PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

{"title":"Desmoplakin related cardiomyopathy with contrasting desmoplakin dermatologic changes: A case series and review of the literature","authors":"Taylor J. Prechtel ,&nbsp;Anita N. Haggstrom ,&nbsp;John J. Parent","doi":"10.1016/j.ppedcard.2025.101845","DOIUrl":"10.1016/j.ppedcard.2025.101845","url":null,"abstract":"<div><h3>Introduction</h3><div>Desmoplakin (<em>DSP</em>) genetic variants cause various cardio-cutaneous phenotypes. DSP related cardiomyopathy (DSP-CM) is a cause of arrhythmogenic cardiomyopathy (ACM). Patient presentation is highly variable, and dermatologic manifestations are often the first sign of impending cardiac dysfunction. A subset DSP-CM patients require advanced cardiac therapies (ACT) such as heart transplantation (HTx).</div></div><div><h3>Case description</h3><div>Patient 1 is a male neonate who presented at birth with tense bullae and erosions scattered on the body. Genetic panel disclosed two <em>DSP</em> variants. He was diagnosed with DSP-related skin fragility. At age 2.5 years, DSP-CM developed, leading to aborted cardiac arrest and HTx at age 4.</div><div>Patient 2 is a 15-year-old male who presented with new onset dilated cardiomyopathy and wooly hair, hypodontia, and onychodystrophy. Genetic testing revealed a <em>DSP</em> variant, which was diagnostic for DSP-CM with woolly hair, keratoderma, and tooth agenesis. He underwent HTx 1 month after presentation.</div></div><div><h3>Discussion</h3><div><em>DSP</em> variants cause a distinct form of ACM. Left dominant cardiomyopathy and systolic dysfunction were the primary manifestations in our patients. Pediatric DSP-CM cases are sparse in the literature. We demonstrate that patients with DSP-CM can successfully undergo HTx with special attention to treatment of their dermatologic disease.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101845"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144221287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"RVOT stenting in unconventional circumstances in the pediatric age group: A case series","authors":"Mohammad Moaaz Kidwai ,&nbsp;Shaad Abqari ,&nbsp;Shamayal Rabbani ,&nbsp;Mohammad Mirza Kamran ,&nbsp;Mohammad Azam Haseen","doi":"10.1016/j.ppedcard.2025.101822","DOIUrl":"10.1016/j.ppedcard.2025.101822","url":null,"abstract":"<div><h3>Background</h3><div>Right ventricular outflow tract stenting (RVOTS) has emerged as an acceptable alternative to Blalock-Taussig-Thomas shunt surgery. This approach is indicated for patients with Tetralogy of Fallot (TOF) and unfavorable pulmonary artery anatomy. The utility of RVOTS has not been established outside the domain of classical TOF patients, i.e., in unconventional circumstances.</div></div><div><h3>Objectives</h3><div>This study was performed to analyze the utility of RVOTS in unconventional circumstances, including reintervention following RVOTS.</div></div><div><h3>Methods</h3><div>This was a retrospective analysis performed in a tertiary care pediatric cardiac center. The details of all patients who underwent RVOTS were analyzed. All patients who underwent RVOTS under unconventional circumstances and who underwent RVOT reintervention were included in this analysis. The patients' immediate outcomes and last follow-up visit were analyzed.</div></div><div><h3>Results</h3><div>Of the 56 patients who underwent RVOTS at our center, 5 patients were included in this analysis; the median age was 3 years (range = 1–13 years). A total of 7 RVOT interventions (including one re-stenting and one balloon dilatation) were performed in these 5 patients. Four patients underwent RVOTS under unconventional circumstances, and one patient underwent re-stenting. All 5 patients had excellent immediate outcomes, and all were discharged in satisfactory condition.</div></div><div><h3>Conclusion</h3><div>RVOTS can be utilized in unconventional situations with good immediate and short-term outcomes, as shown in this case series.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101822"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144490531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence of congenital heart disease among neonates in Fayoum district","authors":"Sayed Ali Amin,&nbsp;Sumer Nady Mohamed Elfakhrany,&nbsp;Ahmed Mahmoud Abd Elmoktader","doi":"10.1016/j.ppedcard.2025.101858","DOIUrl":"10.1016/j.ppedcard.2025.101858","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) is a significant structural irregularity in the heart's chambers or major vessels, affecting its function.