Leukotriene B4 levels in CHD-associated paediatric pulmonary hypertension.

BACKGROUND: The aim of this study is to evaluate the role of leukotriene B4, an inflammatory mediator, in the development of pulmonary hypertension in paediatric patients with CHD with left-right shunt. METHODS: The study included forty patients with CHD with left-right shunts. Based on haemodynamic data obtained from cardiac diagnostic catheterisation, 25 patients who met the criteria for pulmonary arterial hypertension were included in the patient group. The control group comprised 15 patients who did not meet the criteria. The standard cardiac haemodynamic study was conducted. Leukotriene B4 levels were assessed in blood samples taken from both pulmonary arteries and peripheral veins. RESULTS: The median age of patients with pulmonary arterial hypertension was 10 months (range: 3-168), while the median age of the control group was 50 months (range: 3-194). In the pulmonary hypertension group, the median pulmonary artery systolic/diastolic/mean pressures were 38/18/24 mmHg, compared to 26/10/18 mmHg in the control group. Leukotriene B4 levels in pulmonary artery blood samples were significantly higher in the pulmonary arterial hypertension group compared to the controls (p < 0.05). Peripheral leukotriene B4 levels were also elevated in the pulmonary arterial hypertension group in comparison to the control group, though the difference was not statistically significant. CONCLUSION: The discovery of elevated leukotriene B4 levels in pulmonary artery samples from paediatric patients with pulmonary arterial hypertension secondary to CHD with left-to-right shunt suggests that local inflammation may have a pathological role in the development of pulmonary arterial hypertension.

Evaluation of Arrhythmia Prevalence, Management, and Risk Factors in Patients with Transcatheter and Surgically Closed Secundum Atrial Septal Defects.

OBJECTIVE: Atrial septal defect (ASD) accounts for 6-10% of all congenital heart disorders. Secundum ASD closure can be performed surgically or percutaneously. We aimed to identify the various arrhythmias that occur before, during, and after the procedure and evaluate their management. METHODS: The study included a total of 427 patients aged 0-18 years who underwent transcatheter or surgical closure of isolated secundum ASD between January 2008 and January 2020. Postoperative electrocardiogram (ECG) traces, intraoperative arrhythmias, and treatments were recorded for both groups. Echocardiography and ECG were evaluated at postoperative 1 week, 1, 3, and 6 months, and annually thereafter. RESULTS: After transcatheter closure, follow-up basal ECG showed incomplete right bundle branch block pattern in 21 patients and sinus rhythm in 229 patients. After surgical closure, incomplete right bundle branch block pattern was detected in 23 patients, complete right bundle branch block in 3 patients, and complete AV-block (Atrioventricular Block) pattern in 1 patient. The other 150 patients showed sinus rhythm. At least one postoperative follow-up Holter ECG record could be obtained for 104 patients in the transcatheter group and 96 patients in the surgical group. Of 104 patients who underwent transcatheter closure, 97 (93.3%) had normal Holter ECG findings and 7 (6.7%) had arrhythmia. Of the 96 patients who underwent surgical closure, 85 (88.5%) had normal Holter ECG traces and 11 (11.5%) had arrhythmia. There was no statistically significant difference in the frequency of arrhythmia (P = 0.164). CONCLUSION: The higher frequency of arrhythmia in adult studies compared to the pediatric age group once again demonstrates the importance of early diagnosis and treatment of ASD in childhood. The similar incidence of arrhythmia in both groups supports the safety and effectiveness of both closure methods in eligible patients.

A retrospective comparative analysis of factors affecting the decision and outcome of initial intravenous immunoglobulin alone or intravenous immunoglobulin plus methylprednisolone use in children with the multisystem inflammatory syndrome.

