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Background and Objectives: Anthracycline chemotherapy is a cornerstone in pediatric oncology but carries a significant risk of cardiotoxicity. The early detection of cardiac dysfunction is crucial for timely intervention. This study aims to evaluate the predictive value of combining speckle tracking echocardiography (STE) parameters with traditional cardiac biomarkers for the early detection of anthracycline-induced cardiotoxicity in pediatric oncology patients. Methods: A retrospective cohort study was conducted, involving 99 pediatric oncology patients undergoing anthracycline therapy and 50 age- and sex-matched healthy controls. Cardiac function was assessed using STE parameters—global longitudinal strain (GLS), Simpson's method of disk ejection fraction (SMOD EF), and myocardial performance index (MPI)—alongside biomarkers including cardiac troponin I (cTnI) and B-type natriuretic peptide (BNP). Assessments were performed at baseline and at 3, 6, and 12 months post-therapy initiation. Results: A total of 28.3% of patients developed cardiotoxicity based on the LVEF decrease. Significant differences were observed between oncological patients under anthracycline treatment and healthy controls. Patients had reduced GLS (−16.1 ± 4.7% vs. −19.6 ± 5.1%, p < 0.001), a lower SMOD EF (55.7 ± 6.3% vs. 60.2 ± 6.0%, p < 0.001), and a higher MPI (0.38 ± 0.06 vs. 0.33 ± 0.05, p < 0.001). Elevated cTnI levels were found in patients compared to controls (3.1 ± 0.9 ng/mL vs. 1.3 ± 0.6 ng/mL, p < 0.001). Regression analysis showed that combining GLS, SMOD EF, MPI, and cTnI levels significantly predicted cardiotoxicity (odds ratio = 7.12, 95% CI: 3.04–12.76, p < 0.001). Conclusions: Combining STE parameters with cardiac biomarkers enhances the early detection of anthracycline-induced cardiotoxicity in pediatric oncology patients. This combined assessment may facilitate timely interventions to prevent long-term cardiac complications. [ABSTRACT FROM AUTHOR]

Type I collagen, prevalent in the extracellular matrix, is biocompatible and crucial for tissue engineering and wound healing, including angiogenesis and vascular maturation/stabilization as required processes of newly formed tissue constructs or regeneration. Sometimes, improper vascularization causes unexpected outcomes. Vascularization failure may be caused by extracellular matrix collagen and non-collagen components heterogeneously. This study compares the angiogenic potential of collagen type I-based scaffolds and collagen type I/glycosaminoglycans scaffolds by using the chick embryo chorioallantoic membrane (CAM) model and IKOSA digital image analysis. Two clinically used biomaterials, Xenoderm (containing type I collagen derived from decellularized porcine extracellular matrix) and a dual-layer collagen sponge (DLC, with a biphasic composition of type I collagen combined with glycosaminoglycans) were tested for their ability to induce new vascular network formation. The AI-based IKOSA app enhanced the research by calculating from stereomicroscopic images angiogenic parameters such as total vascular area, branching sites, vessel length, and vascular thickness. The study confirmed that Xenoderm caused a fast angiogenic response and substantial vascular growth, but was unable to mature the vascular network. DLC scaffold, in turn, produced a slower angiogenic response, but a more steady and organic vascular maturation and stabilization. This research can improve collagen-based knowledge by better assessing angiogenesis processes. DLC may be preferable to Xenoderm or other materials for functional neovascularization, according to the findings. [ABSTRACT FROM AUTHOR]

Background/Aim: Biomaterials are essential in modern medicine, both for patients and research. Their ability to acquire and maintain functional vascularization is currently debated. The aim of this study was to evaluate the vascularization induced by two collagen-based scaffolds (with 2D and 3D structures) and one non-collagen scaffold implanted on the chick embryo chorioallantoic membrane CAM). Materials and Methods: Classical stereomicroscopic image vascular assessment was enhanced with the IKOSA software by using two applications: the CAM assay and the Network Formation Assay, evaluating the vessel branching potential, vascular area, as well as tube length and thickness. Results: Both collagen-based scaffolds induced noninflammatory angiogenesis, but the non-collagen scaffold induced a massive inflammation followed by inflammatoryrelated angiogenesis. Vessels branching points/Region of Interest (Px^2) and Vessel branching points/Vessel total area (Px^2), increased exponentially until day 5 of the experiment certifying a sustained and continuous angiogenic process induced by 3D collagen scaffolds. Conclusion: Collagen-based scaffolds may be more suitable for neovascularization compared to non-collagen scaffolds. The present study demonstrates the potential of the CAM model in combination with AI-based software for the evaluation of vascularization in biomaterials. This approach could help to reduce and replace animal experimentation in the pre-screening of biomaterials. [ABSTRACT FROM AUTHOR]

