Is it safe to close ASD with the guidance of transthoraxic echocardiography in pediatric population "ten years' experience of a single center".

OBJECTIVE: Main purpose of this study is to emphasize the usage and safety of transthoracic echocardiography (TTE) in percutaneous atrial septal defect (ASD) closure in appropriate pediatric cases. BACKGROUND: Nowadays, percutaneous closure is preferred as treatment modality for ASD in pediatric age group. METHODS: Between the dates December 2003-August 2013; 340 patients whose ASD were closed included in this study. Physical examination, electrocardiogram, TTE were done before the procedure, at the 24th hour, 1st and 6th month after the procedure. After the 6th month, routine control was done annually. Transesophageal echocardiography (TEE) was performed in 184 cases with large, multifenestrated ASD and when TTE views were poor in quality. We selected the 201 patients whose ASD diameter between 10-20 mm and formed 2 homogeneous groups according to the type of echocardiography used (TEE or TTE) in order to compare the role of echocardiography. RESULTS: The demographic features of patients of 2 groups were similar. There was not any statistically difference between ASD, balloon sizing diameters between the groups. No statistically significant difference in the success, complication, and residual shunt rates was found between the groups. Procedure, fluoroscopy time, and amount were significantly higher in TEE group. When hemodynamic variables except pulmonary blood flow to systemic blood flow (Qp/Qs; right-left atrium mean pressure, pulmonary vascular resistance, and peak-mean pulmonary arterial pressure) were compared, there was not statistically difference. CONCLUSION: TEE is an invasive procedure and requires general anesthesia, therefore, it should not be done routinely in ASD but only in selected cases. If the size and the anatomy of ASD is appropriate, TTE should be preferred primarily in percutaneous ASD closure.

Simultaneous transcatheter closure of intralobar pulmonary sequestration and patent ductus arteriosus in a patient with infantile Scimitar syndrome.

Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.

Are endothelial microparticles early markers of pulmonary hypertension?

OBJECTIVE: To find out the relation between endothelial microparticles (EMPs), pulmonary arterial stiffness and thickness of pulmonary artery intima media to determine the prognosis of Eisenmenger syndrome and their correlation with echocardiographic and hemodynamic parameters. METHODS: Sixteen patients with Eisenmenger syndrome and 37 control patients were included. Electron microparticles levels, angiographic and echocardiographic findings were compared. RESULTS: Thickness of pulmonary arterial intima media and systolic and diastolic diameters of pulmonary artery were found significant in the patient group. CD144 and CD146 EMP values of patient group were statistically high. However, there was not any significant difference in pulmonary arterial strain, elasticity and stiffness. Positive significant relationship was found between pulmonary artery intima media thickness and CD144 in patient group. But there was not any significance between CD 146 and pulmonary artery intimamedia thickness. CONCLUSIONS: Invasive methods remain as the gold standard for pulmonary hypertension diagnosis, follow-up and treatment, but it is risky and can even be fatal. Our study showed that EMPs, thickness of pulmonary artery intima media and pulmonary stiffness could be novel noninvasive modalities for the follow-up pulmonary hypertensive patients.

The role of N-terminal proBNP in the clinic scoring of heart failure due to dilated cardiomyopathy in children.

BACKGROUND: We aimed to show sensitivity and specificity of NT-ProBNP in demonstrating the degree of cardiac failure caused by dilated cardiomyopathy (DCMP). METHODS: From January 2006 to July 2012, thirty patients (2 - 168 months) who had DCMP with acute heart failure and 37 healthy children aged (1 - 168 months) were enrolled in this study. Clinical evaluation was done using a modified Ross scoring system. Ross scoring was done before and after treatment. Patients with a score of > 2 points were included in the study. Ross scoring, echocardiographic parameters, serum NT-ProBNP levels and cardiothoracic index (CTI) were measured before and on the 7th day of treatment. Patients were divided into 3 groups according to degree of heart failure. RESULTS: While the change in logNT-ProBNP in the patient group following one-week of treatment was significant (p < 0.05), there was no marked significance in the changes in EF, FS, LVEDD, LVMIz, and CTI. There was a statistically significant difference between logNT-ProBNP levels each Ross clinical group not only before treatment but also on assessment on the 7th day of treatment in the patient group (p < 0.001, Tukey's and Tamhane's T2 post-hoc tests). No significant difference was detected between EF, FS, LVEDDs, LVMIz, and CTI and the stages of acute cardiac failure. The NT-ProBNP levels of patients who became clinically asymptomatic after treatment but still had left ventricular systolic dysfunction were statistically significant when compared to the control group. The cut off value to distinguish healthy children from the patients with left ventricular systolic dysfunction caused by cardiomyopathy was found as 174.3 pg/mL. CONCLUSIONS: NT-ProBNP levels are more effective than conventional echocardiographic parameters for clinical determination of the stage of cardiac failures in children with left ventricular systolic dysfunction due to DCMP. Therefore, it can be used for determining the treatment and management of such patients. Furthermore, the test is simple and beneficial, because of its availability in most clinical chemistry laboratories and its advantage of allowing frequent measurements and assessments.

