Longitudinal Cardiac Evaluation of Children with Multisystem Inflammatory Syndrome (MIS-C) Following COVID-19 by Conventional and Speckle-Tracking Echocardiography.

Multisystem inflammatory syndrome in children (MIS-C), is a rare but severe, hyperinflammatory complication of COVID-19, in which cardiovascular abnormalities are frequently detected. In this prospective study, we describe the echocardiographic findings in patients with MIS-C, with the use of conventional Echocardiography and Speckle-Tracking Echocardiography (STE) with Left Ventricular (LV) Global Longitudinal Strain (GLS) analysis, in the acute and follow-up phase. In total, 25 MIS-C patients [64% females, mean (± SD) age: 8.3 (± 3.72) years] were included. In the acute phase, median (IQR) Troponin and NT-proBNP and mean heart rate, were 8.07 (14.52) pg/mL, 2875.00 (7713.00) pg/mL, and 102.87 (± 22.96) bpm, respectively. Median (IQR) LV Ejection Fraction (LVEF) was 66 (8)% and LVEF impairment was detected in 2/25 (8%) patients. On follow-up (mean time interval:9.50 ± 4.59 months), heart rate was significantly lower, with a mean value of 90.00 (± 14.56) bpm (p-value = 0.017). Median (IQR) LVEF was 66.00 (6.70)% (p-value = 0.345) and all 25 participants had normal LVEF. In 14/25 patients, additional LV-GLS analysis was performed. During the acute phase, mean LV-GLS was - 18.02 (± 4.40)%. LV-GLS was abnormal in 6/14 patients (42.9%) and among them, only one patient had reduced LVEF. On follow-up (median (IQR) time interval:6.93 (3.66) months), mean LV-GLS was -20.31 (± 1.91)% (p-value = 0.07) and in 1/14 patient (7.1%), the LV-GLS impairment persisted. In conclusion, in the acute and follow-up phase, we detected abnormal LV-GLS values in some patients, in the presence of normal LVEF, indicating that STE-GLS is a valuable tool for identifying subclinical myocardial injury in MIS-C.

Heart Failure Post-SARS-CoV-2 Infection in Children with Duchenne Muscular Dystrophy: The Additive Value of Cardiovascular Magnetic Resonance.

In this case series, we describe the diagnosis of post-COVID-19 myocarditis in asymptomatic patients with Duchenne Muscular Dystrophy (DMD) and a mild COVID-19 disease course. These patients were referred for CMR due to electrocardiographic and echocardiographic alterations, which did not exist before COVID-19 infection. CMR identified the presence of severe myocardial inflammation in all patients based on abnormally elevated myocardial T2 ratio, late gadolinium enhancement, native T1 mapping, T2 mapping, and extracellular volume fraction. This was paired with concurrent impairment of left ventricular function. Appropriate treatment was initiated in all cases. Two of the four patients developed episodes of ventricular tachycardia during the following 6 months, and a defibrillator was implanted. Despite the mild clinical presentation, this case series demonstrates the diagnostic strength of CMR in the diagnosis and evaluation of post-COVID-19 myocarditis and serves to increase awareness of this potential complication amongst treating physicians.

Duct stenting in an ELBW infant with aortic arch interruption.

Aortic arch interruption is a rare cardiac malformation. In some cases, it is part of a more complex congenital heart disease. Survival of extremely low birth weight infants with this cardiac disease is very rare. Surgical correction is the only treatment. Ductal stenting as a bridge procedure in small affected infants is a good alternative.

The prevalence of underweight and obesity in CHD. Response to body mass index and age are associated with ventricular end-diastolic pressure in adults with a Fontan circulation.

INTRODUCTION: The aim of the study is to determine the impact of obesity in children with CHD which is severe enough to require invasive catheterisation. METHODOLOGY: This is a retrospective study in a cardiac catheterisation laboratory of a large paediatric hospital. The material consisted of 378 children from 2 to 19 years old who underwent heart catheterisation in the years 2011-2019. Their anthropometric data were collected, and the body mass index was calculated. The BMI centile was then calculated according to the Centers for Diseases Control (CDC). They were classified as overweight (85-95th centile), obese (> 95th centile), normal weight (5th-85 centile), and underweight (< 5th centile). RESULTS: 18.7% of children were underweight, 54.76% were normal weight, 11.64% were overweight, and 14.81% were obese. Overall, 26.45% of the children were overweight. Boys were more likely to have increased body weight than girls, but the difference was not statistically significant. The percentage of increased weight was similar in children with acyanotic CHD as well as heart disease from other causes. On the contrary, children with cyanotic heart disease were more likely to be younger and have a higher percentage of underweight children. CONCLUSIONS: The rates of obesity and overweight in children with CHD depend on the sex, age, and cardiopathy type. In addition, they agree with the findings that have been published in international series studies for children with CHD.

