Annals of Pediatric Cardiology最新文献

{"title":"Trans-catheter closure of large peri-membranous ventricular septal defect with multiple exits using two occluder devices.","authors":"Asit Das, Nur Nabab Mollah, Shubham Tewary, Gaurav Lakhani, Priya Jena","doi":"10.4103/apc.apc_314_25","DOIUrl":"10.4103/apc.apc_314_25","url":null,"abstract":"<p><p>Aneurysmal peri-membranous ventricular septal defects (PM-VSD) may show multiple right ventricular exits separated by tricuspid chordae or trabeculations. Transcatheter PM-VSD closure is increasingly performed as a surgical alternative. An 8-year-old girl with a large PM-VSD, with moderate pulmonary hypertension, presented with dyspnea and recurrent respiratory infections. The defect measured 12 mm with two exits. Closure was successfully achieved using two devices through an antegrade approach with two arteriovenous loops. There was no residual shunt, aortic regurgitation, or conduction block on follow-up. Transcatheter closure of PM-VSDs with multiple exits using two devices is feasible and safe in selected patients.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"241-243"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331513/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389862","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":"Prenatal diagnosis of congenitally calcified tricuspid valve with postnatal follow-up and outcomes.","authors":"Amit Kumar Pandey, Prerna Bhat, Brijesh Kottyail, Indu Nair, Raman Krishna Kumar, Balu Vaidyanathan","doi":"10.4103/apc.apc_368_25","DOIUrl":"10.4103/apc.apc_368_25","url":null,"abstract":"<p><p>Fetal echocardiography demonstrated a hyperechogenic lesion (possibly calcification) obstructing the tricuspid inflow along with hypoplasia of the right ventricle (RV) and duct-dependent pulmonary circulation. Postnatal inflow along with hypoplasia of the right ventricle (RV) and duct-dependent pulmonary circulation. Postnatal surgical exploration, only minimal antegrade flow was established. Histopathology confirmed degenerative valvar tissue with dense calcification. Eventually, due to persistent inflow restriction and inadequate RV growth, the infant underwent single-ventricle palliation (bidirectional Glenn shunt) with uneventful follow-up after the procedure. This case highlights the extreme rarity of congenital valvular calcification, its possible developmental and genetic associations, and the hemodynamic significance of inflow obstruction in causing ventricular hypoplasia.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"234-237"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331505/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389773","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":"Protein-losing enteropathy after the Fontan procedure - A cardiologist's and gastroenterologist's perspective.","authors":"Natalia Nawara-Węgrzyn, Łukasz Cichy, Kinga Kowalska-Duplaga, Aleksandra Morka","doi":"10.4103/apc.apc_254_25","DOIUrl":"10.4103/apc.apc_254_25","url":null,"abstract":"<p><p>Protein-losing enteropathy (PLE) is a severe, multifactorial complication of Fontan circulation that affects approximately 12% of patients with single-ventricle physiology. Because no universal standard therapy exists, management is individualized and guided by the dominant hemodynamic and lymphatic drivers, clinical severity, and local expertise. Chronically elevated central venous pressure and impaired lymphatic drainage promote lymph congestion and leakage into the intestinal lumen, leading to hypoalbuminemia, edema, diarrhea, malnutrition, and immune dysfunction. Treatment is multimodal and includes optimization of Fontan hemodynamics, symptomatic and anti-inflammatory pharmacotherapy, and targeted nutritional strategies (high-protein diet, medium-chain triglycerides, and supplementation). Advances in lymphatic imaging have enabled phenotype-based, lymphatic-directed interventions such as lymphatic embolization and thoracic duct decompression, which can improve outcomes in selected patients. When conservative and interventional strategies fail, heart transplantation remains the definitive option. Emerging evidence also highlights the potential contribution of the gut-liver axis, including intestinal barrier dysfunction and alterations in the microbiome, which may influence inflammation and disease persistence. This review summarizes current concepts in PLE pathophysiology and therapeutic approaches, with emphasis on lymphatic dysfunction and evolving adjunctive targets.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"198-204"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331498/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389747","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 response to - \"Ethical dilemmas in pediatric cardiac interventions: <i>Primum non nocere</i>\".","authors":"Anand P Subramanian","doi":"10.4103/apc.apc_74_26","DOIUrl":"10.4103/apc.apc_74_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"262-263"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331497/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389789","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":"Double trouble in tetralogy: Rare sequential pseudoaneurysms managed by distinct surgical and endovascular approaches.","authors":"Juhi Agarwal, Richa Jayesh Kothari, Vimal Raj, Dheeraj Kumar Tiwari, Sudesh Prabhu","doi":"10.4103/apc.apc_274_25","DOIUrl":"10.4103/apc.apc_274_25","url":null,"abstract":"<p><p>Pseudoaneurysm formation following a modified Blalock-Taussig-Thomas (BTT) shunt in tetralogy of Fallot (TOF) is rare, most commonly involving the subclavian artery; pulmonary artery (PA) pseudoaneurysms are even less frequent. We report an infant initially presenting with cyanosis and diagnosed with TOF, who underwent a right modified BTT shunt. She later developed hemoptysis, and imaging revealed a subclavian artery pseudoaneurysm at the shunt anastomosis, managed surgically. One year later, recurrent hemoptysis led to the diagnosis of a PA pseudoaneurysm at the distal end of the prior shunt. This was successfully treated using a novel endovascular technique adapted from aortic aneurysm repair. This rare case of sequential pseudoaneurysms - subclavian and pulmonary - highlights the importance of maintaining a high index of suspicion, utilizing early cross-sectional imaging, and applying tailored surgical or endovascular approaches to manage complex vascular complications in children with prior shunt surgeries.