Effect of Piracetam and Iron Treatment on Heart Rate Variability in Patients With Breath-Holding Spell.

BACKGROUND: Breath-holding spells are a benign condition primarily seen in 3% to 5% of healthy children aged between six months and five years. Although no specific treatment is recommended due to its benign nature, iron and piracetam are used in severe or recurrent cases. We planned to compare the heart rate variability (HRV) before and after treatment with 24-hour Holter monitoring in patients receiving iron and piracetam treatment and investigate the treatment's effectiveness. METHODS: Twenty-five patients who applied to the outpatient clinic between 2013 and 2015 due to breath-holding spells were included in the study. The patients who received piracetam and iron therapy and underwent 24-hour rhythm Holter monitoring were evaluated retrospectively. RESULTS: Fourteen (56%) of these patients were evaluated as having cyanotic-type and 11 (44%) patients were assessed as having pale-type breath-holding spells. A significant difference was found only between hourly peak heart rate and total power in the group receiving iron treatment. Significant differences were also found among the minimum heart rate, mean heart rate, the standard deviation of RR intervals, the mean square root of the sum of the squares of their difference between adjacent RR intervals, spectpow, and low frequency before and after the treatment in the patients who started piracetam treatment (P < 0.05). CONCLUSIONS: Our study is critical as it is the first to investigate the effects of treatment options on various HRV in patients with breath-holding spells. There were statistically significant changes in HRV parameters in patients receiving piracetam, and the number of attacks decreased significantly. Piracetam treatment contributes positively to the breath-holding spell with regard to efficacy and HRV, therefore it can be used to treat breath-holding spells.

One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Combined (dual) drug therapy for the treatment of patent ductus arteriosus: last approach prior to ligation.

OBJECTIVE: We aimed to evaluate the efficacy of combined (ibuprofen+paracetamol) medical therapy in cases of persistent haemodynamically significant patent ductus arteriosus that are resistant to standard medical monotherapy (ibuprofen and/or paracetamol) in this retrospective multi-centre study. METHODS: The combined therapy included the administration of 15mg/kg/dose of paracetamol every 6 h for 3 days and ibuprofen at an initial dose of 10mg/kg/dose followed by 5 mg/kg/dose every 24 h. After 2 days following the administration of the last dose, the researchers evaluated the efficacy of combined treatment by conducting an echocardiographic examination. RESULTS: Of all 42 patients who received combined therapy, 37 (88.1%) patients exhibited closure of the haemodynamically significant patent ductus arteriosus without requiring surgical ligation. Patients who did not respond to combined therapy had a higher mean birth weight and gestational age compared to those who responded (p < 0.05). CONCLUSION: The researchers believe the success of ibuprofen and paracetamol in haemodynamically significant patent ductus arteriosus treatment may be due to their synergistic efficacy and inhibition of the prostaglandin synthesis pathway through different enzymes. The results of our retrospective trial suggest that combination therapy with paracetamol and ibuprofen can be attempted when monotherapy is unsuccessful in treating haemodynamically significant patent ductus arteriosus, especially in centres without a surgical department.

Fechamento transcateter de fístula de artéria coronária com dispositivo Amplatzer Piccolo™ / Transcatheter closure of coronary artery fistula with Amplatzer Piccolo™ device

As fístulas das artérias coronárias são anomalias congênitas raras e caracterizam uma comunicação anormal entre as artérias coronárias e as câmaras cardíacas ou grandes vasos. Muitos dispositivos que incluem vários coils, plugues vasculares, oclusores e stents recobertos têm sido usados no fechamento transcateter de fístulas de artérias coronárias. Neste relato, realizamos com sucesso o fechamento de uma fístula de artéria coronária, entre a artéria descendente anterior e o ápice do ventrículo direito, usando o dispositivo Amplatzer PiccoloTM, anteriormente chamado de tamanho adicional do AmplatzerTM Duct Occluder. Em nossa opinião, o dispositivo Amplatzer PiccoloTM é adequado para oclusão transcateter de fístula da artéria coronária, podendo ser usado no lugar de plugues vasculares.