BACKGROUND: For children with the multisystem inflammatory syndrome(MIS-C), intravenous immunoglobulins (IVIG) with or without methylprednisolone are the most effective treatment. In this study, IVIG combined with methylprednisolone was compared to IVIG used alone in children with MIS-C. METHODS: This retrospective cohort study was carried out between April 1, 2020, and November 1, 2021. This study covered all children with MIS-C. According to whether they received IVIG alone or IVIG with methylprednisolone as an initial treatment for MIS-C, the patients were split into two groups. The IVIG dosage for the patients in group I was 2 gr/kg, whereas the IVIG dosage for the patients in group II was 2 gr/kg + 2 mg/kg/day of methylprednisolone. These two groups were contrasted in terms of the frequency of fever, length of hospital stay, and admission to the pediatric intensive care unit. RESULTS: The study comprised 91 patients who were diagnosed with MIS-C and were under the age of 18. 42 (46.2%) of these patients were in the IVIG alone group (group I), and 49 (53.8%) were in the IVIG + methylprednisolone group (group II). Patients in group II had a severe MIS-C ratio of 36.7%, which was substantially greater than the rate of severe MIS-C patients in group I (9.5%) (p 0.01). When compared to group I (9.5%), the rate of hypotension was considerably higher in group II (30.6%) (p = 0.014). Additionally, patients in group II had considerably higher mean serum levels of C-reactive protein. The incidence of fever recurrence was 26.5% in group II and 33.3% in group I, however the difference was not statistically significant (p > 0.05). CONCLUSIONS: The choice of treatment for patients with MIS-C should be based on an individual evaluation. In MIS-C children with hypotension and/or with an indication for a pediatric intensive care unit, a combination of IVIG and methylprednisolone may be administered. For the treatment modalities of children with MIS-C, however, randomized double-blind studies are necessary.

Validation of the Turkish Version of the Catheterization Risk Score for Pediatrics.

BACKGROUND: The aim of this study was to perform a validity analysis of the Turkish version of the Catheterization Risk Score for Pediatrics. METHODS: The study sample consisted of 419 pediatric patients who underwent cardiac catheterization. Patient risk factors and outcomes were collected using the revised (r) Catheterization Risk Score for Pediatric score (21 points) and Catheterization Risk Score for Pediatric score-20 point (Nykanen score). The serious adverse events and nonserious adverse event complications that occurred during and after the procedure were recorded. The revised Catheterization Risk Score for Pediatrics and Catheterization Risk Score for Pediatrics score-20 points were administered by pediatric cardiologists. The content validity index was calculated based on expert opinions. Chi-square, correlation, and regression analyses were used. RESULTS: The mean age of the pediatric patients was 4.5 ± 4.8 years. Of the patients, 50.1% were male (n=210) and 85% (n=356) had acyanotic heart disease. The patients' Catheterization Risk Score for Pediatrics score-20 point and revised Catheterization Risk Score for Pediatrics score were 5.9 ± 2.5 (range, 3-16) and 4.0 ± 2.5 (range, 0-16), respectively. Serious adverse events developed in 10.7% (n=45) of the patients and were found to be related with patient status/timing of catheterization, age, weight, respiratory status, and American Society of Anesthesiologist scores (P < .05). Significant positive correlations were found between the incidence of serious adverse events and total revised Catheterization Risk Score for Pediatrics score (21 points), total Catheterization Risk Score for Pediatrics score-20 point, and American Society of Anesthesiologist score (P < .01). CONCLUSION: The revised Catheterization Risk Score for Pediatrics score (21 points) and Catheterization Risk Score for Pediatrics score-20 point are valid tools for predicting preprocedural risk in the Turkish population.

Are clinical features and cardiac biomarkers at admission related to severity in pediatric acute myocarditis?: Clinical features and cardiac biomarkers in pediatric acute myocarditis.

OBJECTIVES: To evaluate the factors associated with intensive care requirement and mortality in pediatric myocarditis. METHODS: Children aged 28 days to 18 years who were diagnosed with acute myocarditis in a pediatric emergency department between January 2010 and September 2020 were enrolled in the study retrospectively. Demographic and clinical features, cardiac biomarkers, and imaging findings were evaluated. Length of hospital stay, need for hospitalization in the pediatric intensive care unit (PICU), treatments, and survival outcomes were recorded. To define the severity of disease, three groups were created and the data were compared in terms of clinical, laboratory, and imaging findings. The patients treated in the pediatric ward were compared with those hospitalized in the PICU. Ventricular dysfunction was defined in patients with a left ventricular ejection fraction (LVEF) of <50% and these patients were compared with those who had an LVEF of >50%. Also, survivor and non-survivor patients were compared. RESULTS: A total number of 62 patients with a median age of 8 years were included. Chest pain and tachycardia were the most common findings on physical examination. The mean LVEF was 59.3 ± 13.0% at admission. Of the patients, 17 were hospitalized in the PICU (27.4%). Chest pain was more common in patients hospitalized in the pediatric ward (p<0.001), and hypotension, vomiting, arrhythmia, were more common and LVEF was lower in patients in the PICU (p = 0.017, p = 0.008, p = 0.006, and p = 0.025, respectively). The children treated in the PICU were younger than those in the pediatric ward (p = 0.009). Troponin I levels were significantly higher in the pediatric ward (p = 0.035), and brain natriuretic peptide (BNP) levels were higher in patients in the PICU (p = 0.012). Death occurred in four patients. Hypotension and vomiting were significantly more common in non-survivors (p = 0.020 and 0.004, respectively). Inotropes and intravenous immunoglobulin (IVIG) were more commonly used in non-survivors (p = 0.001 and p = 0.015, respectively). BNP levels were higher in non-survivors (p = 0.008), and troponin I levels were not different between survivors and non-survivors (p = 0.260). CONCLUSION: In pediatric acute myocarditis, lower LVEF, increased BNP, as well as the presence of hypotension and arrhythmia were found to be related to intensive care requirement. Hypotension and vomiting were found to be more common in non-survivors. Due to the possibility of rapidly worsening disease, physicians should be alert to the presence of these findings.

