days; <i>P</i> < 0.001).</p><p><strong>Conclusions: </strong>Both ductal stenting and m-BTT shunt demonstrated comparable short-term efficacy in palliating cyanosis among infants weighing <5 kg, with reasonable periprocedural safety in both the groups. Ductal stenting was associated with shorter total and ICU length of stay as well as lower rehospitalization rates, suggesting potential recovery advantages, though prospective studies are needed to guide individualized decision-making.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"288-295"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583296","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":"Reappraising the role of procalcitonin in predicting intravenous immunoglobulin resistance in Kawasaki disease.","authors":"Rajkumar Kundavaram, Amber Kumar","doi":"10.4103/apc.apc_73_26","DOIUrl":"10.4103/apc.apc_73_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"355"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399897/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583272","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":"Transposition of great arteries with left ventricular outflow tract obstruction undergoing definitive repair beyond the third decade.","authors":"Mayank Yadav, Vuribindi Manognitha Reddy, Nita Bharti, Srinivas Thimmasarthi, Parag Gharde","doi":"10.4103/apc.apc_3_26","DOIUrl":"10.4103/apc.apc_3_26","url":null,"abstract":"<p><p>A 30-year-old male presented with progressive dyspnea, cyanosis, and chest pain due to unrepaired d-transposition of the great arteries with ventricular septal defect (VSD) and severe left ventricular outflow tract obstruction, underwent successful late biventricular repair including VSD closure, neo-aortic mechanical valve implantation, coronary transfer, and LeCompte maneuver. Despite postoperative stroke and complete heart block requiring pacemaker implantation, he was discharged in stable condition with preserved biventricular function. This case supports the feasibility of anatomic repair beyond the third decade.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"337-341"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399876/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583349","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":"Bile acid dysregulation in Fontan circulation: A Parallel to hyperglycemia in diabetes?","authors":"Ashish H Shah","doi":"10.4103/apc.apc_37_26","DOIUrl":"10.4103/apc.apc_37_26","url":null,"abstract":"<p><p>Survivors of the Fontan circulation increasingly develop late multisystem complications, including liver disease, cardiac dysfunction, and frailty, despite stable hemodynamics. Emerging metabolomic data demonstrate chronically elevated circulating bile acids in Fontan patients, implicating a previously underrecognized metabolic contributor to disease progression. Drawing parallels with diabetes mellitus, in which chronic hyperglycemia drives silent organ injury through metabolic memory, we propose that persistently elevated bile acids act as a similar pathogenic driver in the Fontan circulation. Bile acid-mediated mitochondrial dysfunction, inflammation, and fibrosis may imprint early cellular injury that manifests clinically years later. This framework highlights the potential importance of early bile acid surveillance and timely metabolic intervention to prevent irreversible end-organ damage and improve long-term outcomes in Fontan patients.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"307-311"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399905/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583102","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 cardiac rehabilitation in pediatric fenestrated Fontan completion: A case report despite postoperative complication.","authors":"Roberta da Silva Teixeira, Luciana S M Luz, Helica L G M Lopes, Karyne K Bernandes, Fellipe A M Silva","doi":"10.4103/apc.apc_81_26","DOIUrl":"10.4103/apc.apc_81_26","url":null,"abstract":"<p><p>Early rehabilitation after Fontan surgery in children is poorly described, especially in complicated postoperative courses. We report the clinical evolution and rehabilitation of an 8-year-old girl with double-inlet left ventricle and patent ductus arteriosus who underwent fenestrated Fontan completion. The postoperative period was complicated by bilateral pleural effusions, pulmonary hypertension, hematemesis, psychomotor alteration, and mechanical ventilation with inhaled nitric oxide. A structured bedside rehabilitation program was started early and adjusted according to clinical stability. The program included respiratory exercises, inspiratory muscle training, resistance exercises, cycle ergometry, and gait training. We observed progressive improvement in mobility, respiratory autonomy, and functional capacity. She was discharged from the pediatric cardiac intensive care unit on postoperative day 23, having demonstrated functional recovery. This was evidenced by a reduction in the Functional Status Scale score from 14 to 9. Early individualized rehabilitation after Fontan surgery was feasible and associated with meaningful functional improvement.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 3","pages":"320-323"},"PeriodicalIF":0.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399881/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148583303","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}