Perioperative GABA Blood Concentrations in Infants with Cyanotic and Non-Cyanotic Congenital Heart Diseases

Perioperative stress detection in children with congenital heart disease (CHD), particularly in the brain, is still limited. Among biomarkers, γ-amino-aminobutyric acid (GABA) assessment in biological fluids appears to be promising for its regulatory action on the cardiovascular and cerebral systems. We aimed to investigate cyanotic (C) or non-cyanotic (N) CHD children for GABA blood level changes in the perioperative period. We conducted an observational study in 68 CHD infants (C: n = 33; N: n = 35) who underwent perioperative clinical, standard laboratory and monitoring parameter recordings and GABA assessment. Blood samples were drawn at five predetermined time-points before, during and after surgery. No significant perioperative differences were observed between groups in clinical and laboratory parameters. In C, perioperative GABA levels were significantly lower than N. Arterial oxygen saturation and blood concentration significantly differed between C and N children and correlated at cardiopulmonary by-pass (CPB) time-point with GABA levels. The present data showing higher hypoxia/hyperoxia-mediated GABA concentrations in C children suggest that they are more prone to perioperative cardiovascular and brain stress/damage. The findings suggest the usefulness of further investigations to detect the “optimal” oxygen concentration target in order to avoid the side effects associated with re-oxygenation during CPB.

The role of MDCT in the assessment of cardiac and extra-cardiac vascular defects among Egyptian children with tetralogy of Fallot and its surgical implementation

Background Tetralogy of Fallot (TOF) is considered the most common form of cyanotic congenital heart diseases (CHD), accounting for about 10% of cases. It includes four main cardiac defects, in addition to various extra-cardiac anomalies. Aim This study aimed to evaluate cardiac and extra-cardiac vascular defects associated with TOF among Egyptian children, regarding frequency and types with assessment of multi-slice or multi-detector computed tomography (MDCT) role in their diagnosis. Definitely, full detection of these vascular anomalies has utmost importance when evaluating such patients particularly before surgical intervention. Methods This study included 60 pediatric patients diagnosed as TOF, who underwent MDCT examination in our institute during period of 6 months from (March to September 2020), to confirm their trans-thoracic echocardiography (TTE) findings and detect other vascular abnormalities which cannot be precisely detected with TTE before their surgical interventions. Results The incidence of different extra-cardiac vascular defects diagnosed by MDCT among our patients was 85% which was significantly higher than that detected by TTE (55%). Moreover, MDCT was superior to TTE assessment as regards its diagnostic accuracy (96.6% vs. 80%), sensitivity (98% vs. 76.9%), and specificity (88.9% vs. 85.7%), in addition to both positive and negative predictive values. The most common anomalies detected were affecting the pulmonary artery (80%), followed by aorto-pulmonary vessels (45%), then aortic artery (40%), coronary arteries (20%), and lastly vena cava connection (6.7%). Patients’ demographic characteristics and clinical presentations were also presented. Conclusion This study confirmed that many extra-cardiac vascular defects are commonly associating cardiac lesions in TOF and emphasizing the great value of MDCT in their diagnosis. Certainly, proper detection of these anomalies will help decision-making during preoperative evaluation, corrective interventions, and further management of these cases.

The prevalence and types of coronary artery abnormalities in pediatric patients with Tetralogy of Fallot

Objectives: Tetralogy of fallot (TOF) is one of the most common diseases among cyanotic congenital heart diseases which is associated with 2-23% of coronary artery abnormalities. Pre-operation knowledge the anatomy of coronary arteries in patients with TOF eliminates damage to them during surgery and prevents post-operative complications such as myocardial ischemia and heart failure.Materials and methods:This retrospective study was done on all patients with TOF who were referred for diagnostic catheterization and angiography before total surgical correction from 2006-2016. All patients entered the study and all angiographic views including extreme caudal, LAO cranial , and selective coronary artery angiography were evaluated and reviewed accurately.Results: 332 cases of patients with TOF including from one month to 36 years old were reviewed. The prevalence of coronary artery abnormalities among them was 11.4% (38 from 322 individual). 9.1% and 15.3% of males and females had abnormal coronary anomalies but it was not significant statically (p-value: 0.064). In 13 of 38 patients with coronary anomalies , coronary arteries crossed right ventricular out flow tract (RVOT)(3.4%). The most common coronary abnormality was origin of the both main coronary arteries from left sinus of Valsalva.Conclusion:Compared with other populations, the abnormal arteries among the patients with TOF in our study is significant (11.4%) and accurate assessment of their courses is necessary before surgery. Single origin of coronary arteries from the left side was the most common finding in our study too.

Construction and Investigation of a circRNA-Associated ceRNA Regulatory Network in Tetralogy of Fallot

Background: As the most frequent type of cyanotic congenital heart diseases (CHD), Tetralogy of Fallot (TOF) has a relatively poor prognosis without the corrective surgery. Circular RNA (circRNA) represents a novel class of endogenous noncoding RNAs that regulate target gene expression post-transcriptionally in heart development. Here, we investigate the potential role of ceRNA network in the pathogenesis of TOF. Methods: To identify circRNAs expression profiles in Tetralogy of Fallot, microarrays were used to screen the differentially expressed circRNA between 3 TOF and 3 control human myocardial tissue samples. Then, a dysregulated circRNA- associated ceRNA network was constructed via using the established multi-step screening strategy.Results: In summary, total 276 differentially expressed circRNAs were identified, including 214 up-regulated and 62 down-regulated ones in TOF samples. By constructing the circRNA-associated ceRNA network based on the bioinformatics data, a total of 19 key circRNAs, 9 key miRNAs and 34 key mRNAs were further screened. Moreover, by enlarging the samples size, the qPCR results validated that the positive correlations between hsa_circ_0007798 and HIF1A.Conclusions: The findings in this study provide a comprehensive understanding of the ceRNA network involved in TOF biology, such as hsa_circ_0007798/miR-199b-5p/HIF1A signal axis, and may offer candidate diagnostic biomarkers or potential therapeutic targets for TOF. In addtion, we propose that the ceRNA network regulates TOF progression.

Construction and Investigation of a circRNA-Associated ceRNA Regulatory Network in Tetralogy of Fallot

Background: As the most frequent type of cyanotic congenital heart diseases (CHD), Tetralogy of Fallot (TOF) has a relatively poor prognosis without the corrective surgery. Circular RNA (circRNA) represents a novel class of endogenous noncoding RNAs that regulate target gene expression post-transcriptionally in heart development. Here, we investigate the potential role of ceRNA network in the pathogenesis of TOF. Methods: To identify circRNAs expression profiles in Tetralogy of Fallot, microarrays were used to screen the differentially expressed circRNA between 3 TOF and 3 control human myocardial tissue samples. Then, a dysregulated circRNA- associated ceRNA network was constructed via using the established multi-step screening strategy.Results: In summary, total 276 differentially expressed circRNAs were identified, including 214 up-regulated and 62 down-regulated ones in TOF samples. By constructing the circRNA-associated ceRNA network based on the bioinformatics data, a total of 19 key circRNAs, 9 key miRNAs and 34 key mRNAs were further screened. Moreover, by enlarging the samples size, the qPCR results validated that the positive correlations between hsa_circ_0007798 and HIF1A.Conclusions: The findings in this study provide a comprehensive understanding of the ceRNA network involved in TOF biology, such as hsa_circ_0007798/miR-199b-5p/HIF1A signal axis, and may offer candidate diagnostic biomarkers or potential therapeutic targets for TOF. In addtion, we propose that the ceRNA network regulates TOF progression.