{"title":"Do we need a pediatric cardiac critical care registry in India?","authors":"Rajesh Madavathazathil Gopalakrishnan","doi":"10.4103/apc.apc_295_25","DOIUrl":"10.4103/apc.apc_295_25","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"256-257"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331509/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389855","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}
Shweta Bakhru, Nageswara Rao Koneti, V Gayatri, Sneha Santi Golla
{"title":"Prenatal therapy of everolimus for large fetal cardiac rhabdomyoma in tuberous sclerosis.","authors":"Shweta Bakhru, Nageswara Rao Koneti, V Gayatri, Sneha Santi Golla","doi":"10.4103/apc.apc_237_25","DOIUrl":"10.4103/apc.apc_237_25","url":null,"abstract":"<p><p>Fetal cardiac rhabdomyoma is the most common prenatal cardiac tumor and is frequently associated with tuberous sclerosis complex (TSC). Everolimus, a mammalian target of rapamycin inhibitor, is an established postnatal therapy for TSC-related tumors, but its prenatal use remains rare. A 27-year-old primigravida was referred at 21 weeks' gestation for evaluation of a left ventricular mass. Given the rapid, severe progression of tumor size, maternal oral everolimus therapy was initiated at 28 weeks, with dose titration to achieve drug levels of 3-8 ng/ml. At 32 weeks, the tumor size regressed significantly. Neonatal serum everolimus on day 1 was 5.2 ng/mL, confirming transplacental transfer. Therapy was resumed postnatally, and at 5 months, the tumor had completely resolved, with normal neurodevelopment and no seizures. While short-term safety and efficacy of prenatal everolimus therapy are promising, further studies are needed to establish standardized dosing, safety profiles, and long-term developmental outcomes.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"231-233"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331515/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389795","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":"A randomized controlled trial of electrical cardiometry-guided perioperative hemodynamic management in children undergoing cardiac surgery.","authors":"Rohan S Thottan, Sambhunath Das, Neeti Makhija, Suruchi Hasija, Sandeep Chauhan","doi":"10.4103/apc.apc_21_26","DOIUrl":"10.4103/apc.apc_21_26","url":null,"abstract":"<p><strong>Background and aims: </strong>Children undergoing corrective surgery for congenital heart disease (CHD) are at risk of perioperative hemodynamic instability and low cardiac output syndrome (LCOS). Conventional monitoring relies on static parameters that may inadequately reflect real-time cardiac performance. Electrical cardiometry (EC) provides continuous, noninvasive assessment of cardiac output and related indices. This study evaluated whether EC-guided perioperative management improves early postoperative outcomes compared with conventional monitoring in pediatric CHD surgery.</p><p><strong>Methods: </strong>In this prospective randomized controlled trial, 60 children (0-15 years) undergoing elective corrective CHD surgery were randomized to EC-guided management (Group 1, <i>n</i> = 30) or conventional monitoring (Group 2, <i>n</i> = 30). In Group 1, perioperative fluid and vasoactive therapy were guided by EC-derived parameters, including cardiac index (CI), stroke volume, thoracic fluid content, and stroke volume variation. Group 2 was managed using standard clinical and invasive parameters. The primary outcome was the incidence of LCOS within 48 h postoperatively. The secondary outcomes included vasoactive inotropic score (VIS), major adverse cardiac events, and in-hospital mortality.</p><p><strong>Results: </strong>Baseline characteristics were comparable. Group 1 demonstrated significantly higher mean arterial pressure (MAP) at 8, 16, and 24 h postoperatively, faster lactate clearance, and lower VIS during the early postoperative period. CI correlated positively with MAP and urine output and inversely with lactate levels. No mortality occurred.</p><p><strong>Conclusions: </strong>EC-guided perioperative management aids in achieving early postoperative hemodynamic stability and metabolic recovery in children undergoing corrective CHD surgery and represents a valuable noninvasive adjunct to conventional monitoring.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"139-146"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331503/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389872","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 effect of sildenafil on maternal mortality and fetal adverse outcomes among pregnant patients with congenital heart disease-associated pulmonary artery hypertension: A retrospective cohort study.","authors":"Nabila Sabri Nahdi, Anggoro Budi Hartopo, Dyah Wulan Anggrahini, Irwan Taufiqur Rachman, Lucia Kris Dinarti","doi":"10.4103/apc.apc_150_25","DOIUrl":"10.4103/apc.apc_150_25","url":null,"abstract":"<p><strong>Background and aim: </strong>Maternal and fetal mortality in patients with congenital heart disease-associated pulmonary artery hypertension (CHD-PAH) is reported to be quite high. Sildenafil is the standard of care in PAH. Studies on sildenafil in maternal and fetal clinical outcomes are few. This study aimed to determine the effect of oral sildenafil on the clinical outcomes of mothers and fetuses with CHD-PAH.