Cheatham platinum stent implantation in children with coarctation of the aorta: single-centre short-term, intermediate-term, and long-term results from Turkey.

OBJECTIVES: Our aim was to evaluate patients who were treated by percutaneous stent implantation. METHODS: Patients with aortic coarctation (n = 35) who had been treated with 38 stents - 12 bare and 26 covered - were evaluated. The demographics and procedural and follow-up data were recorded from hospital registers and compared according to patient specifications, for example, weight and coarctation nature. RESULTS: The procedure was successful in all patients. There was a statistically significant difference between the patients with native coarctation (n = 17) and those with recurrent coarctation (n = 18) in terms of pre-procedural blood pressures, systolic gradients, coarctation diameters, and the ratio of the coarctation site diameter to the descending aorta diameter. Although all patients received antihypertensive drugs before the procedure, the drug was discontinued in 26 patients during follow-up (p < 0.001). Stent migration was observed in four patients with recurrent coarctation (11.4%), and peripheral arterial injury was seen in three patients (8.5%). The mean follow-up time was 34 ± 16 months. On average, 21 (6-42) months after the procedure, six patients underwent cardiac catheterisation. At least 2 years after the procedure, tomography was performed in 20 patients (57.2%). Patients who were evaluated by multi-slice computerised tomography revealed no pathologies. There was no statistically significant difference between the five patients weighing less than 20 kg and the other 30 patients in terms of demographic and procedural characteristics, procedure success and complication rates, and follow-up data. CONCLUSION: Stent implantation for aortic coarctation is a method yielding satisfactory results in reducing coarctation gradients, efficient enlargement of the lesion area, and resolution of hypertension for children, including those weighing less than 20 kg.

Clinical features and outcomes of 76 patients with COVID-19-related multi-system inflammatory syndrome in children.

OBJECTIVES: Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission. METHODS: The MIS-C patients under 18 years old diagnosed and treated in three referral centers between July 2020 and March 2021 were included. Data of the patients were retrospectively obtained from their medical records. RESULTS: Overall, 76 subjects (24 females) with a mean age of 8.17 ± 4.42 years were enrolled. Twenty-seven (35.5%) patients were admitted to the PICUs. The two most common systemic involvement patterns were cardiac and gastrointestinal. There was only one lethal outcome in a patient with underlying acute lymphoblastic leukemia. Those with higher procalcitonin levels at admission were found to stay longer in the hospital (r = 0.254, p = 0.027). The risk of PICU admission increased with age (aOR: 1.277; 95% CI: 1.089-1.498; p = 0.003) and with decreased initial serum albumin levels (aOR: 0.105; 95% CI: 0.029-0.378; p = 0.001). CONCLUSION: Although there is a wide clinical variability among the patients with MIS-C, we suggest that those with older age and lower initial serum albumin levels merit close monitoring due to their higher risk for PICU admission. Key Points • Although there is a wide variability regarding the management process among clinicians, MIS-C is a rare, severe, less understood complication of COVID-19 that may cause rapid clinical deterioration in the patients. • Clinicians should be aware of this condition in children with persistent fever and a family history of COVID-19. • Older age and low serum albumin levels are the independent predictors for the pediatric intensive care unit admission among MIS-C patients.

Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome?

Andersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 60% of all cases of the disorder arecaused by mutations in the KCNJ2 gene. A 13-year-old female patient was referred for frequent premature ventricular contractions. Suspicion of ATS due to dysmorphic findings, electrocardiogram changes, and periodic muscle weakness was genetically confirmed. Beta-blocker therapy was initiated as a first-line treatment for bidirectional VT and frequent polymorphic premature ventricular contractions. Despite proper treatment, the VT attacks were not brought under control. Flecainide was added to the treatment regime. The number of premature ventricular contractions was dramatically reduced with flecainide and the VT attacks completely disappeared. This patient is a rare example of ATS in our country. This article provides a description of successful management of rhythm disturbance in a patient with ATS.