Coronary artery fistulas are rare congenital anomalies and characterize an abnormal communication between coronary arteries and cardiac chambers or major arteries. Many devices, including various coils, vascular plugs, occluders, and cover stent, have been used in transcatheter closure of coronary artery fistulas. In this report we successfully closed coronary artery fistula, between left anterior descending artery and right ventricle apex, by using Amplatzer PiccoloTM device, formerly called as AmplatzerTM Duct Occluder additional size. In our opinion, Amplatzer PiccoloTM device is suitable for transcatheter occlusion of coronary artery fistula and can be used instead of vascular plugs.

Serum Nickel and Titanium Levels after Transcatheter Closure of Atrial Septal Defects with Amplatzer Septal Occluder.

INTRODUCTION: There is a concern about release of nickel and titanium after implantation of nitinol-containing devices. OBJECTIVE: To evaluate serum nickel and titanium release after implantation of Amplatzer occluder. MATERIALS AND METHODS: In 38 pediatric patients with no history of nickel sensitivity, blood samples were drawn 24 hours before and 24 hours, 1, 3, 6, and 12 months after implantation. Nickel and titanium concentrations were measured by atomic absorption spectrophotometry. RESULTS: The median serum nickel level which was 0.44 ng/mL before the implantation increased to 1.01 ng/mL 24 hours after implantation and 1.72 ng/mL one month after implantation. The maximum level was detected 3 months after implantation, with a median level of 1.96 ng/mL. During follow-up, the nickel levels decreased to those measured before implantation. Serum nickel levels at the 24th hour, 1st month, and 3rd month following implantation were found to have increased significantly. No patients showed a detectable serum titanium level. DISCUSSION: This is the first study that evaluated both serum nickel and titanium release after implantation of the Amplatzer occluder. Our study shows that nickel is released from the device in the first few months after implantation. Therefore, in patients with nickel allergy, other devices may be considered.

Laron syndrome related to homozygous growth hormone receptor c.784>C mutation in a patient with hypoplastic pulmonary arteries.

Laron syndrome, also known as growth hormone insensitivity, is an autosomal recessive disorder characterised by short stature due to mutations or deletions in the growth hormone receptor (GHR), leading to congenital insulin-like growth factor 1 (IGF1) deficiency. Cardiac abnormalities, such as patent ductus arteriosus or peripheral vascular disease are rare in patients with Laron syndrome, but cardiac hypertrophy has been observed after IGF1 therapy. In this report, we present a 10-year-and-5-month-old girl with severe peripheral-type pulmonary artery hypoplasia and Laron syndrome related to homozygous GHR c.784>C mutation.

Blood pressure is normal, but is the heart?

BACKGROUND: There is no detailed strain analysis of cardiac functions in treated hypertensive pediatric patients. The aim of this study was to evaluate the cardio-protective effects of different drug classes in treated pediatric hypertensive patients. METHODS: Sixty non-obese-treated hypertensive patients with preserved left ventricular (LV) systolic function and 45 age-, sex-, and body mass index-matched healthy subjects underwent clinical evaluation, including 24-h ambulatory blood pressure monitoring, standard echocardiographic examination, tissue Doppler imaging, and two-dimensional Speckle Tracking Echocardiography. The patients were divided into two subgroups based on the effects of the drugs on the Renin Angiotensin Aldosterone System. The subgroup hypertension (HT) 1 received angiotensin-converting enzyme inhibitor or angiotensin receptor blocker, and HT 2 subgroup received calcium channel blocker, ß-blocker, or diuretics. RESULTS: There was no difference between the two groups and subgroups with respect to clinical, demographic, ABPM, ventricular volumes, ejection fraction, and tissue Doppler imaging (TDI) parameters. For patients and controls, respectively, global longitudinal strain was - 18.70 ± 3.41 versus - 21.01 ± 3.82 (P < 0.001), and global radial strain was 40.6 ± 9.8 versus 54.8 ± 12.8 (P = 0.004). Peak LV twist and peak LV torsion were not significantly different. The patient subgroup analyses with each other revealed no difference in systolic and diastolic myocardial deformation properties. CONCLUSIONS: Strain parameters were reduced in all treated hypertensive children compared to normotensive children, and the various cardiac mechanic parameters were similarly abnormal no matter what type of antihypertensive agent was used.

