Ebstein's anomaly in children and young adults: clinical features, arrhythmia, surgical management, and factors affecting arrhythmia and mortality.

Background: Ebstein's anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein's anomaly patients are limited.

Aim: To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein's anomaly patients, and to determine factors affecting arrhythmia presence and mortality.

Topsy-Turvy Heart: Exceedingly Rare Congenital Rotational Heart Disease With Large Aorticopulmonary Window and Severe Tracheobroncial Malacia.

Left images: Top: (A) Echocardiography shows a dilated pulmonary artery, large aortopulmonary window (dotted line), and abnormally positioned aortic arch. (B) MIP image reveals superior RV, inferior LV, and elongated arch vessels (arrows). Bottom: MinIP shows a thin left main bronchus and non-aerated RML (asterisk).

Examining the Role of Exercise Training in Enhancing Life for Adult Congenital Heart Disease: Systematic Review.

Background: Current guidelines advise exercise for most congenital heart disease patients (CHD). However, physical activity remains low in CHD individuals, with limited research on exercise's effects in adults.

Objectives: The aim of this study is to evaluate the safety and efficacy of exercise training on exercise capacity and quality of life in adult congenital heart disease (ACHD) patients.

Evaluation of cardiac function using echocardiography in childhood-onset systemic lupus erythematosus patients treated with hydroxychloroquine.

Background: This study aimed to evaluate the effects of hydroxychloroquine on cardiac functions and left ventricular mass in patients with childhood-onset systemic lupus erythematosus (cSLE).

Research Design And Methods: Fifty patients with cSLE undergoing treatment with hydroxychloroquine underwent echocardiographic evaluation. All patients exhibited negative disease activity markers and were clinically in remission.

Temporal Dynamics and (Para)Clinical Factors Associated With (Long) Viral RNA Shedding in COVID-19 Nonhospitalized Individuals - The COVID-HOME Study.

Understanding temporal patterns and determinants of RNA shedding is important to comprehend SARS-CoV-2 transmission and improve biosafety/isolation guidelines. Nonhospitalized SARS-CoV-2-infected individuals and household members were enrolled between March 2020 and June 2021 and followed prospectively ≥ 3 weeks during acute disease and at 3-, 6-, 12-, and 18-months to obtain (para)clinical data and biospecimens. Flow cytometry-based surrogate assay (FlowSA) detected viable SARS-CoV-2.

Multimodal Prediction of Obsessive-Compulsive Disorder and Comorbid Depression Severity and Energy Delivered by Deep Brain Electrodes.

To develop reliable, valid, and efficient measures of obsessive-compulsive disorder (OCD) severity, comorbid depression severity, and total electrical energy delivered (TEED) by deep brain stimulation (DBS), we trained and compared random forests regression models in a clinical trial of participants receiving DBS for refractory OCD. Six participants were recorded during open-ended interviews at pre- and post-surgery baselines and then at 3-month intervals following DBS activation. Ground-truth severity was assessed by clinical interview and self-report.

The Impact of Gastric Leptin and Leptin Receptor Expression on Metabolic Outcomes Post Laparoscopic Sleeve Gastrectomy.

Purpose: Obesity is a growing global crisis, with predictions that nearly half of the world's population will be overweight or with severe obesity by 2030. Metabolic bariatric surgery, effective for those unable to lose weight through conventional methods, results in significant weight loss and alleviates associated medical problems. This study investigates changes in gastric leptin (LE) and leptin receptor (LER) expression following laparoscopic sleeve gastrectomy (LSG) and explores their metabolic contributions to surgical outcomes.

Challenging clinical management of a patient with Gaucher disease type IIIC homozygous for the D409H mutation, aortic valve calcification and porcelain aorta.

Background: Gaucher disease is a rare lysosomal storage disorder caused by glucocerebrosidase enzyme deficiency resulting in the cumulative deposition of glucocerebroside in macrophages, predominantly effecting bone marrow, liver and spleen. Gaucher disease type IIIC is a rare subtype that is characterized by cardiovascular involvement, eye-movement disorders, and late-onset neurological symptoms.