</div></div><div><h3>Objectives</h3><div>To determine the occurrence of CHD among newborns in the Fayoum district of Egypt.</div></div><div><h3>Methods</h3><div>This cross-sectional observational study included 2247 live births. Subjects were selected over a two-year period from neonates born at Fayoum University Hospital, Fayoum Insurance Hospital, and five other general hospitals between January 2018 and December 2019.</div></div><div><h3>Results</h3><div>Among the studied sample, 2192 newborns (97.6 %) had no congenital heart defect, while 55 (2.4 %) were diagnosed with CHD. Parental consanguinity was reported in 19 cases (34.5 %) with CHD. Among the acyanotic CHD cases: 13 (23.6 %) had ventricular septal defect, 7 (12.7 %) had patent ductus arteriosus, 6 (10.9 %) had atrial septal defect, 5 (9.1 %) had pulmonary stenosis, 2 (3.6 %) had aortic stenosis, 2 (3.6 %) had atrioventricular canal defect, 1 (1.8 %) had coarctation of the aorta, and 1 (1.8 %) had peripheral pulmonary stenosis. Regarding cyanotic CHD, 7 patients (12.7 %) had tetralogy of Fallot, 4 (7.3 %) had pulmonary atresia, 3 (5.5 %) had transposition of the great arteries, and one patient (1.8 %) each had complex CHD, single ventricle, total anomalous pulmonary venous return, and tricuspid atresia.</div></div><div><h3>Conclusion</h3><div>The study reveals that the birth prevalence of CHD in the Fayoum district is consistent with global estimates. Acyanotic CHD, particularly ventricular septal defect, was the most commonly observed cardiac anomaly.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101858"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144662046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Histoplasma endocarditis in a prosthetic pulmonary valve: A case report","authors":"Sharadhi Thalner ,&nbsp;Reem Youssef ,&nbsp;James Wood ,&nbsp;Jeremy L. Herrmann ,&nbsp;J.P. Lavik ,&nbsp;Erin Rudzinski ,&nbsp;Umesh Dyamenahalli ,&nbsp;Mark Ayers","doi":"10.1016/j.ppedcard.2025.101853","DOIUrl":"10.1016/j.ppedcard.2025.101853","url":null,"abstract":"<div><h3>Introduction</h3><div>Infective endocarditis (IE) following transcatheter pulmonary valve placement is a significant concern. Fungal endocarditis, though rare, poses significant diagnostic and therapeutic challenges, particularly when caused by <em>Histoplasma capsulatum</em>.</div></div><div><h3>Case report</h3><div>We present a 24-year-old female with tetralogy of Fallot (ToF) and a history of transcatheter prosthetic pulmonary valve replacement, who developed IE secondary to active histoplasmosis. Initial symptoms included worsening fatigue and jaundice, leading to hospitalization. Echocardiography revealed new stenosis of the pulmonary valve, prompting further investigation. Despite negative blood cultures, serological and molecular tests for histoplasmosis were positive, confirming the diagnosis. Treatment commenced with amphotericin B, followed by surgical valve replacement two weeks later, revealing hyphal elements indicative of <em>H. capsulatum</em>. The patient subsequently received prolonged antifungal therapy with itraconazole with near complete resolution of symptoms and a mild residual gradient.</div></div><div><h3>Conclusion</h3><div>This case emphasizes the necessity for heightened awareness of fungal pathogens in patients with prosthetic devices, particularly in the context of culture-negative endocarditis. Early diagnosis and intervention, including surgical resection and appropriate antifungal treatment, are critical for improving patient outcomes.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101853"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144696942","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcatheter embolization of aortopulmonary collaterals using the Trufill® n-Butylcyanoacrylate (n-BCA) liquid embolic system; A single-center experience","authors":"Joseph Casadonte,&nbsp;Jose Andres Morales Hernandez,&nbsp;Danyal Khan","doi":"10.1016/j.ppedcard.2025.101855","DOIUrl":"10.1016/j.ppedcard.2025.101855","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Aortopulmonary collaterals (APCs) are commonly found in patients with cyanotic heart disease. These APCs compete with normal pulmonary blood flow in patients who have undergone Glenn or Fontan surgery. APCs are also seen in patients with cystic fibrosis (CF), where they are known to cause hemoptysis. Transcatheter occlusion of APCs has previously been described using coils, vascular plugs, and polyvinyl alcohol (PVA) particles. We present a series of patients in which the APCs were embolized using Trufill n-BCA liquid.