Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor of the heart. In the literature, cardiac IMT is often described as an endocardial-based cavitary mass originating from the right side of the heart in infants and adolescents. In this article, we present a 5-year-old boy with a rare cardiac IMT who had no complaints and was diagnosed with murmur during his routine examination. Transthoracic echocardiography showed a homogeneous polypoid mass originating from the pulmonary valve, extending into the main pulmonary artery during systole and causing obstruction of the pulmonary artery and right ventricular outflow tract. Surgical resection of the tumor was performed successfully. There was no tumor recurrence in the control echocardiography at the postoperative first month.

Tissue Doppler and speckle tracking echocardiography assessment of left ventricular function in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis may lead to left ventricular (LV) dysfunction. This dysfunction can be documented by methods such as tissue Doppler echocardiographic (TDI) imaging and two-dimensional speckle tracking echocardiography (STE) in early stage. STUDY DESIGN: This was prospective cohort study. METHODS: A total of 34 patients diagnosed with cystic fibrosis (mean age and SD 9.9 ± 4.9 years) and 37 healthy control subjects with a comparable gender and age distribution (mean age 9.8 ± 4.3) were studied. The results for the two groups were compared along with the results of published reports. RESULTS: No significant relationship was found between the groups in terms of systolic and diastolic measurements of the interventricular septum and posterior left ventricular wall, and ejection fraction (P > .05). Myocardial performance indexes of left ventricular free wall and interventricular septum increased in the patient group compared with the controls (P < .05). As measured by STE, seven segments in the LV myocardial longitudinal strain and three segments in the LV myocardial circumferential strain showed significant reductions in patients with cystic fibrosis compared with controls (P < .05). The longitudinal global, circumferential global, and total global strain values had no significant difference between the groups (P > .05). Longitudinal strain rates and circumferential strain rates were both lower in five segments in the patient group (P < .05). CONCLUSIONS: Tissue Doppler echocardiographic imaging and STE may help identifying subclinical LV dysfunction in cystic fibrosis patients with unremarkable conventional echocardiography. They may be considered for the routine follow-up of cystic fibrosis patients.

The evaluation of right and left ventricular functions in children with episodic wheezing exposed to environmental tobacco smoke.

Doksöz Ö, Nacaroglu HT, Ceylan G, Çelegen M, Asik-Nacaroglu S, Can D, Mese T, Ünal N. The evaluation of right and left ventricular functions in children with episodic wheezing exposed to environmental tobacco smoke. Turk J Pediatr 2017; 59: 42-48. The objective of this study is to examine the right and left ventricular functions in children with episodic wheezing at the ages of 1-3 exposed to environmental tobacco smoke (ETS). Thirty-two children monitored at the Pediatric Allergy and Immunology Department of a Research and Training Hospital with the diagnosis of episodic wheezing were included. The prospective assessment of the patients was performed between May 2013 and February 2014. Twenty-five children with episodic wheezing not exposed to ETS formed the control group. The two groups were compared by conducting transthoracic two-dimensional and tissue Doppler echocardiography (TDE) examination in all of the cases. The average age of the study group (24 boys, 8 girls) was 33.1 ± 8.8 months, the average age of the control group (18 boys, 7 girls) was 31.9 ± 11.9 months. There was no statistically significant difference between the two groups in terms of age, gender, weight, height, and body mass index values. There was no statistically significant difference between the two groups for the right and left ventricular systolic and diastolic functions in the conventional echocardiographic measurements, and for the measurements of TDE. Limited number of patients is a major limitation of the study. These results should be supported by more comprehensive studies.

Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Both an excess of iron and iron deficiency (ID) may lead to significant cardiac problems. Parameters that represent ventricular repolarization heterogeneity, like QT dispersion (QTd), corrected QT dispersion (QTcd), the interval between the peak and the end of the T wave (Tp-e), and Tp-e dispersion, have not been evaluated in otherwise healthy children with low iron levels before. Here we assessed the effects of low iron storage on P wave dispersion (PWd), QTd, Tp-e intervals, and Tp-e dispersion in otherwise healthy children. We prospectively reviewed 283 patients who were referred to pediatric cardiology department for cardiac evaluation due to murmurs and who were found to have no structural heart disease. The patients were divided into three groups according to their ferritin levels: Group 1: ferritin <15 ng/mL (n = 58); Group 2: ferritin 15-25 ng/mL (n = 80); Group 3: ferritin >25 ng/mL (n = 145). P wave duration (PW), QT and Tp-e intervals, and PW, QT, corrected QT (QTc), and Tp-e dispersions were significantly higher in patients whose ferritin level was <15 ng/mL. A negative correlation was found between ferritin level and QT and QTc intervals, and QT, QTc, and Tp-e dispersions. Our results showed that a low serum ferritin level is associated with changes in some ECG parameters such as prolonged PWd, Tp-e interval, QT, QTc, and Tp-e dispersions in otherwise healthy children, and studies of other populations indicated that these parameters may predict arrhythmias in selected patients. These patients may be considered at some risk of developing arrhythmias. Therefore, careful evaluation of these ECG parameters is necessary in otherwise healthy children with low iron stores.

Different haemodynamic patterns in head-up tilt test on 400 paediatric cases with unexplained syncope.