A rare, uncommon disorder called PHACE(S) (P-posterior fossa anomalies, H-hemangioma, A-arterial anomalies, C-cardiac anomalies, E-eye anomalies, and S-sternal cleft) of unknown etiology was rarely reported. Children are susceptible to developing PHACE(S) syndrome from the moment they are born. It may be challenging for a physician to appropriately diagnose and treat children with PHACE due to the multifaceted nature of the disease and the extensive range of consequences that may be associated with it. A one-month-old newborn girl was admitted to hospital with extensive, multiple facial infantile hemangiomas, ulceration of the lower lip hemangioma-like lesion, cardiovascular, sternal, and neurological concomitant malformations. Five days following the initial application of the medication, systemic treatment with propranolol and topical treatment with silver sulfadiazine produced their first noticeable benefits. The lip ulceration was mostly healed and facial hemangioma started to regress. The regression continued under therapy and this effect persists for 6 months since Propranolol therapy ended. No cardiovascular or neurological clinical events have been registered during follow-up. The present case has three peculiarities: (1) high number of facial hemangiomas; (2) presence of subependymal cyst not yet reported in the literature associated with PHACE syndrome; and (3) lack of cardiovascular events during therapy knowing that these events frequently appear in PHACE syndrome patients. [ABSTRACT FROM AUTHOR]

Coarctation of the aorta is a congenital heart defect involving a narrowing of the descending aorta. Blood pressure is higher before the narrowing and lower past the narrowing, with 20 mmHg difference between upper and lower limbs, clinically expressed by absent femoral pulses. In infants, coarctation of the aorta is severe and represent a cardiological emergency, but in child, aortic coarctation is sometime under-diagnosed, patients presenting at the hospital for high blood pressure of unknown etiology, or complaining of headache, or lower limb pain in effort. We want to highlight aortic coarctation as a cause of high blood pressure in children and to drawn attention that this kind of patients, even after aortic coarctation repair may remain with hypertension that has to be treated and patients followed up. We want to share our experience with some cases of surgical correction of aortic coarctation and complications such as recoarctation in special type of gothic aortic arch and also, recoarctation after stent implanting. [ABSTRACT FROM AUTHOR]

Introduction: Most vascular anomalies are clinically easy to recognize. The age is the most prominent element to take into consideration for the diagnostic. Lesions differ by the moment of appearance: congenital hemangiomas and hemangioendotheliomas (occurred at birth); lymphatic- and venous malformations (emerged in the first week of life-unprogressive), infantile hemangiomas (emerged in the first week of life-fast evolutive) and arteriovenous malformations (appeared throughout childhood). One may diagnose up to 90% of cases based on aspect (color, depth, local temperature, presence of murmur). Doppler ultrasound and MRI may help in difficult cases. Materials and methods: We performed a prospective study of 54 cases with vascular anomalies from May 2010 to April 2013. Thirty-six girls and 18 boys treated with Propranolol - unselective beta-blocker. There were 50 vascular tumors and 4 vascular malformations. All patients underwent ECG and echocardiography before treatment. Four patients excluded based on consent withdrawal. Results: Treatment: age m=9,24 months, duration m=9,15 months and response: very good-66,66%, good-12,96%, partial-7,40%, no response-9,25% and aggravation-3,70%. As a conclusion, our results support the use of Propranolol in vascular anomalies. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas (IH) are the most common benign vascular tumors in childhood, affecting 4-5 % of Caucasian infants in their first year of life. We performed a prospective study of 34 cases of IH treated with propranolol from May 2010 to December 2012. Beta blocker treatment was administered in 21 girls and 13 boys aged 2 to 14 months (mean age 5.4 months). All patients were evaluated with electrocardiography and echocardiography prior to start of treatment. Propranolol was administrated at a dose of 1mg/kg/day divided in 3 equal parts (day 1) and 2mg/kg/day divided in 3 equal parts (day 2), continuing with this dose throughout the rest of the treatment. The treatment was initiated during a 24- 48 hours hospitalization, time during which blood pressure and heart rate were measured every 6 hours. After 24 hours of hospitalization we performed an EKG and measured the blood glucose level. None of our patients developed hypotension, hypoglycemia or bronchospasm. The results are: very good-70.96%, good-16.12%, partial-6.45%, no response- 6.45%. Three patients were uncooperative and after 1 month to treatment they have not presented to control and stop the treatment. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to assess our results of the treatment in children with vascular anomalies. Methods: We retrospectively reviewed the charts of 704 patients with 843 vascular anomalies treated in the Department of Pediatric Surgery between January 1996 and June 2012. Results: Sex ratio was females/males 1.5:1, 69% were less than one year of age at the time of admission. The average time of hospital stay was 2 days. 83.5% of vascular anomalies were hemangiomas and 83,6 % of these affected the skin and subcutaneous tissue. 64% of hemangiomas were tuberous and most were located on the head and neck. 11% affected multiple sites and/or were associated with other vascular anomalies. Conclusion: This audit allows us to improve the diagnosis, methods of treatment and improving our clinic's recording. Our results stress on the necessity for implementation of educational programs among the people living in the countryside, so that they would become aware of the importance of addressing the physician for their children's health concerns. [ABSTRACT FROM AUTHOR]