</p><p><strong>Methods: </strong>This study was an observational study with a retrospective cohort method. Patient data were taken from the hospital registry and medical records of patients who met the research criteria from August 2015 to August 2022. Based on sildenafil treatment, subjects were divided into two groups: subjects with oral sildenafil and those without sildenafil. The research outcome was maternal mortality and a composite of fetal adverse outcomes, defined as fetal mortality, oligohydramnios, intrauterine growth retardation (IUGR), and small for gestational age.</p><p><strong>Results: </strong>There were 68 pregnant women who met research criteria, consisting of subjects with sildenafil (<i>n</i> = 32) and without sildenafil (<i>n</i> = 36). A composite of maternal and fetal adverse outcomes occurred in 24 subjects (35.3%). Subjects with oral sildenafil therapy were not associated with maternal mortality. Subjects with oral sildenafil therapy had a lower incidence of fetal adverse outcomes (<i>n</i> = 7, 21.9%) as compared to those without sildenafil (<i>n</i> = 25, 56.8%), <i>P</i> = 0.029. In the multivariate analysis, oral sildenafil therapy had a neutral impact on maternal mortality and fetal adverse outcomes. Other PAH therapy use was a significant independent predictor of maternal mortality. The PVR value was a significant independent predictor of fetal adverse outcomes.</p><p><strong>Conclusion: </strong>Oral sildenafil therapy was safe for pregnant CHD-associated PAH patients. Oral sildenafil therapy reduced fetal adverse outcomes, namely fetal mortality, oligohydramnios, fetal growth restriction, and small for gestational age infants, in patients with CHD-associated PAH. PAH severity had a significant impact on pregnancy outcome.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"156-162"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331514/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389914","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}
Kothandam Sivakumar, Sonia Ali El-Saiedi, Baher Matta Hanna, Pramod Sagar
{"title":"Feasibility of combining additional interventions to facilitate hemodynamics during Transcatheter Fontan procedure without prior surgical preparation.","authors":"Kothandam Sivakumar, Sonia Ali El-Saiedi, Baher Matta Hanna, Pramod Sagar","doi":"10.4103/apc.apc_87_26","DOIUrl":"10.4103/apc.apc_87_26","url":null,"abstract":"<p><p>Transcatheter Fontan is an alternative palliation in severely hypoxic univentricular hearts at high-risk for Fontan surgery. While sternotomy, prolonged intubation, and positive-pressure ventilation impede passive systemic venous return, transcatheter Fontan allows early extubation and mobilization to promote inspiratory venous return, additionally aided by the skeletal muscle pump. Following puncture of the pulmonary artery (PA) and atrium through the transverse pericardial sinus, a long-covered stent bridges the inferior vena cava to the PA. Concurrent interventions create wide inter-atrial communication, interrupt competitive aortopulmonary or antegrade flows, and relieve PA stenosis. This case series details such interventions to facilitate optimal hemodynamics after establishing neo-Fontan circulation.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"244-252"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331504/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389804","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":"Juvenile ankylosing spondylitis unmasked by severe aortic regurgitation and subaortic bump.","authors":"Manjit Mahendran, Jothir Surya Teja Yallanti, Sourabh Agstam, Sivasubramanian Ramakrishnan","doi":"10.4103/apc.apc_303_25","DOIUrl":"10.4103/apc.apc_303_25","url":null,"abstract":"<p><p>Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis that may precede or later progress to ankylosing spondylitis in adulthood. We describe a case of enthesitis-related arthritis diagnosed based on characteristic echocardiographic findings of aortitis, subaortic bump and severe aortic regurgitation.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"253-255"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331490/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389812","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":"Non-surgical management of post-Ross left ventricular pseudoaneurysm assisted by virtual reality imaging.","authors":"Puthiyedath Thejaswi, Pramod Sagar, Kothandam Sivakumar","doi":"10.4103/apc.apc_315_25","DOIUrl":"10.4103/apc.apc_315_25","url":null,"abstract":"<p><p>Infective endocarditis (IE) can cause extensive cardiac tissue destruction, often requiring surgical reconstruction with pericardial patches. The compromised integrity of necrotic tissue increases the risk of pseudoaneurysm formation. Redo surgical repair of pseudoaneurysms is associated with high morbidity; thus, catheter-based interventions may be preferable in select cases. Percutaneous transapical access provides a direct route to left ventricular pseudoaneurysms but demands thorough preprocedural planning and intra-procedural imaging. We report the successful transapical device closure of a large left ventricular pseudoaneurysm following a Ross procedure for aortic valve IE.