OBJECTIVE: To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. METHODS: Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years (median 13; range 5-18), with unexplained syncope were enrolled in the study. Age, sex, baseline heart rate, baseline blood pressure, frequency of symptoms, and/or fainting attacks were recorded. The test protocol consisted of 25 minutes of supine resting followed by 20 minutes of 70° upright positioning. Subjects were divided into nine groups according to their differing haemodynamic patterns. RESULTS: There were no statistically significant differences between the groups with regard to age, gender, baseline blood pressure, and frequency of syncope (p>0.05). The response was compatible with orthostatic intolerance in 28 cases (7.0%), postural orthostatic tachycardia syndrome in 24 cases (6.0%), asymptomatic postural orthostatic tachycardia syndrome in 26 cases (6.5%), orthostatic hypotension in seven cases (1.7%), vasovagal syncope in 38 cases (9.5%), and negative in 274 cases (69.2%). Vasovagal syncope response patterns were of type 3 in nine cases (2.2%), type 2A in 10 cases (2.5%), type 2B in two cases (0.5%), and type 1 (mixed) in 17 cases (4.25%). CONCLUSIONS: In the 400 paediatric cases with unexplained syncope, nine different haemodynamic response patterns to head-up tilt testing were discerned. Children with orthostatic intolerance syndromes are increasingly referred to hospitals because of difficulty in daily activities. Therefore, there is need for further clinical trials in these patient groups.

Clinical and epidemiological features of Turkish children with 2009 pandemic influenza A (H1N1) infection: experience from multiple tertiary paediatric centres in Turkey.

BACKGROUND: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey. METHODS: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals. RESULTS: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis. CONCLUSIONS: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition.

Repair of isolated mitral papillary muscle rupture consequent to blunt trauma in a small child.

Blunt thoracoabdominal trauma is most often caused by high-velocity motor-vehicle accidents or by falls from a height. The clinical spectrum of cardiac injuries arising from this type of trauma varies from myocardial contusion to valvular rupture. Intracardiac valvular rupture is rarely observed, and few cases have been reported. The youngest of the patients in cases reported to date was 6 years of age. Here we report the case of a 2(1/2)year-old child, who sustained mitral valve insufficiency due to isolated rupture of the posterior mitral papillary muscle, which developed after a domestic accident.

Retrospective evaluation of 113 children with scoliosis.

To evaluate demographic features, preoperative pulmonary function tests and echocardiographic examinations of 113 children with scoliosis and to determine the associations between preoperative investigations and postoperative complications. Medical records, preoperative pulmonary function tests and echocardiographic examinations of 113 children with scoliosis who were operated between January 2004 and 2006 were evaluated retrospectively. Associations between preoperative pulmonary function tests, preoperative pulmonary symptoms and postoperative pulmonary complications were investigated. There were 37 male and 76 female patients. The mean ages of the patients at the time of the first and last surgery were 11.2 +/- 3.8 and 11.7 +/- 3.6 years respectively. The mean number of surgery performed for scoliosis was 1.5 +/- 1.4. Idiopathic scoliosis was the most common form constituting 42.5% of the patients. 68.1% of the patients had no preoperative pulmonary symptoms. Normal preoperative FVC, FEV(1), PEF and MEF(25-75) values were detected in 43.4%, 58.4%, 53.1% and 65.5% of the patients respectively. Echocardiograms were found normal in 34.5% of them. The most common cardiac valve anomaly was mitral valve prolapse which was detected in 25.7% of the patients. No significant associations were found between preoperative pulmonary function tests, preoperative pulmonary symptoms and postoperative pulmonary complications of children with scoliosis in our study.

Etanercept for therapy-resistant macrophage activation syndrome.

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report a 4-year-old girl with probable SoJIA who presented with MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). She also failed to respond to intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation of increased serum levels of tumor necrosis factor-alpha (TNF-alpha) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS.

Catheter-associated recurrent intracardiac thrombosis and factor V Leiden mutation in a child with non-Hodgkin's lymphoma.

Patients with cancer have an increased risk for thromboembolism, which might be related to several factors including central venous catheters and chemotherapeutics. Congenital prothrombotic risk factors might also contribute to thrombotic events. In this report, we present a catheter-related recurrent intracardiac thrombosis in a boy with non-Hodgkin's lymphoma and factor V Leiden mutation. Screening for factor V Leiden mutation in children with cancer and recurrent thrombotic events is recommended. Periodic echocardiography may be considered for a group of patients if the catheter tip is in the right atrium and therapy includes L-asparaginase and corticosteroids.

Pyogenic liver abscesses in a child spreading to pulmonary and subcutaneous tissues: case report.

Pyogenic liver abscess is a rare and life-threatening disease in children. Our case is noteworthy because of the rapid advancement of liver abscesses without any other systemic disorder. A 16-year-old girl was admitted to the hospital with fatigue, pallor, weight loss and high fever. In physical examination a fluctuating mass was observed under the scapular area and hepatosplenomegaly was found. In computed tomography, three septated cystic lesions which looked like abscesses were demonstrated in the liver. The abscess was drained through percutaneous route. Right pleural empyema with clinical features of adult respiratory distress syndrome appeared after the first day of treatment. Bacteroides sp. was isolated from pus. On the twentieth day of the therapy, control abdominal computed tomography revealed two new abscesses in the liver. They were drained and the antibiotic therapy was continued with ticarcillin-clavulanate, fluconazole and levofloxacin. By the end of the first week of the therapy, the fever of the patient had abated. This therapy was continued for four weeks; 15 days after the end of the therapy there was prominent healing of the liver lesions with only one necrotic remnant 2 cm in diameter on abdominal computed tomography.