Klippel-Trenaunay Syndrome (KTS) is a rare congenital malformation consisting of venous, capillary and lymphatic abnormalities, in addition to hypertrophy and overgrowth of the bony and/or soft tissue of the affected limbs. We report a unique case of KTS due to its complex association of complications (hydronephrosis, rectorrhagia and splenomegaly). [ABSTRACT FROM AUTHOR]

Aim. In COVID-19 Pandemic, a new hyperinflammatory syndrome was reported with clinical features of Kawasaki disease, named PIMS-TS. We want to present a single center experience where the patients were diagnosed with Kawasaki-like in PIMS-TS with cardiac affliction. Material and method: The study was observational and retrospective, enrolled 14 patients fulfilling the criteria of PIMS-TS with the median age of 9 (IQR, 1.6-11), 9 male (64.2 %) and 5 female (35.8 %). Results: ECG revealed tachycardia and ST-T changes in 60% of patients. In evolution, ECG modified in 20% and consisted of long QT in 7% of cases, bradycardia in 7%, 3% transitory sick sinus syndrome and 3% grade I/II degree atrio-ventricular block. Cardiac disfunction was evidenced in 4 patients (28%), with reduced ejection fraction under 50%, mitral insufficiency in 6 (42.8%), pericardial fluid in 8 (57.1%) and perivascular brightness in 8 (57.1%). The cardiac biomarkers: NT-proBNP (increased in 9), cTroponin T (increased in 7) and cTroponin I (increased in 5) confirmed heart dysfunction. During the hospitalization and under medical treatment, all the modifications recover. Evolution was good for 12 children. Conclusions: Cardiac dysfunction and myocardial injury were confirmed by elevated cardiac biomarkers. Rapid recognition allows prompt treatment for a good outcome. NT-proBNP, cTroponin T and I are of capital significance in monitoring the myocardial injury, the treatment and evolution of these patients. [ABSTRACT FROM AUTHOR]