Case Presentation: We present a 14-year-old adolescent boy diagnosed with Gaucher disease type IIIC at age four with a homozygous D409H mutation who developed severe aortic valve stenosis, extensive aortic calcification and a porcelain aorta despite enzyme replacement treatment since the diagnosis.

Exploring frailty: muscle strength, functional capacity, activities of daily living and cognition in adult congenital heart disease.

Purpose: The study aimed to assess frailty in adults with congenital heart disease (ACHD) and to compare muscle strength, functional capacity, activities of daily living (ADL), and cognition between frail and non-frail ACHD patients.

Materials And Methods: A cross-sectional study design was used. Sixty people with ACHD aged between 18 and 45 years were included.

Are adolescents with premenstrual disorder at risk for cardiac arrhythmias?

Background: One of the most commonly experienced symptoms of premenstrual disorder (PMD) is anxiety, and there is a notable rise in sympathomimetic activity in this patient group. Studies have linked fluctuations in systemic autonomic tone to electrocardiography (ECG) changes. This study aims to investigate the relationship between anxiety, a common symptom of PMD, and alterations in QT dispersion (QTd) and P-wave dispersion (Pd) in adolescent females.

Left Ventricular Pressure-Volume Loop Analyses for Donor Hearts: Proof of Concept in an Ovine Experimental Model.

heart perfusion (ESHP) has emerged as an important strategy to preserve donation after brain death (DBD) and donation after circulatory death (DCD) donor hearts. Clinically, both DBD and DCD hearts are successfully preserved using ESHP. Viability assessment is currently based on biochemical values, while a reliable method for graft function assessment in a physiologic working mode is unavailable.

Investigation of four nickel titanium endodontic instruments, with cyclic fatigue resistance, scanning electron microscopy, and energy dispersive x-ray spectroscopy.

The aim of this study was to compare of four different nickel-titanium (Ni-Ti) endodontic files and evaluate in terms of cyclic fatigue resistance and metallurgical properties. Four different type Ni-Ti root canal files Protaper Next X2 (PTN) (Dentsply Maillefer, Ballaigues, Switzerland), One Curve (OC) #25.06 (Micro Mega, Besancon, France), EndoPlus Flex Plus Gold X2 (EPG) (Turkuaz Dental, Denizli, Turkey), and EndoPlus Flex Plus Blue #25.

Dihydropyrimidinase deficiency with atrioventricular septal defect: a case report.

Objectives: Dihydropyrimidinase deficiency is a rare autosomal recessive disorder of the pyrimidine degradation pathway, with fewer than 40 patients published. Clinical findings are variable and some patients may remain asymptomatic. Global developmental delay and increased susceptibility to 5-fluorouracil are commonly reported.

Efficacy of Alkaline Phosphatase in Critically Ill Patients with COVID-19: A Multicentre Investigator-Initiated Double-Blind Randomised Placebo-Controlled Trial.

Background: Efforts to identify therapies to treat hospitalised patients with COVID-19 are being continued. Alkaline phosphatase (AP) dephosphorylates pro-inflammatory adenosine triphosphate (ATP) into anti-inflammatory adenosine.

Methods: In a randomised controlled trial, we investigated the safety and efficacy of AP in patients with SARS-CoV-2 infection admitted to the ICU.

Eliminating first trimester combined testing: Consequences for early detection of significant fetal anomalies.

Objective: To determine whether implementation of cell-free DNA (cfDNA) testing for aneuploidy as a first-tier test and subsequent abolition of first trimester combined testing (FCT) affected the first trimester detection (<14 weeks) of certain fetal anomalies.

Methods: We performed a geographical cohort study in two Fetal Medicine Units between 2011 and 2020, including 705 fetuses with prenatally detected severe brain, abdominal wall and congenital heart defects. Cases were divided into two groups: before (n = 396) and after (n = 309) cfDNA introduction.

Interventional cardiac catheterization in neonates and premature infants with congenital heart disease: a single center experience.

Background: The increased survival of patients with congenital heart disease over the last three decades has been associated with improvements in diagnosis and treatment. This study aimed to evaluate therapeutic interventional catheterization, outcomes and complications of these procedures in neonates and premature infants.