Do we need a femoral artery route for transvenous PDA closure in children with ADO-I?

OBJECTIVE: The standard procedure in percutaneous closure of patent ductus arteriosus (PDA) with Amplatzer duct occluder-I (ADO-I) is transvenous closure guided by aortic access through femoral artery. The current study aims to compare the procedures for PDA closure with ADO-I: only transvenous access with the standard procedure. METHODS: This study was designed retrospectively and 101 pediatric patients were included. PDA closure was done by only femoral venous access in 19 of them (group 1), arterial and venous access used in 92 patients (group 2) between 2004 to 2012 years. The position of the device and residual shunt in group1 was evaluated by the guidance of the aortogram obtained during the return phase of the pulmonary artery injection and guidance of transthoracic echocardiography. Shapiro-Wilk's test, Mann-Whitney U, chi-squared tests were used for statistical comparison. RESULTS: The procedure was successful in 18 (95%) patients in group 1 and 90 (98%) patients in group 2. Complications including the pulmonary artery embolization (n=1), protrusion to pulmonary artery (n=1), inguinal hematoma (n=3), bleeding (n=2) were only detected in group 2. In other words, while complications were observed in 7 (7.2%) patients in group 2, no minor/major complication was observed in group 1. Complete closure in group 1 was: in catheterization room 14 (77.8%), at 24th hour in 2 (11.1%), at first month in 2 (11.1%). Complete closure in group 2 was: 66 (73.4%) patients in the catheterization room, 21 (23.3%) at 24th hour, 3 (3.3%) at first month, complete closure occurred at the end of first month. CONCLUSION: In percutaneouse PDA closure via ADO-I, this technique can be a choice for patients whose femoral artery could not be accessed, or access is impossible/contraindicated. But for the reliability and validity of this method, randomized multicenter clinical studies are necessary.

Circulating insulin-like growth factor binding protein-4 (IGFBP-4) is not regulated by parathyroid hormone and vitamin D in vivo: evidence from children with rickets.

OBJECTIVE: Insulin-like growth factor binding protein-4 (IGFBP-4), inhibits IGF actions under a variety of experimental conditions. Parathyroid hormone (PTH), 1.25-hydroxy(OH)vitamin D, IGF-I, IGF-II and transforming growth factor (TGF)-b are the major regulators of IGFBP-4 production in vitro. However, little is known about the in vivo regulation of circulating IGFBP-4 in humans. METHODS: We measured serum concentrations of calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), PTH, vitamin D, IGF-I, IGFBP-3, and IGFBP-4 in infants (n=22) with nutritional rickets before and after treatment of rickets with vitamin D (300 000 U single dose po). RESULTS: The mean±SD age of the patients was 1.3±1.6 years (range 0.2-3). Serum Ca and P increased, whereas ALP and PTH decreased after treatment (Ca from 6.6±1.4 to 9.5±1.6 mg/dL, P from 3.9±1.4 to 5.4±0.8 mg/dL, ALP from 2590±2630 to 1072±776 IU/mL and PTH from 407±248 to 27.4±20.8 ng/dL, respectively). Vitamin D levels were low (7.8±2.5 ng/mL) and increased after treatment (18.1±4.0 ng/mL, p<0.001). Serum IGF-I and IGFBP-3 levels both increased after treatment (IGF-I: 13.5±12.2 vs. 23.7±14.2 ng/mL, p<0.001 and IGFBP-3: 1108±544 vs. 1652±424 ng/mL, p<0.001). However, serum IGFBP-4 levels did not change significantly after treatment (18.8±8.0 vs. 21.5±4.8 ng/mL). No correlation between PTH and IGF-I, IGFBP-3 or IGFBP-4 was detected. Significant correlations were observed between PTH and ALP (r=0.53, p<0.05), and between IGF-I and IGFBP-3 (r=0.46, p<0.05). CONCLUSION: The results demonstrate that contrary to in vivo studies, circulating IGFBP-4 levels are not influenced by secondary hyperparathyroidism in vitamin D deficiency rickets since IGFBP-4 levels did not change after normalization of PTH with vitamin D treatment.