Annals of Pediatric Cardiology最新文献

{"title":"Total anomalous pulmonary venous drainage: Overall results, comparison of the sutureless versus the conventional approach, and predictors of adverse events - A 17-year single-center experience.","authors":"Gurpreet Singh, Siti Laura Mazalan, Sangaraamunisen Ramachandran, Ong Ye Shan, Abdul Muiz Jasid, Hamdan Leman","doi":"10.4103/apc.apc_153_25","DOIUrl":"https://doi.org/10.4103/apc.apc_153_25","url":null,"abstract":"<p><strong>Introduction: </strong>Total anomalous pulmonary venous drainage (TAPVD) constitutes approximately 1.5% of all congenital heart defects. Surgical correction has traditionally been performed using the conventional technique; however, the sutureless approach has emerged as a recent alternative. This study aims to compare the surgical outcomes of the two techniques and to identify the variables influencing them.</p><p><strong>Subjects and methods: </strong>This was a retrospective study over a 17-year period from 2007 to 2024. There were 55 patients of TAPVD: supracardiac ( <b><i>n</i></b> = 32), intracardiac ( <b><i>n</i></b> = 18), infracardiac ( <b><i>n</i></b> = 3), and mixed ( <b><i>n</i></b> = 2).</p><p><strong>Results: </strong>The median age of surgical intervention was 43 days (interquartile range [IQR]: 30-120), and the median weight was 3.40 kg (IQR: 3.00-4.30). Prematurity was present in 3 (5.5%) patients, 11 (19.6%) had preoperative pulmonary vein obstruction, 2 (3.6%) had atrial septal defect obstruction, and 23 (41.1%) had preoperative pulmonary hypertension. Twelve (21.4%) patients required preoperative intubation, 4 (7.1%) required inhaled nitric oxide, and 7 (12.5%) had hemodynamic instability/cardiopulmonary resuscitation (CPR). Surgical correction (supracardiac and infracardiac) was by conventional repair in 20 patients and sutureless in 15 patients. When comparing sutureless and conventional TAPVD repair techniques, no statistically significant differences were observed in rates of pulmonary hypertension crisis, hospital stay duration, noninvasive ventilation duration, postoperative infections, or chest re-exploration rates. Similarly, the incidence of pulmonary venous obstruction (PVO) and early mortality was comparable between the two groups. The predictors of early mortality were seen in patients with preoperative pulmonary hypertension (odds ratio [OR]: 5.63, <b><i>P</i></b> = 0.048), preoperative intubation (OR: 9.29, <b><i>P</i></b> = 0.008), and preoperative CPR (OR: 36.67, <b><i>P</i></b> ≤ 0.001). Incidence of postoperative low cardiac output syndrome was strongly associated with preoperative pulmonary hypertension (OR: 3.90, <b><i>P</i></b> = 0.02), while predictors of postoperative extracorporeal membrane oxygenation predominant in patients with preoperative PVO (OR: 24.57, <b><i>P</i></b> = 0.007), associated pulmonary abnormality (OR: 4.57, <b><i>P</i></b> = 0.031), and preoperative intubation (OR: 3.88, <b><i>P</i></b> = 0.05).</p><p><strong>Conclusions: </strong>Early postoperative outcomes were found to be closely associated with the patients' preoperative clinical status. Overall, in our cohort, no difference was seen between the conventional and sutureless technique of TAPVD repair. These findings may be influenced by the study's limited sample size, potentially reducing its statistical power.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"389-395"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757618","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":"Characterizing Friedreich's ataxia cardiomyopathy with serial cardiac magnetic resonance imagings.","authors":"Megan E Gunsaulus, Rajesh Krishnamurthy, Chance Alvarado, Deipanjan Nandi, Linda H Cripe, Megan A Waldrop, Emily A Hayes","doi":"10.4103/apc.apc_22_26","DOIUrl":"https://doi.org/10.4103/apc.apc_22_26","url":null,"abstract":"<p><strong>Background: </strong>Friedreich's ataxia (FA) is a hereditary neuromuscular disorder with cardiac involvement as the leading cause of death. This study examines the progression of cardiac magnetic resonance imaging (CMR) biomarkers in FA-associated cardiomyopathy and their relationships with clinical outcomes.