An evaluation of heart rate variability and its modifying factors in children with type 1 diabetes.

OBJECTIVE: To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. METHODS: This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. RESULTS: The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = -0.263 and -0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = -0.751 and -0.644) and between very low frequency and diabetes duration. CONCLUSION: A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Heart failure after transvenous closure of atrial septal defect associated with atrial standstill and thiamine-responsive megaloblastic anemia.

Despite advances in device closure for atrial septal defect, post-closure heart failure remains a clinical problem in adult patients but is seen only rarely in children. An eight-year-old boy, who had been followed by a local pediatrician with the diagnosis of diabetes mellitus and congenital heart disease, was consulted to us for cardiac re-evaluation. Electrocardiography demonstrated absent P waves, and echocardiography revealed enlargement of the right ventricle and both atria and secundum atrial septal defect. With the diagnosis of atrial standstill, secundum atrial septal defect and thiamine-responsive megaloblastic anemia, acute heart failure developed after transvenous closure of the atrial septal defect, which improved dramatically with thiamine and supportive treatment.

Strain and strain rate echocardiography findings in children with asymptomatic congenital aortic stenosis.

The aim of our study was to evaluate myocardial functions with strain/strain rate echocardiography in asymptomatic patients having congenital aortic stenosis (CAS) with normal cardiac functions as determined by conventional echocardiographic techniques and comparing them with those of healthy controls. A total of 58 patients with various degrees of isolated CAS and 52 healthy controls were enrolled in this study. Conventional and two-dimensional speckle tracking (2DSTE) echocardiography were performed. Global longitudinal strain (LS) (-23.1 ± 3.6 and -23.8 ± 4.7), and longitudinal strain rate (LSR) (-1.49 ± 0.32 and -1.76 ± 0.39) values were lower, whereas circumferential strain (CS) (-25.9 ± 4.7 and -22.8 ± 6.4) and circumferential strain rate (CSR) (-1.82 ± 0.46 and -1.69 ± 0.49) values were greater in the patient group than in the control subjects. The difference was significant for global LSR and CS (p < 0.05) values. Regional analysis showed lower LS values in the basal part of the left-ventricular (LV) free wall and lower LSR values in the basal parts of both of the septum and free wall in the patient group (p < 0.05). CS values in the anteroseptal, posterior, and inferior walls were significantly greater in the patients (p < 0.05). 2DSTE detects subtle alterations in myocardial function in asymptomatic children with CAS. Impairment of LV long-axis function occurred earlier and was more prominent in basal parts of the interventricular septum and the free wall of the left ventricle.

Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

Acute rheumatic fever in the Central Anatolia Region of Turkey: a 30-year experience in a single center.

UNLABELLED: The study was carried out in Dr. Sami Ulus Children's Hospital to investigate and to compare the incidence and findings of acute rheumatic fever patients (ARF) seen in the past 30 years. The medical records of 1,115 patients with ARF admitted to Dr. Sami Ulus Children's Hospital Department of Pediatric Cardiology during 1980-2009 were retrospectively analyzed. Twenty-one percent of those patients were admitted between 1980 and 1989, 44.6% between 1990 and 1999, and 34.2% between 2000 and 2009. The highest incidence was detected in the second decade with a rate of 60.0:100,000. Male/female ratio was 1:18. The age of patients ranged between 2 and 15 years. Carditis was detected in 64.7% of patients, arthritis in 59.1%, and chorea in 14.1%. Mitral regurgitation was the most common echocardiographic finding. Heart failure was detected in 13.8%. Recurrent attacks occurred in 8.1% of patients. The median follow-up was 6.8 years (range, 1.2-10.5 years). The prevalence of chronic rheumatic valvular disease was 58%. Mortality rate was 0.8%. CONCLUSION: Although the incidence of ARF has decreased in the last decade, it still continues to be an important public health problem in Turkish pediatric population.

Thiamine-responsive megaloblastic anemia syndrome with atrial standstill: a case report.

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is an uncommon autosomal recessive disorder. The disease is caused by mutations in the gene, SLC19A2, encoding a high-affinity thiamine transporter, which disturbs the active thiamine uptake into cells. Major features include megaloblastic anemia, diabetes mellitus, and sensorineural deafness. Cardiac malformations with conduction defects and/or dysrhythmias, have also been described in some patients. To our knowledge, only 13 TRMA patients with cardiac defects have been reported. Here, we describe the first case of TRMA syndrome with atrial standstill, probably caused by a 2 base-pair deletion in exon 4 (1147delGT) of the gene SLC19A2.

Gastrointestinal system malformations in children are associated with congenital heart defects.