Rapid Improvement of Pulmonary Functions in Children After Transcatheter Closure of an Atrial Septal Defect.

There are very few studies in the literature on respiratory system functions and complications of children with an atrial septal defect (ASD). The aim of this study is to investigate the pulmonary functions and pulmonary complications before and after transcatheter closure in children with an ASD. In this study, pulmonary function test parameters of 30 ASD patients between 5 and 18 years of age who were eligible to be treated by transcatheter ASD closure were compared with 30 healthy children. The patients undergoing transcatheter ASD closure received pulmonary function tests (PFT) at baseline (1 day before ASD closure), and 3 months after the procedure. Forced vital capacity (FVC), forced expired volume in 1 s (FEV1), peak expiratory flow, and mean forced expiratory flow during the middle half of FVC were measured. The mean age of the 30 ASD patients was 9.59 ± 3.1 years; and 20 (66.6%) were female and 10 (33.3%) were male. The mean age of the control group was 10.15 ± 2.21 years, and 19 (63.3%) were girls and 11 (36.6%) were males. ASD patients had significantly reduced FVC (73.11 ± 24.6%; 86.05 ± 26.1; p = 0.001, respectively), and FEV1 (81.34 ± 26.2% and 99.2 ± 19.6%; p = 0.001; respectively) at baseline. But significant improvement was observed in FVC values in the 3rd-month post-closure comparison of the patient group with the control group (73.11 ± 24.6%; and 88.36 ± 14.5%; p = 0.01, respectively); FEV1 values (81.34 ± 26% and 99.54 ± 18.2%; p = 0.005, respectively) and mean forced expiratory flow between 25 and 75% of vital capacity (MEF25-75) values (94.6 ± 33.4% and 124.2 ± -24.1%; p = 0.01, respectively) were also improved. There was no statistically significant relationship between the PFT measurements at baseline and after closure of the defect and age at transcatheter closure, gender, body height, body weight, ASD diameter, Q p/Q s, right ventricle systolic pressure, or mean pulmonary artery pressure values. At the 3rd month of ASD closure, there was no significant difference in the comparison of the PFT values of the patient and control group. Disturbance in the significant flow limitation of the peripheral airway of ASD patients was observed with PFT. Better pulmonary outcomes were observed in ASD patients after transcatheter closure.

The Surgical Repair of a Hyperimmunoglobulin E Syndrome Associated Ascending Aortic Aneurysm.

Hyperimmunoglobulin E syndrome (HIES) is an immunodeficiency disorder that manifests itself by affecting more than one system. Arterial aneurysms are among the significant complications associated with HIES. Surgical procedures for patients with such aneurysms are uncommon. In this study, we aim to present the case and surgical repair of a male child who was previously diagnosed with HIES and presented with rapidly expanding ascending aortic aneurysm.

Strain and strain rate echocardiography in children with Wilson's disease.

