Annals of Pediatric Cardiology最新文献

{"title":"The geography of congenital heart disease care: Is the world flat?","authors":"Debasis Das, Krishna Subramony Iyer","doi":"10.4103/apc.apc_353_26","DOIUrl":"https://doi.org/10.4103/apc.apc_353_26","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"463-465"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102843","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":"Large left ventricular apical aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy.","authors":"Athira Mohanachandran, Puthiyedath Thejaswi, Kothandam Sivakumar, Ravi Agarwal","doi":"10.4103/apc.apc_163_26","DOIUrl":"https://doi.org/10.4103/apc.apc_163_26","url":null,"abstract":"<p><p>Noonan syndrome (NS) is a multisystem disorder with frequent heart defects, including hypertrophic cardiomyopathy (HCM). Left ventricular (LV) apical aneurysm is an extremely rare presentation in pediatric HCM. A 4-year-old child with NS, PTPN-11 mutation and HCM presented with progressive mid-cavity LV obstruction leading to a large LV apical aneurysm. Magnetic resonance imaging confirmed a large, wide-neck LV apical aneurysm. Surgery combined septal myectomy and patch repair of the aneurysm, resulting in uneventful recovery and significant gradient reduction. While multimodal imaging assists surgical planning, longitudinal surveillance should look for recurrences of aneurysm from the repaired segment, arrhythmia, and myocardial function.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"602-607"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149103184","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":"Pulmonary venous Doppler as a marker of restrictive atrial physiology in fetal critical aortic stenosis after fetal valvuloplasty.","authors":"Nageswara Rao Koneti, Shweta Nathani Bakhru","doi":"10.4103/apc.apc_111_26","DOIUrl":"https://doi.org/10.4103/apc.apc_111_26","url":null,"abstract":"<p><p>Fetal balloon aortic valvuloplasty (fBAV) is performed in carefully selected fetuses with critical aortic stenosis to prevent progression to hypoplastic left heart syndrome. We report a fetus with critical aortic stenosis who underwent successful fBAV at 26 weeks' gestation with restoration of antegrade aortic flow. Serial fetal echocardiography subsequently demonstrated progressive restriction and eventual functional closure of the foramen ovale despite technically successful valvuloplasty. Pulmonary venous Doppler revealed a prominent atrial reversal wave with prolonged reversal duration, while mitral inflow showed marked E-wave predominance, indicating elevated left atrial pressure and severe left ventricular diastolic dysfunction. After birth, the neonate developed pulmonary hypertension physiology with persistently elevated left ventricular end-diastolic pressure despite postnatal balloon aortic valvotomy. This case highlights that pulmonary venous Doppler provides a valuable noninvasive marker of restrictive atrial physiology and may identify fetuses at risk of adverse neonatal hemodynamics despite successful relief of aortic valve obstruction.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"612-615"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626611/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149103154","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":"Transcatheter closure of giant right coronary artery fistula with Amplatzer vascular plug II in neonate.","authors":"Jumpei Oiwa, Tao Fujioka, Akio Inage","doi":"10.4103/apc.apc_25_26","DOIUrl":"https://doi.org/10.4103/apc.apc_25_26","url":null,"abstract":"<p><p>Congenital coronary artery fistula (CAF) is a rare anomaly, accounting for approximately 0.4% of congenital heart defects. It is characterized by an abnormal connection between a coronary artery and one of the cardiac chambers or great vessels. Depending on the degree of shunting and coronary steal, it can lead to severe congestive heart failure, even in the neonatal or early infantile period, sometimes requiring early intervention. We report a neonatal case of heart failure caused by a giant right CAF that was successfully treated with transcatheter closure using an Amplatzer™ Vascular Plug II. Transcatheter closure of CAF in the neonatal period may serve as a minimally invasive and safe therapeutic option.