Incidence of postoperative seizures in neonates following cardiac surgery with regional cerebral perfusion and deep hypothermic circulatory arrest.

Objectives: Historically, our center has primarily used deep hypothermic circulatory arrest, but in recent years some surgeons have selectively used regional cerebral perfusion as an alternative. We aimed to compare the incidence of postoperative electroencephalographic seizure incidence in neonates undergoing surgery with regional cerebral perfusion and deep hypothermic circulatory arrest.

Methods: A retrospective analysis was performed in neonates who underwent surgery between 2012 and 2022 with either deep hypothermic circulatory arrest or regional cerebral perfusion with routine postoperative continuous electroencephalography monitoring for 48 hours.

Hereditary haemorrhagic telangiectasia and SMAD4 mutation in a patient with complex single ventricle heart disease.

We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient's staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.

Indexed oxygen delivery during pediatric cardiopulmonary bypass is a modifiable risk factor for postoperative acute kidney injury.

Background: Acute kidney injury after pediatric cardiac surgery is a common complication with few established modifiable risk factors. We sought to characterize whether indexed oxygen delivery during cardiopulmonary bypass was associated with postoperative acute kidney injury in a large pediatric cohort.

Methods: This was a retrospective analysis of patients under 1 year old undergoing cardiac surgery with cardiopulmonary bypass between January 1, 2013, and January 1, 2020.

Modeling of the Tricuspid Valve and Right Ventricle in Hypoplastic Left Heart Syndrome With a Fontan Circulation.

Background: In hypoplastic left heart syndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplastic left heart syndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction.

Methods: TV of 100 patients with hypoplastic left heart syndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart.

Right ventricular energy metabolism in a porcine model of acute right ventricular pressure overload after weaning from cardiopulmonary bypass.

Acute right ventricular pressure overload (RVPO) occurs following congenital heart surgery and often results in low cardiac output syndrome. We tested the hypothesis that the RV exhibits limited ability to modify substrate utilization in response to increasing energy requirements during acute RVPO after cardiopulmonary bypass (CPB). We assessed the RV fractional contributions (Fc) of substrates to the citric acid cycle in juvenile pigs exposed to acute RVPO by pulmonary artery banding (PAB) and CPB.

Noonan syndrome associated with hypoplastic left heart syndrome.

Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype-phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with -related Noonan syndrome and hypoplastic left heart syndrome variants.

Reintervention for Superior Vena Cava Obstruction After Heart Transplant.

Background: Children undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction.

Methods: A retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021.

Early Outcomes of Patients Undergoing Neoaortic Valve Repair Incorporating Geometric Ring Annuloplasty.

Objectives: During congenital heart surgery, the pulmonary valve and root may be placed into the systemic position, yielding a "neoaortic" valve. With the stress of systemic pressure, the pulmonary roots can dilate, creating aneurysms and/or neoaortic insufficiency (neoAI). This report analyzes the early outcomes of patients undergoing neoaortic valve repair incorporating geometric ring annuloplasty.

Triiodothyronine Supplementation in Infants Undergoing Cardiopulmonary Bypass: A Randomized Controlled Trial.

Cardiopulmonary bypass (CPB) profoundly suppresses circulating thyroid hormone levels in infants. We performed a multicenter randomized placebo controlled trial to determine if triiodothyronine (T3) supplementation improves reduces time to extubation (TTE) in infants after CPB. Infants (n = 220) undergoing cardiac surgery with CPB and stratified into 2 age cohorts: ≤30 days and >30 days to <152 days were randomization to receive either intravenous triiodothyronine or placebo bolus followed by study drug infusion until extubated or at 48 hours, whichever preceded.

Complete atrioventricular septal defect with absent or diminutive primum component: Incidence, anatomic characteristics, and outcomes.

Background: Repair of complete atrioventricular septal defect with absent or diminutive primum defect is challenging because of atrial septal malposition and abnormal anatomy of the left atrioventricular valve. We sought to define the incidence, anatomy, and surgical outcomes of this entity.

Methods: We identified all patients in our institutional database presenting for complete atrioventricular septal defect repair from 2006 to 2018.

Computational fluid dynamics modeling aiding surgical planning in a toddler with Parkes Weber syndrome.

Parkes Weber syndrome is a fast-flow and slow-flow vascular anomaly with limb overgrowth that can lead to congestive heart failure and limb ischemia. Current management strategies have focused on symptom management with focal embolization. A pediatric case with early onset heart failure is reported.

