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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.

Introduction: In this study, we aimed to detect the cytokine that is involved in the early stage of chronic kidney disease and associated with cardiovascular disease. Methods: We included 50 patients who were diagnosed with predialytic chronic kidney disease and 30 healthy pediatric patients in Ege University Medical Faculty Pediatric Clinic, İzmir/Turkey. Interleukin-8 (IL-8), interleukin-10 (IL-10), interleukin-13 (IL-13), and transforming grow factor-β1 (TGF-β1) levels (pg/mL) were measured by ELISA. Carotid-femoral pulse wave velocity (PWV), augmentation index (Aix), carotid intima media thickness (cIMT), and left ventricular mass index (LVMI) were evaluated as markers of cardiovascular disease. The presence of a cardiovascular disease marker was defined as an abnormality in any of the parameters (cIMT, PWV, Aix, and left ventricular mass index (SVKI)). The patient group was divided into two groups as with and without cardiovascular disease. Results: Mean Aix and PWV values were higher in CKD patients than controls (Aix: CKD 32.8±11.11%, healthy subjects: 6.74±6.58%, PWV CKD: 7.31±4.34m/s, healthy subjects: 3.42±3.01m/s, respectively; p=0.02, p=0.03). The serum IL-8 levels of CKD were significantly higher than of healthy subjects 568.48±487.35pg/mL, 33.67±47.47pg/mL, respectively (p<0.001). There was no statistically significant difference between IL-8, IL-10, IL-13, TGF-1, in CKD patients with and without cardiovascular disease (p> 0.05). Discussion: IL-8 is the sole cytokine that increases in pediatric patients with chronic kidney disease among other cytokines (IL-10, IL-13 and TGF-β1). However, we did not show that IL-8 is related to the presence of cardiovascular disease.

The study aimed to revisit the in-hospital predictors of shunt thrombosis (ST) in the foreground of the pulmonary artery size in patients who received modified Blalock-Taussig shunt (mBTS) as the first-stage palliation. Data from 80 patients who received mBTS as their initial palliative procedure between February 2012 and January 2017 was retrospectively collected. The median age and weight of the patients at the time of their mBTS procedure was 4 days (IQR 2-22 days) and 3.2 kg (IQR 2.8-3.7 kg), respectively. Of the 80 patients in the study, 11 (13.8%) developed ST. The diameter and corresponding z scores of the pulmonary arteries were significantly lower in patients with ST. The median shunt size/shunted pulmonary artery size (S/PA) ratio was considerably higher in patients with ST. In logistic regression analysis, pulmonary artery hypoplasia (PAH) [odds ratio (OR) = 13.7 (0.06-0.21), p < 0.001], S/PA ratio ≥ 0.9 [OR = 8.1 (0.03-0.53), p = 0.03], prematurity [OR = 9.5 (0.05-0.33), p = 0.003], and shunt size/weight (S/W) ratio ≥ 1.3 [OR = 6.4 (0.04-0.67), p = 0.012] were found to have a significant impact on ST. The best combination of sensitivity and specificity of the S/W (0.73 and 0.75) and the S/PA ratio (0.73 and 0.80) were achieved at the cut-off value of 1.3 and 0.9, respectively. The Youden index of S/PA was 0.52. While the area under the curve (AUC) of the S/W ratio was 0.686 ± 0.12 (p = 0.049), the AUC of the S/PA ratio was 0.791 ± 0.08 (p = 0.002). In conclusion, instead of weight, considering the size of the target pulmonary artery and thereby, the S/PA ratio would be more instructive in determining shunt size. There were a high number of patients in our study who showed PAH having received a shunt size based on their body weight. By contrast, our results showed that the S/PA ratio of ≥ 0.9 would be a good predictor of in-hospital ST.

Background Gaucher disease (GD) is a lysosomal storage disorder caused by autosomal recessive mutations in the glucocerebrosidase (GBA) gene, which encodes acid β-glucosidase. GD type 3c is a rare group characterised by cardiovascular involvement, and homozygous D448H is the most frequent mutation. Case presentation We describe two patients who had homozygous D448H mutations. The index patient had hepatosplenomegaly, liver insufficiency and cardiac involvement and her sister had severe cardiac involvement with cardiomyopathy and diffuse aortic calcification. The index case’s liver was transplanted at the age of 6 months from a related donor and her sister who had severe cardiovascular disease died at the age of 12 years. Conclusions Our patients had clinical variability. We need to discuss whether liver involvement could be the initial signs in patients with GD type 3c.

Objectives: To demonstrate the usefulness of N-Terminal Pro-B-Type natriuretic peptide (NT-proBNP) as an early biomarker of carbon monoxide-induced myocardial injury in children. It also aimed to identify the correlation between NT-proBNP and left ventricular systolic dysfunction findings shown by echocardiography. Methods: Prospective, observational study conducted at a paediatric emergency department between October 2017 and April 2019 which involved children aged 0–17 years. The patients were divided into three groups based on severity; mild, moderate and severe groups. The patient characteristics, carboxyhaemoglobin, CK-MB Mass (CKMB-M), troponin-T, and NT-proBNP levels were measured, and echocardiography was performed and left ventricular ejection fraction was measured. Results: Sixty-nine patients and 60 healthy controls were included. Male gender, younger age, higher carboxyhaemoglobin levels, and altered mental status were found as independent predictors of carbon monoxide-induced myocardial injury. If the cut-off value for NT-proBNP level is >480 pg/ml, the sensitivity–specificity for decreased left ventricular ejection fraction, which is the strongest carbon monoxide-induced myocardial injury sign, were 100–96%, respectively. A high negative correlation was found between NT-proBNP levels and left ventricular ejection fraction (r = −0.769, p < 0.01) in the carbon monoxide poisoning group, and there was a positive correlation between the carboxyhaemoglobin and NT-proBNP levels (r = 0.583, p < 0.01). Conclusion: Echocardiography is an ideal tool and very sensitive, but its routine use is limited due to its non-availability. An increased level of NT-proBNP (>480pg/ml) may be useful as an ideal biomarker for early detection of carbon monoxide-induced myocardial injury sign and reduced left ventricular ejection fraction which is the most crucial point in making a decision on hyperbaric oxygen therapy.

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AbstractKawasaki disease, known as mucocutaneous lymph node syndrome, is a multi-system disease of unknown aetiology that occurs in young children under 5 years of age. The recurrence rate of Kawasaki disease is as rare as 1–3%. Especially in cases with coronary artery involvement, recurrent Kawasaki disease should be investigated in terms of underlying rheumatologic diseases such as periodic fever syndromes, microscopic polyangiitis, polyarteritis nodosa, and systemic-onset juvenile arthritis. In this study, we report homozygote mutations in mevalonate kinase and familial Mediterranean fever genes in a recurrent Kawasaki disease with coronary dilatation.