</p><p><strong>Materials and methods: </strong>This retrospective, single-center study included FA patients with at least one CMR. CMR assessment included segmental late gadolinium enhancement (LGE), left ventricle (LV) ejection fraction (EF), LV wall thickness, native T1 mapping, and extracellular volume (ECV) fraction. Linear mixed-effects regression models were used to assess CMR parameters in relation to each other and time.</p><p><strong>Results: </strong>Fifteen patients (mean age: 22.7 ± 7.6 years) and 37 CMRs were evaluated over an average of 11.0 ± 5.0 years from neuromuscular symptom onset. LV thickness was most notable along the septum, while LGE was localized to the LV free wall. Native T1 and ECV were globally elevated. Six (40%) patients demonstrated LGE, and those negative at baseline remained negative. LGE increased by 0.5 segments per year ( <b><i>P</i></b> = 0.25), and LVEF declined by 0.73%/year ( <b><i>P</i></b> = 0.06); however, neither reached statistical significance. LV thickness, native T1, and ECV did not change significantly. Maximal LV thickness correlated with cardiac symptoms ( <b><i>P</i></b> = 0.04) and cardiac medications ( <b><i>P</i></b> = 0.024).</p><p><strong>Conclusions: </strong>In this exploratory study, CMR demonstrated patterns of septal hypertrophy, lateral wall fibrosis, and trends toward declining ventricular function. Findings were largely nonsignificant and should be considered hypothesis-generating. Larger, multicenter studies are needed to clarify the role of CMR in disease progression and clinical management.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"364-373"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475841/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762334","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":"Middle aortic syndrome in a young child: A case successfully managed by a hybrid technique.","authors":"Ayad Anass, Sghir Salah, Sellouti Mohamed, Abilkassem Rachid","doi":"10.4103/apc.apc_310_25","DOIUrl":"https://doi.org/10.4103/apc.apc_310_25","url":null,"abstract":"<p><p>Middle aortic syndrome is a rare cause of renovascular hypertension in infants. We report a 21-month-old boy weighing 11 kg who presented with severe systemic hypertension (205/117 mmHg) and left ventricular (LV) dysfunction due to diffuse hypoplasia of the thoracoabdominal aorta. Echocardiography and computed tomography angiography showed two coarctation segments involving the superior mesenteric and renal arteries without inflammatory signs. After medical stabilization, acute pulmonary edema and cardiogenic shock developed. A hybrid transabdominal approach with sequential covered-stent implantation achieved favorable recovery, with normalization of blood pressure and improvement in LV function.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"443-445"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762964","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":"Paradoxical coronary embolism through an atrial septal defect presenting as acute myocardial infarction: A case report.","authors":"Mehraj Uid Din Khan, Aamir Rashid, Sameer Purra, Syed Bilal","doi":"10.4103/apc.apc_353_25","DOIUrl":"https://doi.org/10.4103/apc.apc_353_25","url":null,"abstract":"<p><p>Paradoxical embolism through an atrial septal defect (ASD) is a rare but important cause of acute coronary syndrome (ACS) in patients without conventional cardiovascular risk factors. We report the case of a 40-year-old male with no history of hypertension, diabetes, smoking, dyslipidemia, or family history of premature coronary artery disease who presented with acute chest pain and ischemic electrocardiographic changes. Basic thrombophilia profile screening was nonaffirmative. Troponin-T testing using a point-of-care and quantitative assay was positive. Coronary angiography revealed angiographically normal epicardial coronary arteries. Transthoracic and transoesophageal echocardiography demonstrated a large ostium secundum ASD measuring 22 mm × 24 mm, with a prominent Eustachian valve and significant right-to-left shunting on contrast study, accentuated during the Valsalva maneuver. The patient underwent successful transcatheter ASD closure with complete abolition of shunting. This case highlights paradoxical coronary embolism as a potential mechanism of ACS in young, low-risk individuals and underscores the importance of evaluating intracardiac shunts in patients presenting with ACS and nonobstructive coronaries.