OBJECTIVE: To determine the frequency of congenital heart defects (CHD) in children with gastrointestinal malformations (GISM) and mortality rates in patients with GISM. METHODS: Two hundred and forty two consecutive children patients with GISM followed up in Pediatric Surgery Clinics of our hospital were examined for cardiovascular anomaly by the Department of Pediatric Cardiology, and the CHD incidence was investigated by examining the records of the patients retrospectively. Chi-square test was used for the statistical analysis of data. RESULTS: Two hundred and forty two patients with gastrointestinal system malformations were included in the study. Of 242 patients, 135 (55.8%) were male and 107 (44.2%) were female, and their age range was 0-15 years. The most frequent GISM were anorectal malformations (43.2%), atresia involving stomach, ileum or colon (21%) and esophageal atresia/tracheoesophageal fistula (18.3%). Congenital heart defects were observed in 28.5% of the participants. The most frequent defects were as follows; atrial septal defect (31 patients, 44.9%) a, ventricular septal defect (17 patients, 24.6%) and patent ductus arteriosus (5 patients, 7.2%). There was no significant difference (p>0.05) in mortality rate in patients with CHD (16.7%) and without CHD (13.3%) undergoing operations for GISM. CONCLUSION: We would like to emphasize the importance of the earliest possible cardiological evaluation of all patients with gastrointestinal system malformations.

Ventricular non-compaction in children: clinical characteristics and course.

Isolated left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep intratrabecular recesses. In this study, we aimed to identify the clinical characteristics of children with ventricular non-compaction and determine the factors affecting prognosis. We retrospectively evaluated 29 children with LVNC followed at Dr. Sami Ulus Children Hospital Pediatric Cardiology Department from December 2004 to November 2009. There were 13 females (45%) and 16 males (55%) and the mean age at presentation was 4.8±4.6 years (one month-15 years). Although there was no statistical significance; early presentation age and high left ventricular end-diastolic diameter at the diagnosis were associated with poorer prognosis.

Transcatheter occlusion of tortuous feeding vessel with new vascular plug in an infant with pulmonary sequestration with Scimitar syndrome.

We report the case of a 9-month-old girl with a Scimitar syndrome which was feeding via an anomalous vessel dividing into three tortuous branches and supplying blood to a localized region of the right lower lobe which was successfully occluded using an Amplatzer® Vascular Plug 4 (AGA Medical, Golden Valley, MN, USA). The percutaneous procedure performed via femoral artery, provided an immediate closure of the vessel, excluding the need for surgical repair. This new device is found to be safe and effective for occlusion of the collaterals, especially large ones in pediatric ages.

Plasma endothelin-1 and nitrate levels in Down's syndrome with complete atrioventricular septal defect-associated pulmonary hypertension: a comparison with non-Down's syndrome children.

Children with Down's syndrome (DS)-associated complete atrioventricular septal defect (AVSD) have rapid and aggressive development of pulmonary vascular disease when compared with non-Down's syndrome (ND) children. We aimed to evaluate the role of plasma endothelin-1 (ET-1) and nitrate levels in DS children with complete AVSD-associated pulmonary hypertension (PH) and compare this to ND patients. The study included 20 patients (11 males, nine females) who had complete AVSD associated with PH. Comparisons were made between DS patients (n=12) aged 4 to 8 months (median 5 months) and ND patients (n=8) aged 4 to 12 months (median 7 months). Blood samples were drawn from the inferior vena cava, pulmonary artery, pulmonary vein, and aorta. The plasma ET-1 concentrations of the two groups were compared to the peripheral venous and arterial ET-1 levels, and pulmonary vein nitrate was compared to the peripheral arterial nitrate levels of ten healthy infants. The mean pulmonary artery (PA) pressure and pulmonary vascular resistance (Rp) were significantly higher in the DS group than ND patients, and the pulmonary blood flow (Qp) in ND patients was higher than DS patients. There were no differences between the two study groups in regard to plasma ET-1 and nitrate levels obtained from matched sampling sites. The plasma ET-1 and nitrate levels were significantly higher in both study groups compared to the control subjects. The plasma ET-1 and nitrate levels in DS patients with PH were not different when compared to those of ND patients.

Prognostic value of heart rate turbulence and heart rate variability in children with dilated cardiomyopathy.