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Objectives&lt;/h3&gt;&lt;div&gt;This study aims to evaluate the safety and effectiveness of Trufill® N-Butylcyanoacrylate (n-BCA) for transcatheter embolization of aortopulmonary collateral vessels in patients with congenital heart disease and cystic fibrosis, particularly in cases where traditional embolization methods are limited or in patients with significant hemoptysis.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;From 2009 to 2012, a total of 18 catheterization procedures were performed (in 15 patients), in which APCs were embolized using n-BCA. The mean age was 8.5 years (range: 4 months to 21 years), with a mean weight of 29 kg (range: 7–72 kg). Three patients had cystic fibrosis (CF) and presented with hemoptysis. The remaining patients had cyanotic congenital heart disease and had undergone Bidirectional Glenn or Fontan procedures. One of the congenital heart disease patients had two catheterization procedures (2.5 years apart) for hemoptysis.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;n-BCA embolization of APCs was technically successful in all patients. The three patients with cystic fibrosis who presented with hemoptysis had symptomatic improvement and have not needed repeat catheterization. One patient with cyanotic congenital heart disease and hemoptysis had acute improvement; however, 2.5 years later, she had recurrent hemoptysis and required additional APCs to be embolized. A complication attributable to n-BCA use also occurred in this patient. Following occlusion of an APC arising from the left lateral thoracic artery, she developed erythema of the overlying skin, followed a few days later by the formation of a small ulcer (presumably due to ischemia of the soft tissue/skin). The ulcer resolved without any specific treatment.&lt;/div&gt;&lt;div&gt;The only other complication occurred when n-BCA unintentionally embolized to the ulnar artery during APC embolization, causing partial occlusion in a patient with a previously occluded radial artery. Vascular surgery successfully removed the material, and the patient had no lasting effects. There were no other major n-BCA-related complications such as cerebrovascular accident, pulmonary embolism, or catheter adhesion.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;n-BCA is a liquid embolic agent that is FDA-approved for the embolization of cerebral arteriovenous malformations. PVA particles, previously used for cerebral AVMs, have a high recanalization rate and have ","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101855"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144655446","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Staged biventricular repair for D-transposition of the great arteries with a hypoplastic right ventricle","authors":"Khaled Shams Abdelmagid ,&nbsp;Nirbhay Parashar ,&nbsp;Brian Reemtsen ,&nbsp;Amna Qasim","doi":"10.1016/j.ppedcard.2025.101838","DOIUrl":"10.1016/j.ppedcard.2025.101838","url":null,"abstract":"<div><div>Right ventricular hypoplasia with dextro-transposition of great arteries (D-TGA) is a rare association that precludes arterial switch operation in the neonatal period. Given its rarity, a standardized pathway for surgical decision-making (single vs biventricular repair) does not exist. We report a case of D-TGA-hypoplastic-RV that underwent successful staged biventricular repair.