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"593-596"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626612/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102969","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":"Ultrasound predictors of extubation outcomes in pediatric cardiac surgery: A prospective evaluation of lung, diaphragmatic, and cardiac ultrasound.","authors":"Rajesh Madavathazathil Gopalakrishnan, Rohini Dhar, Sandra Chaithanyam Bijukumar, Georg Gutjahr, A R Meghalakshmi","doi":"10.4103/apc.apc_175_26","DOIUrl":"https://doi.org/10.4103/apc.apc_175_26","url":null,"abstract":"<p><strong>Backgrounds: </strong>Extubation failure after pediatric cardiac surgery increases morbidity and intensive care unit (ICU) stay. Conventional indices such as rapid shallow breathing index (RSBI) and PaO2/FiO2 do not account for multi-organ contributors such as cardiac function, pulmonary aeration, and diaphragmatic performance. This study prospectively evaluated bedside ultrasound parameters - lung ultrasound score (LUS), mean diaphragmatic excursion (DE), and E/e' ratio - as predictors of extubation outcomes.</p><p><strong>Methods: </strong>In this prospective, observational study conducted in a tertiary pediatric cardiac ICU, 102 postoperative children undergoing elective extubation were evaluated using ultrasound at three standardized timepoints (M1: SIMV mode, M2: CPAP/PS mode, and M3: NIV/room air). The primary outcome was extubation failure, defined as reintubation within 48 h. Diagnostic accuracy was analyzed using receiver operating characteristic curves and Youden index-derived thresholds. The secondary outcomes included associations with ventilation duration, ICU stay, incremental value over conventional indices (PaO2/FiO2 and RSBI), and timepoint dynamics.</p><p><strong>Results: </strong>Extubation failure occurred in 11 (10.8%) patients. In SIMV mode (M1), the optimal thresholds were LUS ≥12 (area under the curve [AUC] 0.69, sensitivity 63.6%, specificity 64.8%), mean DE ≤0.82 cm (AUC 0.45, specificity 89%), and E/e' ≥8.9 (AUC 0.38). LUS ≥12 trended toward longer mechanical ventilation and ICU stay but did not reach statistical significance. Adding LUS to conventional indices showed a nonsignificant increase in discrimination from AUC 0.66 to 0.71 (<i>P</i> = 0.145). At later timepoints, LUS performance improved (AUC 0.72 at M3), while DE and E/e' remained poor discriminators. Inter-observer reproducibility for DE was moderate (intra-class correlation coefficients 0.50-0.61).</p><p><strong>Conclusions: </strong>In pediatric cardiac surgical patients, LUS demonstrated a moderate predictive ability for extubation outcomes, whereas diaphragmatic and diastolic parameters were weak predictors. The addition of LUS to conventional indices provided incremental value, supporting its role as a useful bedside adjunct in evaluating extubation readiness.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"534-541"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626614/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102976","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":"Analysis of various electrocardiographic and echocardiographic parameters in beta thalassemia patients with iron overload.","authors":"Haritha Girija, Anuradha Sridhar, Chinnasamy Sivaprakasam Muthukumaran, S Ramkishore, Rajaguru Ganesan, V Mahitha, V Arun","doi":"10.4103/apc.apc_133_25","DOIUrl":"https://doi.org/10.4103/apc.apc_133_25","url":null,"abstract":"<p><strong>Background: </strong>Cardiac magnetic resonance imaging (MRI) T2* is the gold standard investigation for predicting cardiac iron overload. Being costly and resource-limited, other investigations that are cost-effective and easily available are needed.</p><p><strong>Materials and methods: </strong>We studied various electrocardiographic and echocardiographic parameters of transfusion-dependent thalassemia patients between 2022 and 2024 in our tertiary pediatric cardiac center in southern India.</p><p><strong>Results: </strong>Among our 50 patients of the age group 8 years-30 years (mean age group: 19.9 years ± 5.57), 62% of patients were males. Ninety-eight percent of our patients were asymptomatic, though 32% had MRI evidence of severe iron overload. Twenty-two percent of them had ferritin of more than 5000 µg/dL. There was a significant difference in <i>P</i> wave dispersion, mitral valve E/A, and E/e' seen among patients with normal and abnormal cardiac MRI.</p><p><strong>Conclusions: </strong>Regular follow-up with tracking of the electrocardiographic and echocardiographic parameters can be used as an effective screening to guide timing for cardiac MRI and titrate chelation therapy. Early identification of iron overload, prompt initiation of chelation therapy, and ensuring adequate compliance with medical treatment, along with titration of chelation based on serial assessments, play a major role in improving the quality of life and lifespan for these patients.