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Cyanotic critical congenital heart disease (CCHD) is a serious condition associated with high rate of morbidity and mortality. This review highlights key aspects of CCHD, including presentation, diagnosis, and management in the emergency department (ED), based on current evidence. CCHD encompasses several conditions, including truncus arteriosus, dextro-transposition of the great arteries (d-TGA), tricuspid atresia, tetralogy of Fallot (TOF), total anomalous pulmonary venous return (TAPVR), Ebstein's anomaly, critical pulmonary stenosis, and hypoplastic left heart syndrome (HLHS). These conditions should be considered in critically ill neonates presenting with shock and cyanosis. Evaluation involves a thorough assessment of the patient's cardiovascular and respiratory status. Diagnostic tools include pulse oximetry, laboratory testing, electrocardiogram, chest radiography, and ultrasonography. Management requires prompt recognition and hemodynamic stabilization, which may involve the administration of antibiotics, prostaglandins, vasoactive/inotropic agents, and airway management. Expert consultation and coordination of transfer to a tertiary or quaternary care center are crucial. Surgical repair serves as the definitive treatment. A thorough understanding of cyanotic congenital heart disease is essential for emergency clinicians to effectively diagnose and manage this potentially life-threatening condition.

Systemic chronic hypoxia is a feature of many diseases and may influence the communication between bone marrow (BM) and gut microbiota. Here we analyse patients with cyanotic congenital heart disease (CCHD) who are experiencing chronic hypoxia and characterize the association between bone marrow mesenchymal stem cells (BMSCs) and gut microbiome under systemic hypoxia. We observe premature senescence of BMSCs and abnormal d -galactose accumulation in patients with CCHD. The hypoxia that these patients experience results in an altered diversity of gut microbial communities, with a remarkable decrease in the number of Lactobacilli and a noticeable reduction in the amount of enzyme-degraded d -galactose. Replenishing chronic hypoxic rats with Lactobacillus reduced the accumulation of d -galactose and restored the deficient BMSCs. Together, our findings show that chronic hypoxia predisposes BMSCs to premature senescence, which may be due to gut dysbiosis and thus induced d -galactose accumulation. Systemic chronic hypoxia is a feature of many diseases and may influence the communication between bone marrow and gut microbiota. Here, the authors show that chronic hypoxia predisposes bone marrow stem cells to premature senescence, which may be due to gut dysbiosis and gut microbiota-derived d -galactose accumulation.

Background Apgar score and cyanosis assessment may disadvantage darker-skinned babies. This review explored cyanosis and Apgar score assessments in Black, Asian, or minority ethnic neonates compared to White neonates. Material and methods Four databases were searched. Studies of any methodology were included. A narrative synthesis was undertaken. Results Ten studies were included. Three studies involving over 39 million neonates showed Apgar score ≤3 was predictive of neonatal mortality across all ethnicities. Black babies with Apgar score ≤3 had lower mortality rates before 28 days, however, variations in scoring practices were also observed. Three further studies ( n  = 39,290,014) associated low Apgar scores with poorer mental development up to 22 months, especially in mixed ethnicity and Black infants. One study reported inadequate training in assessing ethnic minority neonates. Cyanosis was the focus of three included studies ( n  = 455) revealing poor visual assessment of cyanosis across ethnicities. With pulse oximetry occult hypoxemia occurred slightly more frequently in Black neonates. Tongue color indicated oxygen requirement at birth, regardless of ethnicity. Conclusions Apgar scores correlate well with neonatal mortality in all ethnicities, however scoring variations exist. Cyanosis assessment is challenging, with tongue and lips the best places to observe in the absence of pulse oximetry. Impact Assessment of the color component of the Apgar score and of cyanosis visually are not accurate in babies with darker skin. Small racial differences may exist for pulse oximetry in neonates, but it is more reliable than visual assessment.

Tissue hypoxia increases erythropoietin production and release of immature erythrocytes that can be measured using nucleated red blood cell counts (nRBC). We hypothesized that hypoxia due to congenital heart disease (CHD) is chronic and is better tolerated than hypoxia due to respiratory disease (RD), which is an acute stress in newborns leading to higher nRBC. This study assesses the utility of nRBC as a marker to differentiate hypoxia due to CHD vs RD in term neonates. This was a single-center, retrospective study of term neonates with cyanosis from 2015 to 2022. Neonates < 37 weeks of gestation, with hypoxic-ischemic encephalopathy, and those with other causes of cyanosis were excluded. The patients were divided into 2 groups: cyanotic CHD and cyanotic RD. Clinical and laboratory data done within 12 h and 24–36 h after birth were collected. Data are represented as median and Interquartile range. Of 189 patients with cyanosis, 80 had CHD and 109 had RD. The absolute nRBC count at ≤ 12 h of age was lower in the CHD (360 cells/mm 3 ) compared to RD group (2340 cells/mm 3 ) despite the CHD group having significantly lower baseline saturations. A value of 1070 cells/mm 3 was highly sensitive and specific for differentiating CHD from RD. The positive predictive value for this cut-off value of 1070 cells/mm 3 was 0.94 and the negative predictive value was 0.89. The absolute nRBC is a simple screening test and is available worldwide. A nRBC < 1070 cells/mm 3 in cyanotic newborns should hasten the search for CHD etiology with the possible need for prostaglandin therapy.