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101838"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144070798","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Jervell and Lange-Nielsen syndrome: A case report of a variant in the KCNQ1 gene in a Jordanian child","authors":"Hamzeh Al-Momani ,&nbsp;Ala'a Al-ma'aiteh ,&nbsp;Abd alraheem Abu motawe ,&nbsp;Waleed AlSatari ,&nbsp;Abdallah Ghwirin ,&nbsp;Anas Sheeb ,&nbsp;Osama K. Musallam ,&nbsp;Hazim Alkousheh","doi":"10.1016/j.ppedcard.2025.101844","DOIUrl":"10.1016/j.ppedcard.2025.101844","url":null,"abstract":"<div><div>Jervell and Lange-Nielsen syndrome (JLNS) is a rare autosomal recessive disorder characterized by congenital bilateral sensorineural hearing loss and prolonged QTc interval, which predisposes patients to life-threatening arrhythmias. A 5-year-old girl presented with congenital hearing loss, recurrent syncopal episodes, and severe iron deficiency anemia. Electrocardiogram showed a prolonged QTc interval of 530 ms, and genetic testing identified a homozygous nonsense variant in the KCNQ1 gene (c.1480G&gt;T; p.Glu494Ter). The patient was started on beta-blockers and high-dose iron therapy, which led to a reduction in syncopal episodes and improvement in hematological parameters. She was referred to an electrophysiology center for consideration of an Implantable cardioverter defibrillator (ICD) or possible left cardiac sympathetic denervation. This is the first documented case of JLNS with a KCNQ1 variant reported in Jordan, emphasizing the critical role of genetic testing in diagnosis and management.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101844"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144204064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Balloon- versus self-expandable stents in the endovascular treatment of adults with aortic coarctation: A systematic review and meta-analysis","authors":"Kristine Santos ,&nbsp;Luísa Zanelatto de Araujo ,&nbsp;Sriharsha Koduru ,&nbsp;Mislav Planinc","doi":"10.1016/j.ppedcard.2025.101854","DOIUrl":"10.1016/j.ppedcard.2025.101854","url":null,"abstract":"<div><h3>Background</h3><div>Coarctation of the aorta (CoA) is a congenital cardiovascular defect that may persist into adulthood. Endovascular stenting has become the preferred approach, with balloon-expandable stents (BES) and self-expandable stents (SES) being commonly used. However, there is a notable paucity of comparative data evaluating their relative safety and efficacy.</div></div><div><h3>Objectives</h3><div>We aimed to compare the outcomes of BES and SES in the endovascular management of adults with CoA.</div></div><div><h3>Methods</h3><div>A systematic review and meta-analysis were performed by searching MEDLINE, Scopus, and the Cochrane Library for studies directly comparing BES and SES in adult CoA patients. Eligible studies included randomized controlled trials and observational studies. Pooled odds ratios (OR) with 95 % confidence intervals (CI) were calculated using a random-effects model.</div></div><div><h3>Results</h3><div>Three studies (one RCT, two observational), comprising 282 patients, were included, of whom 53.5 % received BES. No significant differences were observed in the cumulative peri-procedural complications [OR 2.5; 95 % CI 0.4 to 18.2; <em>p</em> = 0.07, I² = 9 %], stent migration [OR 1.1; 95 % CI 0.3 to 4.0; <em>p</em> = 0.94; I² = 51 %], and persistent hypertension post-intervention [OR 0.8; 95 % CI 0.4 to 1.6; <em>p</em> = 0.60; I² = 0 %]. Heterogeneity was minimal across most outcomes.</div></div><div><h3>Conclusion</h3><div>Our analysis suggests that BES and SES offer comparable safety and efficacy in the endovascular treatment of adult CoA. However, the small number of studies and wide confidence intervals warrant cautious interpretation. These findings highlight the need for larger, prospective randomized trials to better guide stent selection in this population.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101854"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144662601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of acute kidney injury using estimated glomerular filtration rate and blood urea nitrogen in pediatric patients undergoing cardiac surgery: Experience from single institution in Afghanistan","authors":"Atefa Ahmadi","doi":"10.1016/j.ppedcard.2025.101850","DOIUrl":"10.1016/j.ppedcard.2025.101850","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Acute kidney injury (AKI) is a significant complication in pediatric cardiac surgery, especially among congenital heart disease (CHD) patients. Its incidence is rising globally due to increased cardiac procedures. Children with cardiac surgery-associated AKI (CSA-AKI) face worse postoperative outcomes, including prolonged mechanical ventilation, higher morbidity, mortality, and healthcare costs. Mechanisms of AKI are multifactorial, involving prolonged cardiopulmonary bypass (CPB), hypoperfusion, and inflammatory responses such as systemic inflammatory response syndrome (SIRS) and compensatory anti-inflammatory response syndrome (CARS). Hemolysis during CPB releases free hemoglobin, causing endothelial dysfunction, while reactive oxygen species (ROS) exacerbate kidney injury.