Methods: In this study, therapeutic catheterization procedures performed on neonates and premature infants with congenital heart disease at a university hospital between February 2000 and October 2019 were retrospectively evaluated.

Percutaneous Transcatheter Retrieval of Central Venous Port Fragments in Pediatric Patients; A Single-center Experience From the Pediatric Cardiology Department.

Background: Split/fracture and embolization of central venous/shunt catheters are rare but serious complications in children. Percutaneous retrieval of intravascular foreign bodies is an important minimal invasive treatment. This study is aimed to represent our largest pediatric sample experience till now of 17 years from a single institution.

Investigation of cardiopulmonary parameters, motor development and muscle strength in children with Down syndrome with and without congenital heart disease.

Background: Children with Down syndrome (DS) frequently have concomitant clinical problems. There are no studies in the literature evaluating gross motor development and handgrip strength in the presence of congenital heart disease (CHD), which is one of the most common comorbidities in population with DS. The aim of this study was to compare cardiopulmonary parameters, gross motor development and handgrip strength in children with DS with and without CHD.

Acute kidney injury in children with moderate-severe COVID-19 and multisystem inflammatory syndrome in children: a referral center experience.

Background: Data on the characteristics of acute kidney injury (AKI) in pediatric COVID-19 and MIS-C are limited. We aimed to define the frequency, associated factors and early outcome of AKI in moderate, severe or critical COVID-19 and MIS-C; and to present a tertiary referral center experience from Türkiye.

Methods: Hospitalized patients ≤ 18 years of age with confirmed COVID-19 or MIS-C at İhsan Doğramacı Children's Hospital, Hacettepe University, between March 2020-December 2021 were enrolled.

ORAI1 defect in a patient with disseminated CMV infection and severe hypotonia.

Background: A clinical presentation similar to severe combined immunodeficiency (SCID) with defective T cell activation but normal lymphocyte development occurs due to certain molecule defects including ORAI1- and STIM1.

Case: A four-month-old girl sufferd from fever, restlessness, diarrhea, and poor weight gain following the neonatal period. There was consanguinity and a positive family history.

Comparison of transanal and transvaginal specimen extraction in laparoscopic colorectal surgery.

Aim: This study aimed to compare the outcomes of transanal and transvaginal NOSES in patients undergoing laparoscopic colorectal surgery.

Material And Methods: This study included 45 patients who were scheduled for NOSES after undergoing laparoscopic colorectal resection in our clinic between January 2019 and March 2020. To ensure homogeneity between the groups, the data of 22 female patients were analyzed in this study.

Cardiac rhabdomyomas: clinical progression, efficacy and safety of everolimus treatment.

Background: Primary cardiac tumors are extremely rare. Cardiac rhabdomyoma is the most common primary cardiac tumor. 50-80% of solitary rhabdomyomas and all multiple rhabdomyomas are associated with tuberous sclerosis complex.

Ex-situ oxygenated hypothermic machine perfusion in donation after circulatory death heart transplantation following either direct procurement or in-situ normothermic regional perfusion.

Background: Heart transplantation in donation after circulatory death (DCD) relies on warm perfusion using either in situ normothermic regional perfusion (NRP) or ex situ normothermic machine perfusion. In this study, we explore an alternative: oxygenated hypothermic machine perfusion (HMP) using a novel clinically applicable perfusion system, which is compared to NRP with static cold storage (SCS).

Methods: In a porcine model, a DCD setting was simulated, followed by either (1) NRP and SCS (2) NRP and HMP with the XVIVO Heart preservation system or (3) direct procurement (DPP) and HMP.

Additive Manufactured Strain Sensor Using Stereolithography Method with Photopolymer Material.

As a result of the developments in additive manufacturing (AM) technology, 3D printing is transforming from a method used only in rapid prototyping to a technique used to produce large-scale equipment. This study presents the fabrication and experimental studies of a 3D-printed strain sensor that can be used directly in soft applications. Photopolymer-based conductive and flexible ultraviolet (UV) resin materials are used in the fabrication of the sensor.