Markedly elevated troponin and NT-proBNP and myocardial dysfunction in an adolescent with severe diabetic ketoacidosis: A case report.

Severe diabetic ketoacidosis (DKA), rarely, may be associated with elevated troponin and proBNP levels in adults with a history of diabetes. However, few cases have reported this association in children with severe and complicated DKA. We describe a case of severe DKA (pH: 6.89, HCO3: 6.5) in a 14-yr-old female adolescent in which the symptoms of DKA were presented days before the diagnosis. The patient was under the effect of acidosis (Kussmaul respiration) for 12 h before admission to our hospital, where she was admitted in a critical clinical condition. After successful treatment with DKA with intensive intravenous fluid and regular insulin, the patient presented with abnormal cardiac rhythm, disturbance of interventricular septum motility, a mild decrease in left ventricular systolic function, negative T waves in leads III and aVF, and a marked increase in troponin and brain natriuretic peptide (NT-proBNP) levels. All abnormal findings completely resolved within 8 days after the initiation of DKA treatment. The phenomenon in our case was transient, and the patient had a good long-term outcome. However, it represents a challenge for clinicians; therefore, emphasis should be given to cardiac monitoring during the course of severe and prolonged DKA in children and adolescents.

Targeted Multiresidue Method for the Analysis of Different Classes of Pesticides in Agro-Food Industrial Sludge by Liquid Chromatography Tandem Mass Spectrometry.

Sludge generated after washing of fruits and vegetables during agro-food processes is a complex matrix and selective methods for the identification and quantification of pesticides' residues are necessary in order to achieve a sustainable and effective management of the total sewage. The present work describes the development and validation of a reliable, simple and fast analytical method based on liquid chromatography-tandem mass spectrometry (LC-MS/MS) for the determination of 47 pesticides of different chemical classes, including organosphosphates, pyrethroids, neonicotinoids, triazoles and others, in sludge samples after QuEChERS sample preparation. The necessity of the individual steps of QuEChERS was investigated and the LC-ESI-MS/MS conditions were optimized to achieve maximum sensitivity of the target analytes. The method limits of detection (LODs) ranged between 0.0005 mg/kg (imidacloprid) and 0.05 mg/kg (beta cyfluthrin). The recoveries ranged between 71-120% and the repeatability of the method was ≤25% expressed as relative standard deviation. The method was applied to sludge samples generated after washing of fruits in an agro-fruit-packaging unit in Greece. The results showed the presence of 37 pesticides' active substances with concentrations ranging from low ppbs, such as fludioxinil (5 µg/kg) up to low ppms such as beta cyfluthrin (3.5 mg/kg) and with their sum concentration reaching up to 19 mg/kg.

Friedreich's Ataxia: Case series and the Additive Value of Cardiovascular Magnetic Resonance.

BackgroundFriedreich's ataxia (FA) is an autosomal-recessive neurodegenerative disease characterised by neurologic, cardiac and endocrine abnormalities. Currently, Friedreich cardiomyopathy (FA-CM) staging is based on early ECG findings, high sensitivity troponin (hsTNT) ≥14 ng/ml and echocardiographic left ventricular (LV) morphologic and functional evaluation. However, further parameters, accessible only by cardiovascular magnetic resonance (CMR), such as myocardial oedema, perfusion defects, replacement and/or diffuse myocardial fibrosis, may have a place in the staging of FA-CA. Our aim was to elucidate the additive value of CMR in FA-CM.MethodsThree FA cases were assessed using ECG, 24 h Holter recording, hsTNT, routine ECHO including wall dimension, valvular and ventricular function evaluation and CMR using 1.5T Ingenia system. Ventricular volumes-function, wall dimensions and fibrosis imaging using late gadolinium enhancement (LGE) was performed.ResultsAll FA patients had non-specific ECG changes, almost normal 24 h Holter recording, mild hypertrophy with normal function assessed by echocardiography and increased hsTNT. However, the CMR evaluation revealed the presence of LGE >5% of LV mass, indicative of severe fibrosis. Therefore, the FA patients were re-categorized as having severe FA-CA, although their LVEF remained normal.ConclusionThe combination of classical diagnostic indices and CMR may reveal early asymptomatic FA-CM and motivate the early initiation of cardiac treatment. Furthermore, these indices can be also used to validate specific treatment targets in FA, potentially useful in the prevention of FA-CM.