Shear stress associated with cardiopulmonary bypass induces expression of inflammatory cytokines and necroptosis in monocytes.

Cardiopulmonary bypass (CPB) is required during most cardiac surgeries. CBP drives systemic inflammation and multiorgan dysfunction that is especially severe in neonatal patients. Limited understanding of molecular mechanisms underlying CPB-associated inflammation presents a significant barrier to improve clinical outcomes.

Review: Contemporary biological therapies for cardiovascular diseases.

Cardiovascular diseases are top cause of mortality in the world. Current interventional therapy and pharmacotherapy may alleviate symptoms or slow disease progression but are unable to cure or treat them. Molecular and pathophysiological advances have paved the way for contemporary biological therapies to be tested and standardized for the treatment of these diseases.

Metabolic Response to Stress by the Immature Right Ventricle Exposed to Chronic Pressure Overload.

Background The right ventricle exposed to chronic pressure overload exhibits hypertrophy and decompensates when exposed to stress. We hypothesize that impaired ability to increase myocardial oxidative flux through pyruvate dehydrogenase leads to hypertrophied right ventricular (RV) dysfunction when exposed to hemodynamic stress, and pyruvate dehydrogenase stimulation can improve RV function. Methods and Results Infant male Yorkshire piglets (13.

Small microplastic particles (S-MPPs) in sediments of mangrove ecosystem on the northern coast of the Persian Gulf.

We present a study of small microplastic particles (S-MPPs) in the sediments of mangrove ecosystem of Khor-e- Khoran, a Ramsar site in Iran. The spatial distribution of S-MPPs (<1 mm) in mangrove surface sediments were investigated, which provided new insights into the detection and composition of S-MPPs in the study area. S-MPPs were extracted via the air-induced overflow (AIO) extraction procedure, and then they were counted and categorized according to the particle shape, color and size.

Successful management of absent sternum in an infant using porcine acellular dermal matrix.

Congenital absent sternum is a rare birth defect that requires early intervention for optimal long-term outcomes. Descriptions of the repair of absent sternum are limited to case reports, and no preferred method for management has been described. Herein, we describe the use of porcine acellular dermal matrix to reconstruct the sternum of an infant with sternal infection following attempted repair using synthetic mesh.

Untargeted metabolite profiling and phytochemical analysis of Micromeria fruticosa L. (Lamiaceae) leaves.

Micromeria fruticosa is an important crop, is widely used in the Mediterranean basin as food and in folk medicine, owing to its health-promoting properties, partially due to the secondary metabolite composition. However, complete information on the phyto-metabolites in M. fruticosa is still lacking.

Metabolic Response of the Immature Right Ventricle to Acute Pressure Overloading.

Background: Surgical palliation or repair of complex congenital heart disease in early infancy can produce right ventricular (RV) pressure overload, often leading to acute hemodynamic decompensation. The mechanisms causing this acute RV dysfunction remain unclear. We tested the hypothesis that the immature right ventricle lacks the ability to modify substrate metabolism in order to meet increased energy demands induced by acute pressure overloading.

Microplastics contamination in molluscs from the northern part of the Persian Gulf.

Microplastics (MPs) are well-known emerging contaminants in the marine environment. A key route by which MPs can directly affect marine life is through ingestion. The objective of the present study was to evaluate the occurrence of MPs in marine life and seafood for human consumption in the Persian Gulf.

Homozygous mutation in the NPHP3 gene causing foetal nephronophthisis.

We present a case of a foetal sonographic finding of hyper-echogenic kidneys, which led to a strategic series of genetic tests and identified a homozygous mutation (c.424C > T, p. R142*) in the NPHP3 gene.

Brugada Syndrome: An Electrical Storm without Warning.

Brugada Syndrome (BrS) is an inherited channelopathy causing sodium channel dysfunction in cardiac myocyte. These patients are prone to develop Ventricular Fibrillation (VF) or polymorphic Ventricular Tachycardia (VT). Next to coronary artery disease and cardiomyopathies, BrS is an important cause of sudden cardiac death.

Synthesis and characterization of hybrid materials consisting of n-octadecyltriethoxysilane by using n-hexadecylamine as surfactant and Q(0) and T(0) cross-linkers.

Novel hybrid xerogel materials were synthesized by a sol-gel procedure. n-octadecyltriethoxysilane was co-condensed with and without different cross-linkers using Q(0) and T(0) mono-functionalized organosilanes in the presence of n-hexadecylamine with different hydroxyl silica functional groups at the surface. These polymer networks have shown new properties, for example, a high degree of cross-linking and hydrolysis.