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"222-226"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331502/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389787","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":"Erratum: Impact of a national policy on congenital heart disease mortality in Brazil.","authors":"","doi":"10.4103/apc.apc_209_26","DOIUrl":"10.4103/apc.apc_209_26","url":null,"abstract":"<p><p>[This corrects the article on p. 224 in vol. 18, PMID: 41938244.].</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"264"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331501/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389501","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}
Dicky Fakhri, Yohana Reena Bunyamin, Jennifer Jesslina Gunawan
{"title":"Revisiting the rhythm: A systematic review and meta-analysis of long-term arrhythmia outcomes after extracardiac versus intracardiac Fontan completion.","authors":"Dicky Fakhri, Yohana Reena Bunyamin, Jennifer Jesslina Gunawan","doi":"10.4103/apc.apc_40_26","DOIUrl":"10.4103/apc.apc_40_26","url":null,"abstract":"<p><p>Studies on the arrhythmia incidence in the Fontan population, especially comparing intracardiac (IC) and extracardiac (EC) Fontan, are limited, with inconsistent findings. This meta-analysis aims to compare arrhythmia risk in EC and IC Fontan patients. Eligible studies comparing arrhythmia outcomes following different Fontan procedures, including EC conduit (ECC), lateral tunnel (LT), and classic Fontan, were identified through a systematic search of the PubMed, Cochrane Library, EMBASE, and Google Scholar databases from 2016 to 2025. Random-effects meta-analysis was performed to pool odds ratios (ORs). A subgroup analysis based on follow-up duration and a restricted analysis comparing ECC versus LT were conducted to explore heterogeneity. Twelve publications with 6280 patients were included in the final analysis. In our systematic review and meta-analysis, the EC Fontan was associated with significantly lower odds of arrhythmia compared to the IC Fontan (OR = 0.38, 95% confidence interval [CI]: 0.27-0.52, <i>P</i> < 0.001), with substantial heterogeneity (<i>I</i> <sup>2</sup> = 74%). In a restricted analysis comparing only ECC with LT, the association remained significant (OR = 0.52, 95% CI: 0.39-0.69; <i>P</i> < 0.001). EC Fontan is linked to a lower observed arrhythmia burden compared to IC Fontan. However, this finding should be interpreted cautiously, as the observed effect may partly reflect the higher arrhythmia burden in classic Fontan, as well as differences in surgical era and follow-up duration. Given the persistent risk of arrhythmia in all Fontan patients, individualized management and long-term surveillance are essential.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"163-172"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331506/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389830","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}
Justo Jose Santiago, Angélica L Ortiz, Alexandra Hurtado-Ortiz, Libardo Augusto Medina, Hector Conrado Jimenez, José L Zunzunegui
{"title":"Endovascular implantation of a Melody valve in the mitral position with extracorporeal membrane oxygenation support in a pediatric patient.","authors":"Justo Jose Santiago, Angélica L Ortiz, Alexandra Hurtado-Ortiz, Libardo Augusto Medina, Hector Conrado Jimenez, José L Zunzunegui","doi":"10.4103/apc.apc_239_25","DOIUrl":"10.4103/apc.apc_239_25","url":null,"abstract":"<p><p>Reoperation for dysfunctional mitral bioprostheses carries substantial risk. Endovascular valve-in-valve (V-in-V) implantation is an alternative, but pediatric experience is scarce. We report the feasibility of the Melody valve V-in-V with elective extracorporeal membrane oxygenation (ECMO) support in an inoperable pediatric patient. A transvenous antegrade, transesophageal echocardiography-guided procedure was performed with venoarterial ECMO. The transmitral gradient fell from 30 to 8 mmHg without paravalvular leak or left-ventricular outflow obstruction. ECMO was discontinued within 24 h. At 3-year follow-up, valve function remains preserved, and the patient is stable. Melody V-in-V with elective ECMO is a viable rescue strategy for high-risk pediatric candidates.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 2","pages":"215-218"},"PeriodicalIF":0.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331500/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148389964","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}