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"432-436"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475842/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757563","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":"TTN mutation-positive hypertrophic cardiomyopathy in congenitally corrected transposition of the great arteries: A rare coexistence.","authors":"Sangram Chakraborty, Bhargavi Dhulipudi, Shweta Bhakru, Nageswara Rao Koneti","doi":"10.4103/apc.apc_317_25","DOIUrl":"https://doi.org/10.4103/apc.apc_317_25","url":null,"abstract":"<p><p>Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac malformation, in which the morphologic right ventricle (RV) supports the systemic circulation. Systemic RV hypertrophy is usually secondary to pressure overload, while intrinsic myopathic hypertrophy due to sarcomeric gene mutation is exceedingly uncommon. We report a 37-year-old male who presented with exertional dyspnea and chest discomfort. Echocardiography and cardiac magnetic resonance (CMR) imaging revealed atrioventricular and ventriculoarterial discordance consistent with CCTGA, accompanied by asymmetric hypertrophy of the systemic RV and interventricular septum without significant outflow obstruction. Late gadolinium enhancement demonstrated patchy myocardial fibrosis. Genetic testing identified a heterozygous truncating variant in the <b><i>TTN</i></b> gene (exon 46, c. 14414del, p.Thr4805 LysfsTer24), confirming familial hypertrophic cardiomyopathy (HCM). The present case highlights an extremely rare coexistence of CCTGA and TTN-related HCM of the systemic RV. Recognition of this overlap requires high clinical suspicion, multimodal imaging, and molecular testing, which together enable accurate diagnosis, family counseling, and long-term management of this unique phenotype.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"450-453"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475827/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757226","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 and predictors of mortality among children and adolescents with congenital heart disease in Ethiopia: A retrospective follow-up study.","authors":"Mohammed Nasir, Sura Markos, Shibikom Tamrat","doi":"10.4103/apc.apc_235_25","DOIUrl":"https://doi.org/10.4103/apc.apc_235_25","url":null,"abstract":"<p><strong>Background: </strong>Congenital heart disease (CHD) is a leading noninfectious cause of mortality during childhood and adolescence, particularly in developing countries where timely diagnosis and treatment are limited. Despite its public health significance, data on mortality trends and their predictors in resource-limited settings remain scarce. This study aimed to assess mortality trends and identify predictors among children with CHD in a resource-limited setting.</p><p><strong>Materials and methods: </strong>We conducted a retrospective follow-up study on 6228 children and adolescents with CHD who received care at the Cardiac Center of Ethiopia, either as outpatients or inpatients, with follow-up between 2015 and 2024. Data were collected through medical record reviews and phone interviews with caregivers from January 1 to August 1, 2025. Joinpoint was used to assess trends in CHD-related mortality by calculating the annual percent change (APC), and the average APC (AAPC) was used to evaluate overall mortality trends. Predictors of CHD-related mortality were identified using a Cox proportional hazards model.</p><p><strong>Results: </strong>The median age of patients was 0.7 years (interquartile range, 0.3-3.8 years), with a female-to-male ratio of 1.42:1. During follow-up, 827 patients (13.3%) died. The overall AAPC in mortality per 1000 CHD cases was -8.19% (95% confidence interval [CI]: -11.2--5.47, <i>P</i> < 0.001), indicating a statistically significant annual decline. A marked decrease occurred between 2019 and 2022 (APC: -40.94%; 95% CI: -47.41--27.50; <i>P</i> = 0.04). Malnutrition increased mortality risk by 2.9-fold (adjusted hazard ratio [AHR]: 2.9; 95% CI: 1.9-3.9; <i>P</i> < 0.001), pulmonary hypertension (PH) by 3.5-fold (AHR: 3.5; 95% CI: 2.2-6.6; <i>P</i> < 0.001), and the presence of syndromic associations (with or without extracardiac anomalies) by 30% (AHR: 1.3; 95% CI: 1.1-2.2; <i>P</i> = 0.04). Conversely, undergoing surgery or an interventional procedure reduced mortality risk by 80% (AHR: 0.2; 95% CI: 0.1-0.8; <i>P</i> < 0.001).