Recurrent arterial ischemic strokes in a patient with patent foramen ovale and ductus arteriosus: Presentation of our management and review of the literature.

Ischemic stroke in children is a relatively rare entity, relative to the adult population. The most common potential risk factors include cardiac embolism, prothrombotic states and vasculopathies. The diagnosis is concerning for the need to identify the underlying cause. Treatment of the proximate source of ischemia can often protect against future events. We present the case of a 7-year-old patient who initially presented with an ischemic brain insult which was repeated, despite the initiation of anticoagulation therapy. The investigation revealed patent foramen ovale and patent ductus arteriosus and because of the recurrent ischemic ictuses, transcatheter closure of both defects was decided. A brief description of the literature is also presented.

Monitoring of glyphosate and AMPA in soil samples from two olive cultivation areas in Greece: aspects related to spray operators activities.

The persistence of glyphosate and its primary metabolite AMPA (aminomethylphosphonic acid) was monitored in two areas in Southern Greece (Peza, Crete and Chora Trifilias, Peloponnese) with a known history of glyphosate use, and the levels of residues were linked to spray operators' activities in the respective areas. A total of 170 samples were collected and analysed from both areas during a 3-year monitoring study. A new method (Impact Assessment Procedure - IAP) designed to assess potential impacts to the environment caused by growers' activities, was utilised in the explanation of the results. The level of residues was compared to the predicted environmental concentrations in soil. The ratio of the measured concentrations to the predicted environmental concentrations (MCs/PECs) was > 1 in Chora the first 2 years of sampling and < 1 in the third year, whilst the MCs/PECs ratio was < 1 in Peza, throughout the whole monitoring period. The compliance to the instructions for best handling practices, which operators received during the monitoring period, was reflected in the amount of residues and the MCs/PECs ratio in the second and especially the third sampling year. Differences in the level of residues between areas as well as sampling sites of the same area were identified. AMPA persisted longer than the parent compound glyphosate in both areas.

Oedema-fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging.

Duchenne muscular dystrophy (DMD) is an X-linked muscle disorder characterized by progressive, irreversible loss of cardiac and skeletal muscular function. Muscular enlargement in DMD is attributed to oedema, due to the increased cytoplasmic Na+ concentration. The aim of this review was to present the current experience and emphasize the role of cardiovascular magnetic resonance (CMR) in the diagnosis of this condition. DMD patients' survival depends on ventilatory assistance, as respiratory muscle dysfunction was the most common cause of death in the past. Currently, due to improved ventilatory assistance, cardiomyopathy has become the main cause of death, even though clinically overt heart failure may be absent. CMR is the technique of choice to assess the pathophysiologic phenomena taking place in DMD, such as myocardial oedema and subepicardial fibrosis. The classic index to assess oedema is the T2-weighted short-tau inversion recovery (T2w-STIR), as it suppresses the signal from flowing blood and resident fat and enhances sensitivity to tissue fluid. Furthermore, CMR is the most reliable technique to detect and quantify fibrosis in DMD. Recently, the new indices T2, T1 mapping (native and postcontrast) and the extracellular volume (ECV) allow a more accurate approach of myocardial oedema and fibrosis. To conclude, the assessment of cardiac oedema and subepicardial fibrosis in the inferolateral wall of the left heart ventricle are the most important early finding in DMD with preserved ventricular function, and CMR, using both the classic and the new indices, is the best technique to detect and monitor these lesions.

Cardiac profile of asymptomatic children with Becker and Duchenne muscular dystrophy under treatment with steroids and with/without perindopril.

