Evaluation of electrocardiographic parameters for early diagnosis of autonomic dysfunction in children and adolescents with type-1 diabetes mellitus.

BACKGROUND: The aim of this study was to identify the sensitivity of electrocardiogram (ECG) in early diagnosis of cardiac autonomic function disorder in children with type 1 diabetes mellitus. METHODS: A total of 150 children and adolescents with type 1 diabetes mellitus were enrolled between June 2009 and June 2010, as well as 100 age- and sex-matched healthy control children. Twelve-lead ECG was done in all cases and heart rate, QT and QTc interval, dispersion of P wave (Pd), and of QT (QTd) and QTc interval (QTcd) were measured. The clinical and demographic features such as age, gender, duration of follow up and level of HbA1c and fasting glucose were obtained and the effects of these parameters on ECG measurements were investigated. RESULTS: The mean age of the patients and controls was 11.61 ± 3.72 years and 10.92 ± 3.2 years, respectively. QT and QTc interval and QTcd interval were significantly higher in diabetic children compared to healthy controls but these ECG findings were not associated with the duration of diabetes or glycemic state. Pd was significantly higher in the diabetic patients with HbA1c >7.5% compared to control, and this was also found in patients that were followed up >1 year. CONCLUSIONS: Cardiac autonomic function disorder, which is one of the most important causes of morbidity and mortality, may emerge in the course of type 1 diabetes mellitus. It can be diagnosed on ECG even when the patients are asymptomatic.

Congenital anomalies of coronary arteries in children: the evaluation of 22 patients.

Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing coronary angiography, the data for the pediatric population are few. This study of 22 children with coronary artery anomalies evaluated them in terms of demographic and clinical features and analyzed their angiographic findings and surgical results. Databases in the Department of Pediatric Cardiology at the University of Uludag were searched for all the patients with a diagnosis of congenital coronary artery anomaly who underwent coronary angiography between 1993 and 2013. Patients with coexistent congenital heart disease were excluded from the study. The study noted 22 patients (0.9 %; 10 boys and 11 girls) with coronary artery anomalies. The mean age of these patients was 58.77 ± 52.04 months (range, 1 month-16 years). Coronary arteriovenous fistula (50 %) and anomalous left coronary artery from the pulmonary artery (ALCAPA) (36 %) were the most common anomalies. In addition, the study included one patient with diffuse coronary artery hypoplasia, one patient with muscular bridge, and one patient with left main coronary artery originating from the right aortic sinus valsalva. Of the 11 patients who had coronary atrioventricular fistula, 7 were asymptomatic, whereas 75 % of the patients with ALCAPA syndrome were admitted because of heart failure. Although 13 patients had an exact diagnosis by echocardiography, 50 % of the patients with ALCAPA syndrome had their diagnosis determined by catheter angiography performed because of severe mitral regurgitation or dilated cardiomyopathy. The mortality rate for all the patients was found to be 18.1 %. Eight patients with coronary arteriovenous fistula have been followed up without surgery to the present. In contrast, seven patients with ALCAPA syndrome have undergone surgery, and three have died. Two of these patients died during the postoperative period, and the remaining patient died suddenly during the preoperative period at home. Isolated congenital coronary artery anomalies are very rare in the pediatric population. Although most congenital coronary artery anomalies are clinically silent, they may be associated with severe symptoms in children. Recognition of potentially serious anomalies such as ALCAPA syndrome is mandatory so that early surgical treatment can be prescribed.

Evaluation of subvalvular aortic stenosis in children: a 16-year single-center experience.

Subvalvular aortic stenosis accounts for 1-2 % of all congenital heart disease and for 8-20 % of cases of left-ventricular outflow tract (LVOT) obstruction in children. Recurrence of subaortic stenosis (SAS) is not uncommon after surgical management. This study was performed to investigate the clinical and surgical outcomes and to estimate the predictability of recurrences of SAS. Seventy-nine patients age 3-21 years with SAS between 1994 and 2010 were reviewed. Fifty-one patients had discrete SAS, whereas the remaining 15 patients had fibromuscular ridge-type SAS. Mean follow-up time without surgery was 22 months (range of 1-94). Forty-one patients with a diagnosis of SAS underwent surgery. Recurrence rates were 22.7 % (15 patients), and these patients developed SAS at a mean of 4.7 years follow-up. We performed second surgical membrane resection in only 1 patient. The risk of recurrence of SAS was only linked to higher preoperative LVOT gradient. Twenty-three patients had no aortic regurgitation (AR) at preoperative echocardiography. Of these, 39.1 % had trivial, 8.7 % had mild, and 8.7 % had moderate AR after surgery; there was no significant AR. We conclude that surgical intervention was required most of the time in patients with SAS, and surgical outcomes was excellent even if there were associated cardiac defects. The risk of recurrences was higher, especially in patients with higher initial LVOT gradients, although a second surgery was rarely necessary in these patients.