OBJECTIVE: This study aimed to evaluate strain and strain rate echocardiography in children with Wilson's disease to detect early cardiac dysfunction. METHODS: In this study, 21 patients with Wilson's disease and a control group of 20 age- and gender-matched healthy children were included. All the patients and the control group were evaluated with two-dimensional (2D) and colour-coded conventional transthoracic echocardiography by the same paediatric cardiologist using the same echocardiography machine (Vivid E9, GE Healthcare, Norway) in standard precordial positions, according to the American Society of Echocardiography recommendations. 2D strain and strain rate echocardiography were performed after the ECG probes of the echocardiography machine were adjusted for ECG monitoring. Longitudinal, transverse and radial strain, and strain rate were assessed from six basal and six mid-ventricular segments of the left ventricle, as recommended by the American Society of Echocardiography. RESULTS: Left ventricular wall thickness, systolic and diastolic diameters, left ventricular diameters normalised to body surface area, end-systolic and end-diastolic volumes, cardiac output and cardiac index values were within normal limits and statistically similar in the patient and control groups (p > 0.05). Global strain and strain rate: the patient group had a statistically significant lower peak A longitudinal velocity of the left basal point and peak E longitudinal velocity of the left basal (VAbasR) point, and higher global peak A longitudinal/circumferential strain rate (GSRa) compared to the corresponding values of the control group (p < 0.05). Radial strain and strain rate: end-systolic rotation [ROT (ES)] was statistically significantly lower in the patient group (p < 0.05). Longitudinal strain and strain rate: end-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed that rotational strain measurement of the anterior and lateral segments of the patient group were statistically significantly lower than the corresponding values of the control group (p < 0.05). Segmental analysis showed statistically significantly lower values of endsystolic longitudinal strain [STSR (ES)] of the basal lateral (p < 0.05) and end-systolic longitudinal strain [SLSC (ES)] of the basal septal segment (p < 0.05) in the patient group. End-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed statistically significantly lower values of endsystolic longitudinal strain [SLSC (ES)] of the mid-anterior and basal anterior segments (p < 0.05), end-systolic longitudinal strain [STSR (ES)] measurements of the posterior and mid-posterior segments, end-systolic longitudinal displacement [DLDC (ES)] of the basal posterior, mid-posterior and mid-antero-septal segments in the patient group. CONCLUSION: Cardiac arrhythmias, cardiomyopathy and sudden cardiac death are rare complications but may be seen in children with Wilson's disease due to copper accumulation in the heart tissue. Strain and strain rate echocardiography is a relatively new and useful echocardiographic technique to evaluate cardiac function and cardiac deformation abnormalities. Our study showed that despite normal systolic function, patients with Wilson's disease showed diastolic dysfunction and regional deformation abnormalities, especially rotational strain and strain rate abnormalities.

Huge Left Atrial Pseudoaneurysm in a 5-Month-Old Baby Presented with Supraventricular Tachycardia.

UNLABELLED: Cardiac pseudoaneurysm is an extremely rare condition in children. It can be congenital or acquired and has been primarily described in the left side of the heart. The congenital form of pseudoaneurysm is thought to be related to muscular dysplasia of the muscular layer of the heart. The acquired form is related to myocardial infarction, infective endocarditis or chest trauma. In this report we described a 5-month-old baby with left atrial pseudoaneurysm who was referred to our hospital due to tachycardia and hemodynamic instability. After diagnosis of left atrial pseudoaneurysm, aneurysmectomy was performed and the left atrial wall was successfully repaired by surgery three days after admission. In conclusion, due to life threatening complications such as dissection, arrhythmias, compression of aneurysm and tromboembolic complications, pseudoaneurysm should be treated by surgery. KEY WORDS: Chest trauma; Left atrial pseudoaneurysm; Supraventricular arrhythmias.

[Carriage of Streptococcus pyogenes in primary school children: M-protein types, pyrogenic toxin genes, and investigation of the clonal relationships between the isolates]. / Ilkokul çocuklarinda Streptococcus pyogenes tasiyiciligi: M-protein tipleri, pirojenik toksin genleri ve izolatlar arasi klonal iliskinin arastirilmasi.