The aim of our study is to evaluate the prognostic value of heart rate turbulence and heart rate variability in children with dilated cardiomyopathy (DCM). Twenty-five children with DCM and 24 age- and sex-matched healthy children who were admitted between January 2002 and September 2004, enrolled in this prospective study at our hospital. After the echocardiographic examination, three-channel 24-ambulatory ECG recordings were obtained in all patients with DCM and in the control group. Time domain heart rate variability parameters were obtained in both groups. Heart rate turbulence was measured in DCM patients, but we could not calculate heart rate turbulence in the control group since no ventricular ventricular premature complexes (PVC) were found in the 24-hour ECG monitoring in the control group. The mean follow-up period of the DCM group was 13.4 months (3-26 months). Five patients died (20%) during the follow-up period. Triangle index, turbulence slope (TS), age and availability of nonsustained ventricular tachycardia (VT) on 24-hour ECG monitoring were prognostic factors according to the correlation analyses. Only triangle index was detected as an independent risk factor among the prognostic factors according to the logistic regression analyses. This study assessed the prognostic value of heart rate turbulence and heart rate variability in children with dilated cardiomyopathy. Further studies are needed to investigate the prognostic value of heart rate turbulence.

Heart rate variability and autonomic dysfunction in SSPE.

Autonomic nervous system involvement in subacute sclerosing panencephalitis was studied in 29 patients by analysis of heart rate variability and compared with a control group which consisted of 20 age- and sex-matched healthy subjects. Holter recordings for 24 hours were obtained, and all recordings were analyzed using time-domain parameters. The patients with subacute sclerosing panencephalitis were found to have significantly lower values of standard deviation of all normal sinus intervals and triangular index when compared with the control group. Of 23 patients who had regular follow-up, 15 died in a period ranging from 1 to 13 months. Twelve of these patients had heart rate variability parameters lower than normal, but no significant difference was observed between the heart rate variability values of patients who survived or died. Also, there was no association between brain magnetic resonance imaging findings and heart rate variability indices. It is concluded that patients with subacute sclerosing panencephalitis have autonomic dysfunction; and this was thought to be mainly due to central involvement.

Tolmetin and salicylate therapy in acute rheumatic fever: Comparison of clinical efficacy and side-effects.

BACKGROUND: The arthritis of rheumatic fever is very responsive to treatment with salicylates, but there are many adverse reactions, especially hepatotoxicity, due to aspirin (acetylsalicylic acid) therapy. These side-effects change the course and duration of rheumatic fever. Other non-steroidal anti-inflammatory drugs may be equally effective, although no reports are available. METHODS: We studied 72 patients with rheumatic fever who were admitted to Dr Sami Ulus Children's Hospital between 1995 and 1999. Twenty patients with arthritis were treated with tolmetin (25 mg/kg per day; group I) and 52 patients with arthritis and/or mild carditis were put on aspirin therapy (75-100 mg/kg per day) for 4-6 weeks (group II). Arthritis had disappeared at the same time in both the aspirin and tolmetin groups (P = 0.675). RESULTS: The erythrocyte sedimentation rates of patients upon admission, at the first week and at the end of therapy were not different in the two groups (P > 0.05). No adverse effect of tolmetin therapy was observed, whereas side-effects of salicylate were observed in 19 patients (36.5%) in the aspirin group. Hepatotoxicity, gastric irritation and salicylism were found in 16, four and three patients, respectively. Renal toxicity and Reye syndrome were not demonstrated. Because of these side-effects of aspirin, therapy had to be stopped for 10-20 days and the duration of hospitalization in this group was lengthened unnecessarily. CONCLUSION: Tolmetin was safe and effective treatment for arthritic rheumatic fever patients without carditis. Tolmetin can be used particularly in patients who cannot tolerate aspirin.

Cardiovascular changes in children with pneumonia.

Pneumonia is an important cause of death in childhood, especially in the infancy period. Since the respiratory and cardiovascular systems cannot be thought of independently, it is important to detect cardiovascular changes during pneumonia. We prospectively studied 50 children aged 2-24 months admitted to the hospital because of pneumonia in order to evaluate their cardiovascular findings with noninvasive methods. Patients were classified according to the World Health Organization (WHO) criteria of pneumonia, and evaluated by obtaining complete blood counts, serum electrolytes, renal and liver function tests, blood gases, creatinine kinase MB fraction (CK-MB), chest X-ray, electrocardiography (ECG) and echocardiography at the admission and after clinical recovery. Right ventricular systolic pressure (RVSP) was found to be high in 70% of patients and there was a correlation between the severity of the pneumonia and RVSP All patients had normal left ventricular dimensions and functions, but in 68% of them CK-MB was elevated. In these infants, T voltages in D1 derivation showed a significant rise with clinical recovery. Seven cases developed congestive heart failure, and three died. CK-MB was found to be high in all patients who died and in six of seven patients with congestive heart failure. Our findings suggest that myocarditis incidence in children with pneumonia may be higher than previously known. Since myocarditis can be a fatal disease, its detection is important by noninvasive techniques like ECG, echocardiography and cardiac enzyme measurements.