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"490-496"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149103015","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":"Evidence-based treatment strategies for hypoplastic left heart syndrome: The role of EFNA1/EphA.","authors":"Sophie G Davis, Xian Wu, Leslie Kennedy, Lisandra de Castro Braz, Yaser Azizi, Jitka Ismail Virag","doi":"10.4103/apc.apc_121_26","DOIUrl":"https://doi.org/10.4103/apc.apc_121_26","url":null,"abstract":"<p><p>Cardiovascular health among the US general population remains largely suboptimal. Major contributing factors include diet, physical activity, and sleep, and these influences begin early in life, despite broad access to health information regarding healthy choices and access to medical care. Advances in perinatal care allow earlier detection of both maternal and congenital conditions, enabling interventions and planning that can improve outcomes. Research exploring the foundations of hypoplastic left heart syndrome (HLHS) has significantly expanded in the last 25 years. HLHS represents a prototypical complex congenital heart defect arising from multifactorial perturbations in left-sided cardiac development, with converging contributions from genetic susceptibility, dysregulated signaling pathways, and environmental modifiers. Early diagnosis, now largely achieved through fetal and postnatal echocardiography, enables timely identification of ductal-dependent systemic circulation and facilitates coordinated perinatal management strategies. Contemporary management remains rooted in staged single-ventricle palliation or cardiac transplantation, supported by intensive perioperative care aimed at maintaining systemic perfusion and improving survival, albeit with significant long-term morbidity. Increasingly, advances in genomics and molecular cardiology are redefining the field, with patient-specific cellular models and pathway-targeted approaches offering the potential to uncover disease mechanisms and develop novel, mechanism-based therapies that move beyond surgical palliation. This review summarizes current knowledge on the etiology of HLHS, current treatments, and emerging therapeutic strategies, with emphasis on the potential for modulation of EFNA/EphA signaling to influence multiple aspects of disease progression.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"542-552"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149103123","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":"Subclinical cardiac dysfunction in transfusion-dependent beta-thalassemia children, an experience from central India: A comparative cross-sectional study.","authors":"Rajkumar Motiram Meshram, Sagar Chopde, Ashma Rashid","doi":"10.4103/apc.apc_126_26","DOIUrl":"https://doi.org/10.4103/apc.apc_126_26","url":null,"abstract":"<p><strong>Background: </strong>Cardiac complications are the leading cause of mortality in children with transfusion-dependent beta-thalassemia (TDT). Tissue Doppler imaging (TDI) is a readily available, noninvasive technique used to detect subclinical cardiac dysfunction by deriving the Tei index.</p><p><strong>Objective: </strong>The objective of this study was to determine subclinical cardiac dysfunction in children with TDT and correlate findings with serum ferritin levels.</p><p><strong>Materials and methods: </strong>This comparative cross-sectional study was conducted at a tertiary care institute in Central India. The study included 50 children with TDT (aged 2-18 years) as cases and 50 age-and sex-matched outpatient children with minor illnesses as controls, selected via simple random sampling over 1 year. Demographic, clinical, transfusion, and anthropometric data were collected. Hematological and biochemical investigations, along with electrocardiography, were performed. Conventional two dimensional echocardiography and TDI were conducted using an ALPINION 5 color Doppler system. M-mode, B-mode, and Doppler parameters were measured per American Society of Echocardiography guidelines. Statistical analysis utilized Chi-square, independent <i>t</i>-tests, and Pearson correlation coefficients (<i>P</i> < 0.05 was considered significant).</p><p><strong>Results: </strong>Demographic variables were comparable between groups. However, the body mass index was significantly lower, and systolic blood pressure was significantly higher in cases than in controls. While 36% of cases were sero-reactive to Hepatitis C, all participants were negative for hepatitis B surface antigen and human immunodeficiency virus. Hemoglobin was significantly lower, whereas serum ferritin and cardiothoracic ratios were significantly higher in cases. Conventional echocardiography revealed significantly larger left ventricular internal diameter at end-diastole, mitral annular plane systolic excursion, and left atrial diameters in cases. Notably, both left and right ventricular Tei indices were significantly higher in cases compared to controls (<i>P</i> < 0.0001). Serum ferritin (<i>r</i> = 0.32) and systolic blood pressure (<i>r</i> = 0.29) had significant positive correlation with left ventricular Tei index.