</p><p><strong>Conclusion: </strong>Mortality among children and adolescents with CHD in Ethiopia has shown a significant decline in recent years. However, malnutrition, PH, syndromic associations, and lack of surgical or interventional care remain key predictors of poor outcomes. Strengthening timely access to interventions, enhancing nutritional and multidisciplinary support, and prioritizing early identification of high-risk patients are essential to further reduce CHD-related mortality in resource-limited settings.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"379-388"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757306","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":"Pediatric extracorporeal membrane oxygenation support at a single center: Outcomes by indication, prognostic factors, and duration.","authors":"L Lily Rosenthal, Andreas W Flemmer, Kai Martin Förster, Mathias Bruegel, Jürgen Hörer, Nikolaus A Haas, Christian Hagl, Sebastian Sadoni, Oliver J Muensterer, Sebastian Georg Michel","doi":"10.4103/apc.apc_115_26","DOIUrl":"https://doi.org/10.4103/apc.apc_115_26","url":null,"abstract":"<p><strong>Background: </strong>Pediatric extracorporeal membrane oxygenation (pECMO) provides life-saving cardiopulmonary support for children with severe cardiac or respiratory failure. Identifying prognostic factors may improve risk stratification and clinical decision-making.</p><p><strong>Methods: </strong>We retrospectively reviewed 149 patients (<18 years) who received pECMO at a single center between 2003 and 2025. Patients with multiple extracorporeal membrane oxygenation (ECMO) runs during the same hospitalization were excluded. Demographic, clinical, laboratory, and outcome data were collected. The primary endpoint was 30-day survival.</p><p><strong>Results: </strong>Among 149 patients, the median age was 245 days, and the median weight was 6.3 kg. Indications for ECMO were acute cardiac failure (56%), respiratory failure (37%), and extracorporeal cardiopulmonary resuscitation (ECPR; 11%). Kaplan-Meier analysis demonstrated significant survival differences among groups (log-rank <i>P</i> = 0.010). Mean survival times were 24.07 days (95% confidence interval [CI] 20.72-27.41) for cardiac failure, 28.99 days (95% CI 24.64-33.35) for respiratory failure, and 14.13 days (95% CI 7.72-20.55) for ECPR. In multivariable Cox regression analysis, pre-ECMO lactate (hazard ratio [HR] 1.042, 95% CI 1.008-1.078; <i>P</i> = 0.017) and post-ECMO lactate (HR 1.067, 95% CI 1.024-1.110; <i>P</i> = 0.002) were independent predictors of mortality. Pre- and post-ECMO aspartate aminotransferase and pH were not significantly associated with mortality.</p><p><strong>Conclusions: </strong>Elevated lactate levels before and after ECMO initiation independently predicted mortality in pediatric patients receiving ECMO. Lactate may serve as a useful biomarker for prognostication and risk stratification in this population.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"396-404"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475837/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757489","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":"Two decades of pediatric aortic stenosis: Balloon versus surgical interventions: A single center study.","authors":"Yousef Erfani Karimzadeh Tosi, Shirin Ghiasi, Zahra Barati Farimani, Mohammadreza Abolvahedi, Mohammad Abbasi Tashnizi, Ali Mottaghi Moghadam, Hassan Mottaghi Moghadam","doi":"10.4103/apc.apc_259_24","DOIUrl":"https://doi.org/10.4103/apc.apc_259_24","url":null,"abstract":"<p><strong>Introduction: </strong>Congenital valvular aortic stenosis (AS) represents 3%-6% of congenital heart diseases. This study aimed to compare the outcomes of balloon valvuloplasty (BV) and surgical valvotomy (SV) in patients with AS.</p><p><strong>Methods: </strong>This retrospective study was conducted on 69 patients. A retrospective analysis was done on 69 patients (who underwent BV or SV) at a tertiary heart center in Mashhad, Iran (between 2000 and 2023).