BACKGROUND: To evaluate cardiovascular function in boys with Duchenne (DMD) and Becker (BMD) muscular dystrophy, using cardiac magnetic resonance (CMR). METHODS: This is a single point cross sectional study of twenty-four boys with genetically ascertained DMD, and 10 with BMD, aged 10.5 ± 1.5 years (range 9-13), were prospectively evaluated by a 1.5 T system and compared with those of age-sex matched controls. The DMD patients were divided in 2 groups. Group A (N = 12) were under treatment with both deflazacort and perindopril, while Group B (n = 12) were under treatment with deflazacort, only. BMD patients did not take any medication. Biventricular function was assessed using a standard SSFP sequence. Late gadolinium enhancement (LGE) was assessed from T1 images taken 15 min after injection of 0.2 mg/Kg gadolinium DTPA using a 3D-T1-TFE sequence. RESULTS: Group A and BMDs were asymptomatic with normal ECG, 24 h ECG recording and echocardiogram. Group B were asymptomatic but 6/12 had abnormal ECG and mildly impaired LVEF. Their 24 h ECG recording revealed supraventricular and ventricular extrasystoles (all at 12-13 yrs). LV indices in Group A and BMD did not differ from those of controls. However, LV indices in Group B were significantly impaired compared with controls, Group A and BMDs (p < 0.001). An epicardial LGE area = 3 ± 0.5% of LV mass was identified in the posterolateral wall of LV only in 6/12 patients of Group B, but in not in any BMD or Group A. CONCLUSION: Children with either BMD or DMD under treatment with both deflazacort and perindopril present preserved LV function and lack of LGE. However, further large scale multicenter studies are warranted to confirm these data, including further CMR mapping approaches.

Distribution and function of carbamate hydrolase genes cehA and mcd in soils: the distinct role of soil pH.

Synthetic carbamates constitute a significant pesticide group with oxamyl being a leading compound in the nematicide market. Oxamyl degradation in soil is mainly microbially mediated. However, the distribution and function of carbamate hydrolase genes (cehA, mcd, cahA) associated with the soil biodegradation of carbamates is not yet clear. We studied oxamyl degradation in 16 soils from a potato monoculture area in Greece where oxamyl is regularly used. Oxamyl showed low persistence (DT50 2.4-26.7 days). q-PCR detected the cehA and mcd genes in 10 and three soils, respectively. The abundance of the cehA gene was positively correlated with pH, while both cehA abundance and pH were negatively correlated with oxamyl DT50. Amongst the carbamates used in the study region, oxamyl stimulated the abundance and expression only of the cehA gene, while carbofuran stimulated the abundance and expression of both genes. The cehA gene was also detected in pristine soils upon repeated treatments with oxamyl and carbofuran and only in soils with pH ≥7.2, where the most rapid degradation of oxamyl was observed. These results have major implications regarding the maintenance of carbamate hydrolase genes in soils, have practical implications regarding the agricultural use of carbamates, and provide insights into the evolution of cehA.

Integrated biodepuration of pesticide-contaminated wastewaters from the fruit-packaging industry using biobeds: Bioaugmentation, risk assessment and optimized management.

Wastewaters from fruit-packaging plants contain high loads of toxic and persistent pesticides and should be treated on site. We evaluated the depuration performance of five pilot biobeds against those effluents. In addition we tested bioaugmentation with bacterial inocula as a strategy for optimization of their depuration capacity. Finally we determined the composition and functional dynamics of the microbial community via q-PCR. Practical issues were also addressed including the risk associated with the direct environmental disposal of biobed-treated effluents and decontamination methods for the spent packing material. Biobeds showed high depuration capacity (>99.5%) against all pesticides with bioaugmentation maximizing their depuration performance against the persistent fungicide thiabendazole (TBZ). This was followed by a significant increase in the abundance of bacteria, fungi and of catabolic genes of aromatic compounds catA and pcaH. Bioaugmentation was the most potent decontamination method for spent packing material with composting being an effective alternative. Risk assessment based on practical scenarios (pome and citrus fruit-packaging plants) and the depuration performance of the pilot biobeds showed that discharge of the treated effluents into an 0.1-ha disposal site did not entail an environmental risk, except for TBZ-containing effluents where a larger disposal area (0.2ha) or bioaugmentation alleviated the risk.

Management of coronary artery aneurysms using abciximab in children with Kawasaki disease.