Next-Generation Sequencing Analysis of MVK, NLRP3, TNFRSF1A, and PSTPIP1 Genes in Patients without MEFV Gene Variation and Genotype-Phenotype Correlation.

Objective: In this study, we aimed to evaluate other interleukin-1b-mediated monogenic autoinflam- matory diseases (AIDs) (tumor necrosis factor receptor-1-associated periodic syndrome, hyperimmuno- globulin D syndrome, cryopyrin-associated periodic syndrome (CAPS), pyogenic arthritis, pyoderma gangrenosum, and acne syndrome) by the next-generation sequencing method (NGS) in cases with clinical Familial Mediterranean Fever symptoms, and no variant detected in the MEFV gene. Methods: The cases included in this study and their parents were interviewed and filled in a survey form. The targeted genetic panel for interleukin-1b-mediated AIDs covering four genes (MVK, NLRP3,TNFRSF1A, and PSTPIP1) was studied for cases with a negative result from the MEFV gene analysis. The genetic analysis was conducted using the targeted NGS method. Results: Variants were found in 16 out of the 40 patients in the study sample. These variants were pri- orly reported in variant databases, and three of them were identified as definitely pathogenic (V377I of the MVK gene, C52Y of the TNFRSF1A gene, and I313V of the NLRP3 gene), four as a variant of uncer- tain significance (VUS) (R92Q of the TNFRSF1A, A372V of the PSTPIP1, and V198M and Q703K of the NLRP3), and one as benign polymorphism (S52N of the MVK gene). The median age of onset among variant-positive cases was 10.5 (3.5-18) years. The most common clinical findings in the variant-positive group were arthralgia, fever, and abdominal pain. While three out of 40 patients met the classification criteria before genetic analysis, only one patient was diagnosed with CAPS as a result of genetic analy- sis, and other patients were considered as nonspecific phenotype. Conclusion: The use of NGS gene panels seems beneficial in diseases with heterogeneous clinical manifestations such as systemic AIDs. Although the number of variants detected is high, clinical diag- nosis rates remain low. The genotype­phenotype relationship in these diseases is still unclear.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

A Comparative Study of Children with MIS-C between Admitted to the Pediatric Intensive Care Unit and Pediatric Ward: A One-Year Retrospective Study.

OBJECTIVES: This descriptive study aimed to compare the clinical and laboratory features of the children with the multisystem inflammatory syndrome in children (MIS-C), requiring pediatric intensive care unit (PICU), admission with the MIS-C patients who did not require PICU admission. PATIENTS AND METHODS: This study was conducted between March 2020 and February 2021 at the University of Health Sciences Dr. Behçet Uz Children's Hospital, a referral center for pediatric infectious diseases in the Aegean Region of Turkey. All hospitalized patients aged 18 years old or less with MIS-C according to the definition of the universal guidelines were included in the study. Data of the patients with the diagnosis of MIS-C were recorded and collected from the electronic medical records of the hospital. The data included demographic characteristics, presenting signs and symptoms, laboratory findings and clinical data. RESULTS: A total of 58 patients with MIS-C were included in this study. Thirty-eight (65.5%) patients were male. The median age was 6 years (2 months-16 years). The patients admitted to PICU were 15 (25.9%). The rate of pulmonary involvement was 81.3% (n = 13) in the PICU group. The median procalcitonin, C-reactive protein, erythrocyte sedimentation rate, D-Dimer and ferritin values were significantly higher in the PICU group compared to non-PICU group (p < 0.001, p = 0.02, p < 0.001, p = 0.006 and p = 0.031). CONCLUSIONS: Besides the depressing cardiac functions reported before, the pulmonary involvement and signs of shock are important factors for PICU admission in children with MIS-C.