Thrombolysis with recombinant tissue plasminogen activator in 7 children.

The information about the thromboembolic events, the optimal treatment choice, the dose, and duration of antithrombotic therapy in children are limited. More clinical data are required. Recombinant tissue plasminogen activator (r-tPA) is increasingly used in pediatric thrombosis. We retrospectively analyzed the clinical course of 7 children (9.3 ± 2.1 years; 34 days to 16 years) with arterial thrombosis (n = 1) and intracardiac thrombosis (n = 6). The children were treated with r-tPA. The dose ranged between 0.2 and 0.4 mg/kg per h infused for 3 to 4 hours. This dose was repeated between 2 to 7 times till the thrombolysis was achieved. Treatment side effects were closely monitored. Complete clot lysis was achieved in all cases. None of them had severe bleeding except mild recurrent epistaxis occurring in 2 cases. In conclusion, r-tPA is an effective and safe therapy under close hemostatic control in children.

Early left ventricular diastolic dysfunction in premature infants born to preeclamptic mothers.

AIM: To evaluate the cardiac function in premature infants born to preeclamptic mothers and its clinical consequences. METHODS: This was a prospective observational cohort study performed in a tertiary neonatal intensive care unit. Fifty-three premature infants born to preeclamptic mothers comprising the study group were evaluated and compared with 42 premature infants born to normotensive mothers (control group). Relationship between echocardiographic measures and neonatal morbidity were assessed as the main outcome measures. RESULTS: Left ventricle end-diastolic dimension (LVEDD), peak flow velocities during early diastole (peak E wave), peak flow velocities during atrial contraction (peak A wave), and peak E/A ratio were significantly lower in the study group. Within the study group, these parameters were also significantly lower in infants with respiratory problems. LVEDD was significantly smaller in preeclamptic infants with intrauterine growth retardation (IUGR). CONCLUSION: Left ventricle diastolic dysfunction (LVDD) was detected in premature infants born to preeclamptic mothers in the first week after delivery. LVDD was associated with higher incidence of respiratory problems, transient tachypnea of the newborn, longer duration of oxygen requirement, and IUGR.

Carvedilol therapy in pediatric patients with dilated cardiomyopathy.

Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. The objective of this study was to determine the dosing, efficacy and side effects of carvedilol for the management of dilated cardiomyopathy in children. Sixteen children with idiopathic dilated cardiomyopathy, aged 7 months to 138 months and with an ejection fraction less than 40%, were treated with carvedilol. The average initial dose was 0.1 mg/kg/day and it was uptitrated to 0.4 mg/kg/day. After six months on carvedilol, there were improvements in clinical scoring system from an average of 2.94 to 2.50 (p<0.05), in mean fractional shortening from 17.2 +/- 6.1% to 22.7 +/- 5.1% (p<0.05), and in ejection fraction from 35.2 +/- 6.8% to 43.1 +/- 11.2% (p<0.05). No side effect was observed during the study period. Two patients died due to serious infection. Carvedilol in addition to standard therapy for dilated cardiomyopathy in children improves cardiac function and symptoms. It is well tolerated, with minimal adverse effects, but close monitoring is necessary.

Suspicion, diagnosis, and successful surgical treatment of anomalous origin of the anterior descending coronary artery from the pulmonary trunk.

Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare. We suspected such anomalous origin in a boy aged 2.5 years owing to the echocardiographic demonstration of mitral regurgitation and abnormal diastolic flow in the ventricular septum. The diagnosis was confirmed by angiography. The anomalous artery was successfully reimplanted into the aorta.

Risk of vasovagal syncope and cardiac arrhythmias in children with mitral valve prolapse.

OBJECTIVE: Postural phenomena, cardiac arrhythmias and autonomic dysfunction are responsible for presyncope and syncope in patients with mitral valve prolapse (MVP). In this study, arrhythmia and vasovagal syncope incidence were investigated in children with MVP. METHODS: Between April 2005 and December 2006, 37 children with MVP and 26 healthy children were evaluated.Telecardiography, electrocardiography (ECG), echocardiography, Holter monitoring, exercise test and head-up tilt test were performed. RESULTS: The MVP group consisted of 19 boys and 18 girls with a mean age of 11.8 years. The control group was similar with respect to age and gender. Telecardiography, ECG, Holter monitoring, exercise test and QTc of all children were within normal limits. There was a statistically significant difference between the two groups in terms of QT dispersion. The tilt table test was positive in 11 of 37 (29.7%) children with MVP and in 1 of 26 (3.8%) normal healthy children. This difference was statistically significant (P < 0.01). CONCLUSION: Arrhythmia and syncope frequency was found to be higher in children with MVP than in the normal population. The risk of vasovagal syncope indicated by a positive tilt test was found to be increased in children with MVP. Therefore, patients and families must be informed about the conditions that may predispose to vasovagal syncope and caution should be recommended in these patients.