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"227-230"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331510/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389918","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 circulation: Beyond hemodynamics to a unified disease paradigm.","authors":"Debasis Das","doi":"10.4103/apc.apc_173_26","DOIUrl":"10.4103/apc.apc_173_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"115-117"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331518/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389907","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":"Asymmetric calcification of the extracardiac Fontan conduit: Quantitative insights from long-term computed tomography imaging.","authors":"Yuchen Cao, Masaaki Koide, Masafumi Yashima, Aya Miyazaki","doi":"10.4103/apc.apc_114_26","DOIUrl":"10.4103/apc.apc_114_26","url":null,"abstract":"<p><p>The long-term structural integrity of expanded polytetrafluoroethylene (ePTFE) conduits used for extracardiac total cavopulmonary connection (EC-TCPC) is not well established. Although conduit calcification has been reported, quantitative data remain limited. We retrospectively analyzed 20 EC-TCPC patients who underwent computed tomography more than 5 years after Fontan completion (median: 13.7 years). Cross-sectional area (CSA) was measured at five sites and expressed as a percentage of the original CSA (%CSA), and calcification volume was indexed to body surface area. Median values of mean and minimum %CSA were 74.0% and 65.5%, respectively. %CSA correlated inversely with calcification volume index (ρ = -0.47, <i>P</i> = 0.04). Calcification was greater along the lesser curvature than the greater curvature (0.66 vs. 0.20 mm³/m², <i>P</i> < 0.001). These findings suggest curvature-specific vulnerability in ePTFE conduits and support the importance of long-term structural surveillance in Fontan patients.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"193-197"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331499/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389869","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":"Advances and unmet needs in pharmacologic therapy for pediatric heart failure: Insights from the 2025 International Society for Heart and Lung Transplantation Guidelines.","authors":"Bibhuti B Das","doi":"10.4103/apc.apc_85_26","DOIUrl":"10.4103/apc.apc_85_26","url":null,"abstract":"<p><strong>Backgrounds: </strong>Pediatric heart failure (PHF), most commonly resulting from cardiomyopathies, congenital heart disease, or acquired cardiac disorders, remains a major cause of childhood morbidity and mortality. The 2025 International Society for Heart and Lung Transplantation (ISHLT) guidelines provide the most comprehensive update in over a decade, offering a contemporary framework for pharmacologic management when underscoring persistent gaps in pediatric-specific evidence.</p><p><strong>Methods: </strong>This narrative review synthesizes the 2025 ISHLT Guidelines alongside contemporary pediatric and adult HF guidance, emphasizing pharmacologic recommendations, areas of extrapolation from adult trials, and persistent evidence gaps across diverse PHF phenotypes.</p><p><strong>Results: </strong>The updated guideline introduces an ejection fraction (EF)-based classification system and refines pharmacologic recommendations for acute and chronic PHF. It reaffirms Class I support for foundational neurohormonal therapies in PHF with reduced EF, incorporates randomized control trial-based recommendations for sacubitril-valsartan and ivabradine, and offers limited, extrapolated recommendations for agents such as sodium-glucose cotransporter-2 inhibitors, mineralocorticosteroid receptor antagonists, and soluble guanylate cyclase stimulators. The guideline also outlines phenotype-specific considerations for high-risk groups, including congenital heart disease and cancer therapy-related cardiomyopathy. Persistent unmet needs include the absence of disease-modifying therapies for PHF with preserved EF and insufficient evidence to guide management of complex congenital and syndromic conditions.</p><p><strong>Conclusion: </strong>The 2025 ISHLT Guidelines provide a structured, contemporary approach to pharmacologic therapy in PHF when clearly delineating areas where evidence remains limited. Addressing these limitations will require coordinated multicenter research, real-world data integration, and learning health systems capable of translating emerging therapies into evidence-based pediatric practice.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"118-138"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331508/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389885","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":"Emerging therapeutic strategies for the failing Fontan circulation.","authors":"Ashish H Shah","doi":"10.4103/apc.apc_296_25","DOIUrl":"10.4103/apc.apc_296_25","url":null,"abstract":"<p><p>Patients with Fontan circulation face progressive multisystem dysfunction driven by venous hypertension, lymphatic failure, and metabolic derangements. This state-of-the-art review synthesizes evidence across hemodynamic, lymphatic, pulmonary vascular, and metabolic domains to outline emerging therapies and a pragmatic care framework. We summarize data on exercise rehabilitation; pulmonary vasodilators; lymphatic imaging-guided interventions; metabolic modulation including bile acid sequestration and SGLT2 inhibition; arrhythmia prevention; mechanical circulatory support; and transplantation, including combined heart-liver strategies. Recognizing limitations of conventional randomized trials in this heterogeneous, small population, we propose adaptive, registry-embedded, and platform designs with harmonized phenotypes and core outcomes. This review aims to bridge mechanistic insight and clinical application, offering clinicians a concise roadmap and researchers a set of testable hypotheses to improve survival, function, and quality of life.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"205-214"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331517/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389897","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":"Bacteremia caused by <i>Neisseria macacae</i> in the long-term postoperative period following the Rastelli procedure.","authors":"Tsubasa Shimozono, Daisuke Hazeki, Yuichi Kodama, Yuki Ogata, Tomoyuki Matsuba, Toshiro Fukushige","doi":"10.4103/apc.apc_30_26","DOIUrl":"10.4103/apc.apc_30_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"260-261"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331516/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389852","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}