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  • Prevalence of Bicuspid Aortic Valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis
  • Abstract Turner syndrome (TS) is a rare disorder affecting 25–50 in 100000 female newborns. Bicuspid aortic valve (BAV) is assumed to be the most common congenital heart defect (CHD) in TS. In literature, reported BAV prevalence in TS ranges between 14% and 34%. The specific BAV prevalence in TS is still unknown. The aim of this study was to give a more precise estimation of BAV prevalence in TS by conducting a meta-analysis of TS-studies, which detected BAV by either cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT). We searched PubMed, Cochrane Library, and Web of Science databases to…
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  • Modification of Serum Galectin-3 and Reversal of Cardiac Remodeling Following Pediatric Transcatheter Atrial Septal Defect Closure
  • Abstract Objectives: We aimed to evaluate the effect of percutaneous atrial septal defect (ASD) closure in children using right heart indices and serum galectin-3 (Gal-3), as potential biomarkers of right heart remodeling. Methods: This case–control prospective study included 40 children with ASD and 25 control subjects. An echocardiographic evaluation was performed before the procedure, as well as 24 h, 1 month, and 6 months after intervention. Serum Gal-3 was measured before, and 1 month after the procedure. Results: Serum Gal-3 concentration, right atrial (RA) dimensions, right ventricular (RV) dimensions, indexed RA area, and right index of myocardial performance (RIMP) were significantly…
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  • 2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping
  • Abstract A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome.
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  • Pulmonary Perfusion Asymmetry in Patients after Repair of Tetralogy of Fallot: A 4D Flow MRI-Based Study
  • Abstract Background: Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). While MPA stenosis is well studied, few data are available on BPAS in rTOF. We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR. Methods: 130 consecutive patients (mean age at CMR 14.3 ± 4.6 years) were retrospectively reviewed. 96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit. A pulmonary blood flow ratio (right pulmonary artery (RPA)/left…
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  • Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect
  • Abstract Background: Partial anomalous pulmonary venous connection (PAPVC) is frequently associated with atrial septal defect (ASD), especially sinus venosus defect (SVD). Although Waggstaffe described the pathology of SVDs in 1868, the exact anatomic features and the nature of SVD remains controversial. SVDs with no posterior atrial rim were observed in recent years. However, no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD. The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD. Methods: From January 2011 to…
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  • A Rare Case of Infective Mediastinitis after Melody Valve Implantation
  • Abstract Pulmonary valve implant is frequently necessary in children and adults with congenital heart disease. Infective endocarditis represents a rare but life-threatening complication after transcatheter pulmonary valve implantation. There are various treatments for native or prosthetic valve endocarditis. Surgical intervention, combined with intravenous antibiotic treatment, is of paramount importance, in case of concomitant mediastinal infection, in order to ensure the radical debridement of all infected tissue, avoiding any recurrent endocarditis. In this report, we describe a rare case of mediastinitis, associated with an infected endocarditis, occurring 8 months after Melody (Medtronic®, Minneapolis, USA) valve implant, successfully treated with the implantation of…
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  • Transcatheter Device Closure of a Perimembranous Ventricular Septal Defect in Congenitally Corrected Transposition of the Great Arteries
  • Abstract The majority of patients with congenitally corrected transposition of the great arteries, also known as transposition of the great arteries {S,L,L} have ventricular septal defects (VSD), most commonly perimembranous VSD (pmVSD). Transcatheter device closure of pmVSD in these patients has not been widely described. We present a case of device closure of pmVSD in L-TGA with an Amplatzer Duct Occluder II (ADOII) device using a deployment starting in the subpulmonary left ventricle. The case demonstrates some of the technical advantages of the ADOII device for VSD closure, specifically its low profile, symmetric shape, and soft material. These characteristics are advantageous…
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  • Pregnancy in Patients with Shone Complex: A Single-Center Case Series
  • Abstract Background: There is limited literature written on the course and outcomes for pregnant mothers with Shone complex. Methods: We describe a case series of five pregnancies in four women with Shone complex within a multidisciplinary cardio-obstetrics clinic from 2016–2018. Results: Maternal age ranged from 21–39 years. Three patients had preserved left ventricular function while one had moderately decreased function. Gestational age at presentation ranged from 6–15 weeks. There were three successful pregnancies (mean gestational age = 37 weeks, range 35–39 weeks) with one patient accounting for two unsuccessful pregnancies. All infants were delivered via Cesarean section. One infant required a…
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  • Medium-Term Results of Balloon Valvuloplasty of Native Pulmonary Valve Stenosis with and without Supravalvular Obstruction in Childhood
  • Abstract Objectives: Factors influencing results of balloon valvuloplasty (BVP) of pulmonary valve stenosis (PS) in children are investigated. Background: BVP has become the standard of care for PS, medium-term results are not uniform and depend on various preconditions. Methods: We analysed the medium-term results of BVP of PS in children in an observational, single centre study. Need for additional procedure was defined as outcome after initial BVP. Results: We included 143 children (83 female) at a median (IQR) age of 2.6 (0.26–9.24) months and body weight of 5 (3.4–8) kg at BVP with a follow–up of 5.04 (1.6–10.2) years. We used…
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