</p><p><strong>Results: </strong>The mean age of BV patients (<i>n</i> = 24, 17 males) was 67.08 ± 55.92 months, while it was 97.44 ± 64.18 months in SV patients (<i>n</i> = 45, 33 males). Both treatments reduced the pressure gradients without significant differences (<i>P</i> > 0.05). Aortic valve replacement (AVR) was performed in 20% of SV and 4.1% of BV patients. Over 60% remained intervention-free for at least 5 years. Moderate-to-severe aortic insufficiency developed in 19% of SV and 7% of BV patients. A second-time SV was required in 6.7% and 4.2% of SV and BV cases, respectively.</p><p><strong>Conclusion: </strong>BV and SV showed comparable outcomes, with BV demonstrating shorter hospitalization, fewer complications, and lower AVR rates, though reintervention remained necessary for both.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"405-412"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762322","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":"Therapeutic challenge in a 5-year-old girl with Gorlin-Goltz syndrome and cardiac fibroma.","authors":"Agnieszka Pskit-Hanuszczak, Tomasz Książczyk, Radosław Pietrzak, Mariusz Furmanek, Mariusz Kuśmierczyk, Bożena Werner","doi":"10.4103/apc.apc_82_26","DOIUrl":"https://doi.org/10.4103/apc.apc_82_26","url":null,"abstract":"<p><p>Gorlin-Goltz syndrome (GGS) is a rare hereditary autosomal dominant condition. Cardiac fibromas are one of the clinical manifestations of this disease. The presence of these benign heart tumors may lead to life-threatening complications. We present the case of a 5-year-old girl with GGS and a large cardiac fibroma who had ventricular fibrillation during respiratory tract infection. Several options of treatment were considered in the secondary prevention of cardiac arrest. A partial resection of the tumor was performed to prevent recurrence of malignant arrhythmia. The loop recorder implanted postoperatively didn't record any episodes of sustained ventricular arrhythmia in a 9-month follow-up. In conclusion, cardiac fibromas, especially with the presence of infections, increase risk of ventricular arrhythmias. Partial resection of tumor can be effective and relatively safe in the secondary prevention of ventricular fibrillation in patients with large cardiac fibromas with GGS.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"454-456"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475834/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148763036","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 patent ductus venosus in an adolescent using a KONAR-Multifunctional Occluder device.","authors":"Bhushan Shah, Abhimanyu Uppal, Sudesh Prajapathi, Shakti Singh","doi":"10.4103/apc.apc_18_26","DOIUrl":"https://doi.org/10.4103/apc.apc_18_26","url":null,"abstract":"<p><p>Patent ductus venosus (PDV) is a rare intrahepatic congenital portosystemic shunt that can result in hepatic encephalopathy and hepatopulmonary syndrome due to diversion of portal blood away from hepatic metabolism. Although typically diagnosed in infancy, PDV may rarely remain undetected until adolescence. Management of late presenters is challenging, as abrupt closure of large shunts may precipitate portal hypertension, and coil embolization carries a higher risk of systemic migration. We report a 16-year-old boy presenting with unexplained hypoxemia (SpO₂ 84% on room air), digital clubbing, and hyperammonemia. Bubble contrast echocardiography demonstrated an extracardiac right-to-left shunt, while abdominal imaging confirmed a large PDV with associated diffuse pulmonary arteriovenous malformations. Temporary balloon occlusion testing demonstrated hemodynamic suitability for closure. Transcatheter occlusion using a Konar-Multifunctional Occluder resulted in significant improvement in arterial oxygen saturation and normalization of serum ammonia levels, sustained during one-year follow-up. This case highlights the importance of meticulous hemodynamic assessment and demonstrates the safety and efficacy of the Konar-MFO device in late-presenting PDV.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"19 4","pages":"417-421"},"PeriodicalIF":0.8,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13475840/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148757733","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}