Background To investigate the occurrence of reversed differential cyanosis (RDC) in case of (supra)cardiac total anomalous pulmonary venous return (TAPVR), we explored the hemodynamic changes and oxygen saturation levels during the fetal-to-neonatal transition in (supra)cardiac TAPVR, thereby revealing determinant factors of RDC. Methods A computational model was used to simulate the cardiovascular fetal-to-neonatal transition up to 24 h after birth. Abnormalities associated with TAPVR, like patent ductus arteriosus (PDA) and persistent pulmonary hypertension of the neonate (PPHN), were imposed on the model. Hemodynamic impact on flow distribution and right-sided pressures as well as oxygen saturations were assessed. Results Model findings demonstrated that RDC in (supra)cardiac TAPVR was dependent on two key factors: (1) the type of pulmonary venous connection being supracardiac or cardiac, and (2) the presence of a patent ductus arteriosus exhibiting right-to-left shunting. Persistence of RDC was mainly determined by the latter; an increase in pulmonary-to-systemic pressure difference by PPHN or PDA-induced pulmonary over-circulation contributed to persistence of RDC. Conclusion This study highlights the significance of RDC in (supra)cardiac TAPVR and suggests to incorporate early screening ( < 24 h after birth) and to consider RDC as an immediate fail in screening protocols to ensure prompt detection of (supra)cardiac TAPVR. Impact Utilizing a validated computational model for the cardiovascular fetal-to-neonatal transition, this study sheds light on the complex hemodynamics in neonates with (supra)cardiac Total Anomalous Pulmonary Venous Return (TAPVR). Model findings suggest that the often-present pulmonary over-circulation in neonates with TAPVR might significantly contribute to the anomaly’s frequent omission during pulse-oximetry screening beyond the first 24 h after birth. This study highlights the diagnostic value of reversed differential cyanosis in early screenings within the first 24 h after birth. By including RDC as an immediate fail in early pulse-oximetry screenings, the likelihood of missing (supra)cardiac TAPVR cases could be reduced.

Adults with cyanotic congenital heart diseases (CCHD) have a higher risk for bleeding, but also for thrombosis. Rotational thromboelastometry (RT), using tissue factor (EXTEM), a contact activator (INTEM) or cytochalasin (FIBTEM), assesses coagulation by determining the time to initiation of clotting (CT) and clot firmness (MCF) including platelet-fibrin-interaction. The aim of this study was to evaluate RT and whole blood impedance aggregometry (IA) in CCHD compared with a control group without chronic cyanosis (NCCHD). These were used to establish normal reference ranges. We prospectively included 124 patients (76 CCHD, 48 NCCHD). Mean oxygen saturation in CCHD was 81.5%, and 98% in NCCHD (p <0.001). Fifty-five CCHD and 1 NCCHD had pulmonary hypertension. Eisenmenger syndrome was present in 39 CCHD (51.3%). Hemoglobin, hematocrit, and reticulocyte levels were significantly higher in CCHD, and they also showed more thrombocytopenia. Platelet aggregation was under normal range in 89.5% of CCHD after triggering with ADP, in 85.5% after triggering with arachidonic acid (ASPI) and in 73.7% after TRAP-6. RT showed significantly longer clotting times and reduced clot firmness in both EXTEM and INTEM tests. FIBTEM-MCF was also significantly reduced. Moderate inverse correlation was found between platelet count and erythrocytes (r = −0.608, p <0.001). Significant correlations were found between platelet number and RT-parameters as well as with all IA parameters. In conclusion, according to RT and IA, CCHD present hypocoagulable disorders. No signs of hypercoagulability were found.

Dye and nitro-compound pollution has become a significant issue worldwide. The adsorption and degradation of dyes and nitro-compounds have recently become important areas of study. Different methods, such as precipitation, flocculation, ultra-filtration, ion exchange, coagulation, and electro-catalytic degradation have been adopted for the adsorption and degradation of these organic pollutants. Apart from these methods, adsorption, photocatalytic degradation, and chemical degradation are considered the most economical and efficient to control water pollution from dyes and nitro-compounds. In this review, different kinds of dyes and nitro-compounds, and their adverse effects on aquatic organisms and human beings, were summarized in depth. This review article covers the comprehensive analysis of the adsorption of dyes over different materials (porous polymer, carbon-based materials, clay-based materials, layer double hydroxides, metal-organic frameworks, and biosorbents). The mechanism and kinetics of dye adsorption were the central parts of this study. The structures of all the materials mentioned above were discussed, along with their main functional groups responsible for dye adsorption. Removal and degradation methods, such as adsorption, photocatalytic degradation, and chemical degradation of dyes and nitro-compounds were also the main aim of this review article, as well as the materials used for such degradation. The mechanisms of photocatalytic and chemical degradation were also explained comprehensively. Different factors responsible for adsorption, photocatalytic degradation, and chemical degradation were also highlighted. Advantages and disadvantages, as well as economic cost, were also discussed briefly. This review will be beneficial for the reader as it covers all aspects of dye adsorption and the degradation of dyes and nitro-compounds. Future aspects and shortcomings were also part of this review article. There are several review articles on all these topics, but such a comprehensive study has not been performed so far in the literature.