</p><p><strong>Conclusion: </strong>Children with TDT exhibit latent systolic and diastolic dysfunction as evidenced by TDI. This highlights the critical importance of identifying asymptomatic cardiac dysfunction in children and adolescents to prevent cardiac-related mortality.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"513-519"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626624/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102889","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":"Trends in pediatric rheumatic heart disease in the Indonesia-Malaysia-Thailand Growth Triangle economic sub-region (Indonesia, Malaysia, and Thailand), 1990-2023: A Global burden of disease study.","authors":"Muhammad Iqhrammullah","doi":"10.4103/apc.apc_69_26","DOIUrl":"https://doi.org/10.4103/apc.apc_69_26","url":null,"abstract":"<p><strong>Background: </strong>The extent to which socio-demographic development translates into reductions in rheumatic heart disease (RHD) burden in the Indonesia-Malaysia-Thailand Growth Triangle (IMT-GT) Economic Sub-Region have not been assessed. This study aimed to assess temporal trends, socio-demographic gradients, and development-adjusted performance of pediatric RHD from 1990 to 2023.</p><p><strong>Methods: </strong>Data from the Global Burden of Disease Study 2023 for Indonesia, Malaysia, and Thailand were analyzed. Age-specific incidence, mortality, and disability-adjusted life years (DALYs) were estimated by the sex. Temporal trends were assessed using generalized additive models to estimate average annual percentage changes (AAPC). The association between DALY rates and the socio-demographic index (SDI) was evaluated using elasticity analysis and performance relative to SDI-based expectations was quantified as the gap between observed and expected DALY rates.</p><p><strong>Results: </strong>Between 1990 and 2023, the burden of RHD declined across the IMT-GT sub-region, with substantial cross-country variation. AAPCs indicated modest declines in DALYs and mortality in all countries, whereas incidence showed no consistent long-term decrease. Higher SDI was significantly associated with lower DALY rates across all pediatric age groups (P < 0.001). SDI-benchmarked comparisons indicate that Indonesia has not achieved the level of DALY reduction expected for its development status, whereas Thailand has exceeded these expectations and Malaysia has remained on the predicted trajectory.</p><p><strong>Conclusions: </strong>The burden of pediatric RHD has declined in the IMT-GT Economic Sub-region over the past three decades, driven primarily by reductions in mortality. Sociodemographic development was strongly associated with lower DALY rates, but substantial cross-country differences in development-adjusted performance persist.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"466-474"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102992","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":"When coronaries speak last; atypical Kawasaki disease beyond the classical criteria - A four-case series.","authors":"Arife Sancaktar, Filiz Ekici, Firat Kardelen, Mehmet Uzunoglu, Yunus Emre Gürhopur","doi":"10.4103/apc.apc_90_26","DOIUrl":"https://doi.org/10.4103/apc.apc_90_26","url":null,"abstract":"<p><p>Coronary artery aneurysms (CAAs) are the most serious complication of Kawasaki disease (KD). Delayed diagnosis is associated with an increased risk of CAA, particularly in atypical presentations lacking classic mucocutaneous features. We report four children diagnosed with atypical KD only after coronary abnormalities were detected on echocardiography. Initial presentations included acalculous cholecystitis with marked transaminase elevation, aseptic meningitis, and an inflammatory arthritis. None of the cases had fulfilled complete diagnostic criteria at admission. Coronary imaging showed single- or multi-segment aneurysms ranging from small to giant (maximum <i>Z</i>-score +11.7). All received intravenous immunoglobulin and high-dose acetylsalicylic acid; corticosteroids, anticoagulation, or biologic therapy were added as treatment in cases with giant CAA. CAA persisted in patients with giant aneurysms. Early echocardiography is essential to establish the diagnosis in atypical KD and it may prevent irreversible coronary complications.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 5","pages":"581-584"},"PeriodicalIF":0.8,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13626619/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149102947","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}