M-protein and pyrogenic toxins are the most important virulence factors of Streptococcus pyogenes, and they play significant role in the pathophysiology of acute rheumatoid fever and scarlet fever, respectively. In this study, the pharyngeal carriage of S.pyogenes of the primary school children, clonal relationship of the strains, M-protein types, and the presence of pyrogenic toxin genes were aimed to be investigated. A total of 668 throat cultures obtained from children (age range: 6-16 years) in two primary schools in our region, were included in the study. The clonal relationships of the isolated group A streptococci (GAS) strains were investigated by DiversiLab assay (BioMérieux, France), and the clonal relatedness was confirmed by pulsed-field gel electrophoresis (PFGE) method. M-protein (emm) typing was performed by DNA sequencing as suggested by Centers for Disease Control and Prevention (CDC). The genes encoding pyrogenic toxins, speA and speC, were investigated by an in-house multiplex polymerase chain reaction (PCR) method. S.pyogenes was isolated from 134 (20.05%) of the throat samples. The GAS carriage rate of the students aged ≥10 was statistically higher than those 7-9 years age group (%22 vs %16.4, p<0.05). The M protein gene could be characterized only among 123 isolates by DNA sequencing, and 20 different emm types were detected. The most frequent emm type was emm1 (n=38, 30.9%) followed by emm12 (n=18, 14.6%), emm89 (n=10, 8.1%), emm118 (n=9, 7.3%), and emm4 (n=7, 5.7%). Pyrogenic toxin genes were found in 25 (18.6%) of the isolates, including speA in 11 isolates (8.2%) and speC in 12 isolates (8.9%) and both genes were detected in 2 isolates (1.5%). Sixty-two different Rep (Repetitive extragenic palindromic)-PCR profiles were detected in 134 S.pyogenes isolates by DiversiLab method. Thirteen different clusters were formed by a total of clonally related 36 isolates revealing a strain clustering ratio of 26.9%. Clonal relationship of all isolates in the same cluster was confirmed by PFGE method. In this study, relatively high percentage of GAS carriage was observed among primary school children in our region. The coverage rate of the 30-valent vaccine was determined to be over 90% with respect to M-protein types. Since the pyrogenic toxin-encoding genes were found in one fifth of the isolates from the studied subjects, we concluded that the carrier population may also have high risk for scarlet fever. We also concluded that, the clonal relationship ratio determined among the isolates may be a risk in school transmission of GAS.

Combined effect of aerosolized iloprost and oxygen on assessment of pulmonary vasoreactivity in children with pulmonary hypertension.

OBJECTIVE: The evaluation of pulmonary vascular reactivity plays a significant role in the management of patients with pulmonary hypertension. Inhaled nitric oxide in combination with oxygen (O2) has become widely used as an agent for pulmonary vasodilator testing. However, inhaled nitric oxide is not available in many developing countries. Recently, aerosolized iloprost was suggested as an alternative to nitric oxide for this purpose. In the present study, aerosolized iloprost was used together with O2 in the pulmonary vasoreactivity test of children with severe pulmonary hypertension. Thus, the synergistic effect of both vasodilators was utilized without extending the duration of cardiac catheterization. METHODS: The prospective cohort study registered a total of 16 children with severe pulmonary hypertension whose median age was 4.5 years. Hemodynamic parameters were quantified before and after the vasoreactivity test. Increased left-to-right shunt, pulmonary vascular resistance of <6 Woods units (WU)/m(2) and a pulmonary-systemic resistance ratio of <0.3, as well as a decrease >10% in the pulmonary vascular resistance and pulmonary-systemic vascular resistance ratio after the vasoreactivity test were accepted as a positive response. The data were analyzed using Wilcoxon signed-rank and the Mann-Whitney U tests. RESULTS: Eleven children gave a positive response to the vasoreactivity test, while 5 children did not respond. Pulmonary vascular resistance dropped from 9.98 ± 1.39 WU/m(2) to 5.08 ± 1.05 WU/m(2) (p=0.013) and the pulmonary-systemic vascular resistance ratio fell from 0.68 ± 0.08 to 0.32 ± 0.05 (p=0.003) in the children who were responsive. No side effects were observed related to iloprost administration. CONCLUSION: Administration of inhaled iloprost in combination with O2 for pulmonary vasoreactivity testing can be useful for correctly identifying pulmonary vasoreactivity without extending the duration of cardiac catheterization.

Tissue Doppler, strain, and strain rate measurements assessed by two-dimensional speckle-tracking echocardiography in healthy newborns and infants.