Background/aim: Biomaterials are essential in modern medicine, both for patients and research. Their ability to acquire and maintain functional vascularization is currently debated. The aim of this study was to evaluate the vascularization induced by two collagen-based scaffolds (with 2D and 3D structures) and one non-collagen scaffold implanted on the chick embryo chorioallantoic membrane (CAM)., Materials and Methods: Classical stereomicroscopic image vascular assessment was enhanced with the IKOSA software by using two applications: the CAM assay and the Network Formation Assay, evaluating the vessel branching potential, vascular area, as well as tube length and thickness., Results: Both collagen-based scaffolds induced non-inflammatory angiogenesis, but the non-collagen scaffold induced a massive inflammation followed by inflammatory-related angiogenesis. Vessels branching points/Region of Interest (Px^2) and Vessel branching points/Vessel total area (Px^2), increased exponentially until day 5 of the experiment certifying a sustained and continuous angiogenic process induced by 3D collagen scaffolds., Conclusion: Collagen-based scaffolds may be more suitable for neovascularization compared to non-collagen scaffolds. The present study demonstrates the potential of the CAM model in combination with AI-based software for the evaluation of vascularization in biomaterials. This approach could help to reduce and replace animal experimentation in the pre-screening of biomaterials., (Copyright © 2024, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized., Objectives: The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years., Methods: Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years., Results: At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age., Conclusions: Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages., Competing Interests: Funding Support and Author Disclosures This work was supported by the British Heart Foundation (grant FS/16/72/32270) to Drs Norrish and Kaski. This work is (partly) funded by the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. Dr Norrish is supported by Great Ormond Street Hospital Children’s Charity. Drs Field and Kaski are supported by Max’s Foundation and Great Ormond Street Hospital Children’s Charity. Dr Kaski is supported by a Medical Research Council–National Institute for Health Research Clinical Academic Research Partnership award. This work was financially supported by the Foundation for Paediatric Research of Finland (Dr Ojala). Dr Fernandez has received speaker fees from Sanofi-Genzyme. Dr Kubus is supported by MH CZ – DRO, Motol University Hospital (00064203). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Aims: Children presenting with hypertrophic cardiomyopathy (HCM) in infancy are reported to have a poor prognosis, but this heterogeneous group has not been systematically characterized. This study aimed to describe the aetiology, phenotype, and outcomes of infantile HCM in a well-characterized multicentre European cohort., Methods and Results: Of 301 children diagnosed with infantile HCM between 1987 and 2019 presenting to 17 European centres [male n = 187 (62.1%)], underlying aetiology was non-syndromic (n = 138, 45.6%), RASopathy (n = 101, 33.6%), or inborn error of metabolism (IEM) (n = 49, 16.3%). The most common reasons for presentation were symptoms (n = 77, 29.3%), which were more prevalent in those with syndromic disease (n = 62, 61.4%, P < 0.001), and an isolated murmur (n = 75, 28.5%). One hundred and sixty-one (53.5%) had one or more co-morbidities. Genetic testing was performed in 163 (54.2%) patients, with a disease-causing variant identified in 115 (70.6%). Over median follow-up of 4.1 years, 50 (16.6%) underwent one or more surgical interventions; 15 (5.0%) had an arrhythmic event (6 in the first year of life); and 48 (15.9%) died, with an overall 5 year survival of 85%. Predictors of all-cause mortality were an underlying diagnosis of IEM [hazard ratio (HR) 4.4, P = 0.070], cardiac symptoms (HR 3.2, P = 0.005), and impaired left ventricular systolic function (HR 3.0, P = 0.028)., Conclusions: This large, multicentre study of infantile HCM describes a complex cohort of patients with a diverse phenotypic spectrum and clinical course. Although overall outcomes were poor, this was largely related to underlying aetiology emphasizing the importance of comprehensive aetiological investigations, including genetic testing, in infantile HCM., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Atrioventricular septal defect (AVSD) is a cardiac malformation that accounts for up to 5% of total congenital heart disease, occurring with high frequency in people with Down Syndrome (DS). We aimed to establish the surgical challenges and outcome of medical care in different types of AVSD in children with DS compared to those without DS (WDS). Methods: The study included 62 children (31 with DS) with AVSD, evaluated over a 5 year period. Results: Complete AVSD was observed in 49 (79%) children (27 with DS). Six children had partial AVSD (all WDS) and seven had intermediate types of AVSD (4 with DS). Eight children had unbalanced complete AVSD (1 DS). Median age at diagnosis and age at surgical intervention in complete AVSD was not significantly different in children with DS compared to those WDS (7.5 months vs. 8.6). Median age at surgical intervention for partial and transitional AVSDs was 10.5 months for DS and 17.8 months in those without DS. A large number of patients were not operated: 13/31 with DS and 8/31 WDS. Conclusion: The complete form of AVSD was more frequent in DS group, having worse prognosis, while unbalanced AVSD was observed predominantly in the group without DS. Children with DS required special attention due to increased risk of pulmonary hypertension. Late diagnosis was an important risk factor for poor prognosis, in the setting of suboptimal access to cardiac surgery for patients in Romania. Although post-surgery mortality was low, infant mortality before surgery remains high. Increased awareness is needed in order to provide early diagnosis of AVSD and enable optimal surgical treatment., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Olariu, Popoiu, Ardelean, Isac, Steflea, Olariu, Chirita-Emandi, Stroescu, Gafencu and Doros.)