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Objectives&lt;/h3&gt;&lt;div&gt;This study aimed to assess the incidence of AKI in pediatric patients undergoing cardiac surgery, utilizing estimated glomerular filtration rate (eGFR) and blood urea nitrogen (BUN) levels while considering factors like age, gender, surgery type, complexity, and CPB duration to enhance understanding of postoperative renal outcomes.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;A retrospective cross-sectional study was conducted at the French Medical Institute for Mother and Children (FMIC) in Kabul, analyzing data from 383 pediatric patients (ages 0–18) who underwent open-heart surgery between January 1, 2022, and September 30, 2024. Patients with pre-existing renal dysfunction or incomplete data were excluded. AKI was defined and staged using the Kidney Disease: Improving Global Outcomes (KDIGO) criteria. Logistic regression analyses identified AKI predictors, reporting odds ratios (OR), and &lt;em&gt;p&lt;/em&gt;-values.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Among 383 pediatric patients (median age 5 years; 57 % male), renal function declined significantly post-surgery, with median GFR decreasing from 123.9 to 89.9 mL/min/1.73 m&lt;sup&gt;2&lt;/sup&gt; (30% reduction; &lt;em&gt;p&lt;/em&gt; &lt; 0.001). AKI occurred in 33 % (&lt;em&gt;n&lt;/em&gt; = 128), classified as Stage 1 (13 %), Stage 2 (16 %), and Stage 3 (4 %) per KDIGO criteria. Significant predictors of AKI included higher preoperative creatinine (aOR = 32.97, &lt;em&gt;p&lt;/em&gt; = 0.02), elevated postoperative BUN (aOR = 1.11, &lt;em&gt;p&lt;/em&gt; = 0.010), longer bypass duration (aOR = 1.02 per minute, &lt;em&gt;p&lt;/em&gt; = 0.014), higher baseline GFR (aOR = 1.012, &lt;em&gt;p&lt;/em&gt; = 0.009), and younger age (aOR = 0.26, &lt;em&gt;p&lt;/em&gt; = 0.03). Higher postoperative GFR was protective (aOR = 0.97, &lt;em&gt;p&lt;/em&gt; &lt; 0.001). The model demonstrated moderate explanatory power (Nagelkerke R&lt;sup&gt;2&lt;/sup&gt; = 0.44) and showed good discrimination (AUC = 0.81; 95 % CI: 0.76–0.86; p &lt; 0.001).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;AKI occurred in 33 % of pediatric cardiac surgery patients, with key risk factors including younger age, higher preoperative creatinine, elevated postoperative BUN, longer bypass time, and higher baseline GFR. Postoperative","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101850"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144321135","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Untreated congenital heart disease leading to Eisenmenger syndrome: Cultural Implications and barriers to care","authors":"Lauren Hirth ,&nbsp;Connor Smith ,&nbsp;Daniel McAree ,&nbsp;Mary Kay Olive ,&nbsp;Jonathon Hagel","doi":"10.1016/j.ppedcard.2025.101837","DOIUrl":"10.1016/j.ppedcard.2025.101837","url":null,"abstract":"<div><div>Pulmonary arterial hypertension in children can occur from many causes, but when secondary to excessive pulmonary blood flow due to congenital heart disease, timely management is essential. Excessive pulmonary blood flow can occur in ventricular septal defects (VSDs) and in patent ductus arteriosus (PDA), among other defects, and if longstanding, can lead to irreversible changes to the pulmonary vasculature and development of Eisenmenger syndrome (ES). We present the case of a pediatric patient who developed ES due to a hemodynamically significant PDA and VSD, as well as a sizable atrial septal defect, that were not evaluated for many years in the setting of both language and cultural barriers to accessing care in the Amish community. While demonstrating slight improvements in oxygen saturations with medical therapies tailored toward the family's cultural needs, her prognosis remains guarded. We review the essential need for the medical community to proactively provide care to patient populations with identifiable barriers and to appropriately intervene to prevent morbidity and mortality.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101837"},"PeriodicalIF":0.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}