Eisenmenger syndrome: identifying the clues for arrhythmia.

OBJECTIVE: The aim of this case-controlled, cross-sectional study is to investigate the tendency towards arrhythmia using noninvasive arrhythmia markers (QT dispersion and heart rate variability) in children with Eisenmenger syndrome. METHODS: We studied 23 patients, whose pulmonary-to-systemic resistance ratio was calculated to be greater than 0.75, and who were diagnosed as Eisenmenger syndrome between 1990 and 2001. Twenty healthy children were studied as the control group. Electrocardiographic recordings with calculation of QT dispersion, Holter monitoring, echocardiographic studies and heart rate variability (HRV) analysis were performed in both groups. Catheterization records were analyzed in all the patients. RESULTS: QT and QTc dispersion were higher (p=0.007 and p=0.006, respectively) and PR interval was longer (p=0.009) in the patients with Eisenmenger syndrome, than those in the control group. In addition, low frequency component, high frequency component, very low frequency component, and total power, obtained from HRV analysis were significantly lower in the patients with Eisenmenger syndrome (p=0.001, p=0.006, p=0.009 and p=0.011, respectively). Evaluation of Holter recordings revealed pathologic findings in 21.7% of the patients with Eisenmenger syndrome. Pulmonary-to-systemic resistance ratio of the patients with pathologic Holter findings were higher than in the patients with normal Holter recordings (p=0.011). It was also shown that there was a positive correlation between OT dispersion and pulmonary-to-systemic resistance ratio (p=0.048, r=0.416) and between QT dispersion and PR interval (p=0.009, r=0.532) in the patients with Eisenmenger syndrome. CONCLUSION: Dispersion of repolarization, being associated with high pulmonary-to-systemic resistance ratio, is increased and autonomic modulation of heart rate is impaired in patients with Eisenmenger syndrome. These findings suggest that arrhythmia risk for patients with Eisenmenger syndrome is higher than in normal controls.

The effect of age and gender on the electrocardiogram in children.

Our aim was to determine, using a computer program for measurement and analysis, the effects, if any, of age and gender on the electrocardiographic measurements in a large cohort of Turkish children. We analyzed standard simultaneous 12-lead electrocardiograms from 2241 healthy Turkish children, aged from 1 day to 16 years, to calculate limits of normality of a variety of electrocardiographic measurements for 12 age groups. Clinically significant differences were shown to exist, and the results compared with previously established normal limits. Differences were demonstrated for gender in measurements of both amplitude and duration, particularly in the adolescent period. We have provided tables and figures showing age and gender-dependent means and upper limits of normal electrocardiographic parameters for the important leads.

Treatment of infective endocarditis with recombinant tissue plasminogen activator.

Infective endocarditis (IE) caused by microbial infection is virtually always fatal if untreated. High-dose and long-term antibiotic treatment is required to eradicate microorganisms. If increased risk of embolic events, persistent infection, and progressive cardiac failure are present, surgery is indicated. However, surgery can carry an increased risk of mortality and morbidity in critically ill children of whom other treatment options such as administering, a thrombolytic agent; recombinant tissue plasminogen activator (r-tPA) could be an alternative choice. Here, we report a 14-year-old male with Down syndrome and acute myeloblastic leukemia, diagnosed with IE characterized by two large vegetations on aortic and mitral valves, who was successfully treated with r-tPA.

Bilateral multiple pulmonary arteriovenous fistulas and duplicated renal collecting system in a child with Noonan's syndrome.

Noonan's syndrome involves the association of multiple congenital abnormalities, with a variety of cardiac defects. We describe here the association of Noonan's syndrome with multiple pulmonary arteriovenous fistulas and bilateral duplicated renal collecting systems. To the best of our knowledge, this is the first reported case of an association of the Noonan phenotype with pulmonary arteriovenous fistulas.

Permanent form of junctional reciprocating tachycardia and tachycardia-induced cardiomyopathy treated by catheter ablation: a case report.

The permanent form of junctional reciprocating tachycardia (PJRT) is usually refractory to drug therapy, and these patients are at risk of developing tachycardia-induced cardiomyopathy. The electrocardiogram insribes inverted P waves in leads 2, 3, aVF as well as left lateral leads, along with a P-R interval shorter than R-P interval during the tachycardia. This report describes a three-year-old male patient with PJRT who underwent successful radiofrequency catheter ablation (RFA) of accessory pathway. On transthoracic echocardiography of patient, decreased ventricular systolic function was observed. RFA was performed by applying radiofrequency pulses. Echocardiograms of the patient, two months after catheter ablation, demonstrated progressive improvement of ventricular function. Transcatheter radiofrequency ablation of accessory pathways in patients with PJRT is an effective, and possibly preferable, form of treatment, especially in cases of tachycardia refractory to multiple pharmacologic treatments or when left ventricular dysfunction is present.