INTRODUCTION: There are limited data regarding the possible benefits of abciximab in children with Kawasaki disease (KD), who developed serious cardiac abnormalities non-responsive to standard treatment. MATERIALS AND METHODS: We retrospectively identified children with KD who were treated with abciximab from 2007 to 2015. Data regarding clinical course, treatment, echocardiographic data and follow-up at 1 and 6months were retrieved. RESULTS: During the study period, fifteen children were identified who were diagnosed with KD and were given abciximab. The median age at onset of symptoms was 11months (range: 2months-6years). The median day of disease at admission was 10days (range: 4-26days) and the median day of administration of abciximab was 17days (range: 9-40). Twelve children were diagnosed with complete and three with incomplete KD. Aneurysms were found in 8 children: 2 had ectatic coronary arteries and 5 presented with both ectasia and aneurysms. At 1month follow-up, echocardiographic findings showed regression in the size of aneurysms in 11 children, resolution of the aneurysms or ectasia of coronary arteries in 3 children, while one child who could not take aspirin because of G6PD deficiency died. After 6months of follow-up, echocardiographic findings showed resolution of coronary abnormalities in 12 (80%) children, whereas 2 children (13.3%) presented with significant regression of aneurysms. CONCLUSIONS: Abciximab may have an important role in the management of severe cardiac complications of KD, although prospective randomized controlled studies are needed to fully evaluate its role.

Haddad Syndrome.

Congenital central hypoventilation syndrome (CCHS) causes predominantly sleep apnoea and is one of a growing number of inherited disorders characterised by autonomic nervous system dysfunction/dysregulation (ANSD). In association with Hirschsprung's disease (HSCR), it presents as Haddad's syndrome. We report a case of Haddad's syndrome complicated by sinus node dysfunction.

Transcatheter closure of large atrial septal defects with deficient aortic or posterior rims using the "Greek maneuver". A multicenter study.

OBJECTIVES: We report a modification ("Greek maneuver") of the standard atrial septal defect (ASD) closure technique using the Amplatzer septal occluder (ASO) to facilitate closure of large ASDs with deficient aortic or posterior rims. METHODS: 185 patients (median 10.8, range 3 to 52 years) with large ASDs (mean diameter 26±7 mm, range 20-40 mm) with a deficient aortic (134 patients) or posterior (51 patients) rim underwent catheter closure with the ASO using the "Greek maneuver" under transesophageal guidance. The Greek maneuver is applied when protrusion of the aortic edge of the deployed left disk of the device in to the right atrium is detected by echo. To circumvent this left disk is recaptured and the whole delivery system is pushed inward and leftward into the left atrium where the left disk and the 2/3 of right disk are simultaneously released. This maneuver forces the left disk to become parallel to the septum preventing the protrusion of the device into the right atrium. RESULTS: The ASO was successfully implanted and was associated with complete closure in 175/185 (95%) of the patients. There were no early or late complications related to the procedure during a follow-up period ranging from 6 months to 7 years. CONCLUSIONS: The "Greek maneuver" is a simple quite useful trick that facilitates closure of large ASDs associated with or without deficient aortic or posterior rims.

Quantitative and qualitative differences in the metabolism of pesticides in biobed substrates and soil.

Biobed substrates commonly exhibit high degradation capacity. However, degradation does not always lead to detoxification and information on the metabolic pathways of pesticides in biobeds is scarce. We studied the degradation and metabolism of three pesticides in selected biomixtures and soil. Biomixtures stimulated degradation of terbuthylazine and metribuzin, whereas chlorpyrifos degraded faster in soil. The latter was attributed to the lipophilicity of chlorpyrifos which increased adsorption and limited biodegradation in organic-rich biomixtures. Although the same metabolites were detected in all substrates, qualitative and quantitative differences in the metabolic routes of pesticides in the various substrates were observed. Chlorpyrifos was hydrolyzed to 3,5,6-tricholorpyridinol (TCP) which was further degraded only in compost-biomixture CBX1. Metabolism of terbuthylazine in compost biomixtures (BX) and soil resulted in the formation of desethyl-terbuthylazine (DES) which was fully degraded only in the compost-biomixture CBX2, whereas peat-based biomixture (OBX) promoted the hydroxylation of terbuthylazine. Desamino- (DA) (dominant) and diketo- (DK) metribuzin appear as intermediate metabolites in all substrates and were further transformed to desamino-diketo-metribuzin (DADK) which was fully degraded only in compost-biomixture GSBX. Overall, lower amounts of metabolites were accumulated in biomixtures compared to soil stressing the higher depuration efficiency of biobeds.