OBJECTIVES: To evaluate cardiac maturational and haemodynamic alteration in healthy newborns and infants and determine reference values in this period using tissue Doppler, strain, and strain rate echocardiography. MATERIAL AND METHODS: The study included 149 healthy subjects. Babies from 1 day to 3 months were selected from the well-baby nursery department, and infants were selected from paediatric clinics during routine visits for health maintenance. Subjects were allocated to four groups: preterm (36-37 weeks, n = 32), term (≥ 38 weeks, n = 32), 1 month of age (n = 47), and 3 months of age (n = 38). Standard echocardiographic evaluations, pulsed wave Doppler, tissue Doppler echocardiography, strain, and strain rate studies were applied by the same person using a MyLab50 echo machine. Longitudinal and circumferential systolic strain and strain rate measurements were assessed by two-dimensional speckle-tracking echocardiography in all subjects. RESULTS: The longitudinal systolic velocity, strain, and strain rate values derived from left ventricle apical four-, three-, and two-chamber images, and circumferential systolic velocity, strain, and strain rate values derived from left ventricle short-axis images decreased from the base to the apex in all subjects (p < 0.001). CONCLUSION: Significant cardiac haemodynamic alterations occurred during the newborn and early infancy periods and were detected by tissue Doppler, strain, and strain rate echocardiography. Although two-dimensional speckle-tracking echocardiography is useful and can produce improved, reliable results in clinical practice, it has some limitations. Therefore, more studies on this issue are required.

Report of seven children with hepatopulmonary syndrome.

Hepatopulmonary syndrome is an important pulmonary vascular complication of liver disease. Its diagnosis is based on the presence of hypoxaemia and the demonstration of intrapulmonary shunting by contrast-enhanced echocardiography or perfusion lung scanning. Awareness of this condition is critical to improve the outcomes of patients with chronic liver disease and/or portal hypertension because hepatopulmonary syndrome receives additional priority on the waiting list for transplantation. A non-invasive measurement of the blood oxygen saturation with pulse oximetry is recommended as a screening tool for this syndrome. The aim of this report was to present clinical and laboratory findings and follow-up of seven paediatric patients who were diagnosed with HPS at our centre.

Serum Pentraxin 3 and hs-CRP Levels in Children with Severe Pulmonary Hypertension.

BACKGROUND: Pulmonary arterial hypertension secondary to untreated left-to-right shunt defects leads to increased pulmonary blood flow, endothelial dysfunction, increased pulmonary vascular resistance, vascular remodelling, neointimal and plexiform lesions. Some recent studies have shown that inflammation has an important role in the pathophysiology of pulmonary arterial hypertension. AIMS: The aim of this study is to evaluate serum pentraxin 3 and high sensitive (hs)-C reactive protein (hs-CRP) levels in children with severe pulmonary arterial hypertension (PAH) secondary to untreated congenital heart defects and evaluate the role of inflammation in pulmonary hypertension. STUDY DESIGN: Cross sectional study. METHODS: After ethics committee approval and receiving consent from parents, there were 31 children were selected for the study with severe PAH, mostly with a left-to-right shunt, who had been assessed by cardiac catheterisation and were taking specific pulmonary vasodilators. The control group consisted of 39 age and gender matched healthy children. After recording data about all the patients including age, gender, weight, haemodynamic studies and vasodilator testing, a physical examination was done for all subjects. Blood was taken from patients and the control group using peripheral veins to analyse serum Pentraxin 3, N-terminal pro-Brain Natriuretic Peptide (NT-ProBNP) and hs-CRP levels. Serum Pentraxin-3 levels were measured by enzyme linked immunosorbent assay (ELISA) and expressed as ng/mL. Serum hs-CRP levels were measured with an immunonephelometric method and expressed as mg/dL. The serum concentration of NT-proBNP was determined by a chemiluminescent immunumetric assay and expressed as pg/mL. RESULTS: Serum Pentraxin- 3 levels were determined to be 1.28±2.12 (0.12-11.43) in the PAH group (group 1) and 0.40±0.72 (0.07-3.45) in group 2. There was a statistically significant difference between the two groups (p<0.01). Serum hs-CRP levels were measured as 2.92±2.12 (0.32-14.7) mg/dL in group 1 and 0.35±0.16 (0.07-3.45) mg/dL in group 2. The hs-CRP level was increased in the PAH group to a significant degree (p<0.01). CONCLUSION: Our study showed that pentraxin 3 and hs-CRP levels were increased significantly in the PAH group. We consider that inflammation plays an important role in severe pulmonary hypertension and progressive pulmonary arterial hypertension in children with PAH.