Fatma Sedef Tunaoglu

Chest pain in children referred to a cardiology clinic

One hundred consecutive patients (54 girls, 46 boys) referred to a pediatric cardiology department with the primary complaint of chest pain were evaluated. The age distribution was 2.5–16.0 years (mean 11.3 years for girls and 9.9 years for boys). The history showed 17% of patients with chest pain, 22% with heart disease, and 19% with recent death in the family. The time course of the pain was longer than 1 week in 92 patients. Localization was on the left precordium in 60 patients, and there was no radiation from the original site in 66 cases. Ninety-two percent of cases were idiopathic in origin. Of the 74 patients who had a psychiatric interview, 55 (74%) had psychiatric symptoms and 5 required psychiatric care. Anxiety, conversion disorder, and depression were the main psychiatric symptoms.

Persistent left superior vena cava: Experience of a tertiary health-care center

Background:  The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC.

Abnormality of the Left Ventricular Sympathetic Nervous Function Assessed by I‐123 Metaiodobenzylguanidine Imaging in Pediatric Patients with Neurocardiogenic Syncope

The purpose of this study was to assess the left ventricular sympathetic nervous system function in the patients with neurocardiogenic syncope (NCS) using I‐123 metaiodobenzylguanidine (MIBG) imaging of the heart, and to compare the plasma noradrenaline (NA) and MIBG results of tilt positive and tilt negative patients following a head‐up tilt test (HUT). The study included 30 patients. Their physical and laboratory examinations did not show a pathology that may be the cause of their syncope. HUT test was positive in 13 patients and negative in 17 patients. Plasma NA concentrations were higher in the HUT positive than the HUT negative group at the beginning and at the 10th minute of the test. Specific I‐123 MIBG uptake assessed as the cardiac to mediastinal activity ratio in the delayed image was significantly higher in HUT positive group. The higher levels of MIBG uptake and plasma NA observed in HUT positive patients may reflect the greater capacity of NA storage in cardiac adrenergic neuronal tissue in patients with NCS. The results of this study support the critical role of autonomic nervous system in the pathophysiology of NCS and the excessive sympathetic nervous stimulation as the trigger of paradox reflex. (PACE 2003; 26:1926–1930)

A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

Abstract. Muscular dystrophies are a group of sex-linked diseases with frequent myocardial involvement. In this study 14 patients with Duchenne Muscular Dystrophy (DMD), 7 with Becker Muscular Dystrophy (BMD), and 8 female carriers who were asymptomatic were evaluated with echocardiography and multigated radionuclide ventriculography (MUGA). All showed predominant systolic and minor diastolic left ventricular dysfunction determined both by echocardiography and MUGA when compared with healthy controls. In conclusion, it is recommended that DMD, BMD, and female carriers be evaluated and closely monitored for cardiac functions. Though MUGA and echocardiography are both effective and sensitive techniques, echocardiography is more convenient for availability and serial evaluation.

Cardiac side effects of long-acting beta-2 agonist salmeterol in asthmatic children

Abstract Background: Salmeterol is a new long‐acting β2 selective adrenoreceptor agonist. There are some reports about the cardiac side‐effects of salmeterol in asthmatic adults. The aim of this study was to determine the cardiac side effects of salmeterol in children.

Reference Values of the 6-Minute Walk Test in Healthy Turkish Children and Adolescents Between 11 and 18 Years of Age

INTRODUCTION: Normal values of the 6-min walk distance (6MWD) for children have not been well demonstrated. This limits the interpretation of the 6MWD in children. METHODS: A cross-sectional prospective study was designed. A total of 949 (467 males, 482 females) healthy children were recruited randomly from 7 Turkish secondary schools in Ankara. The 6-min walk tests were conducted according to the American Thoracic Society guidelines. RESULTS: The mean 6MWD decreased between the ages of 12 and 14 y for both boys and girls, and then gradually increased until 17 y of age. In the best fitting and most efficient linear, quadratic, and categorical regression models, the age of the subjects, heart rate after the test, and physical activity level of the subjects were significantly related to the 6MWD. Nevertheless, these variables explained < 20% of the variance in the 6MWD. CONCLUSIONS: The 6MWD does not increase in a straight linear fashion from the age of 12 until adulthood. Correlation of the 6MWD with anthropometric features is very weak, so in evaluating the 6MWD, the standard curves should be used.

Traditional single patch versus the "Australian" technique for repair of complete atrioventricular canal defects.

PurposeA variety of operative techniques has been used to repair complete atrioventricular (AV) canal defects and satisfactory outcomes after single patch repairs have been reported. We report our comparative results of repairing complete AV canal defects between 1998 and 2006 using the traditional single patch and the “Australian” techniques.MethodsFourteen patients underwent traditional AV canal repair with the single patch technique (Group 1) and 11 patients underwent repair with the “Australian” technique (Group 2). All patients were examined with preoperative echocardiography and cardiac catheterization, and were followed up with echocardiography to evaluate AV valve and ventricular function.ResultsThere were two early postoperative deaths in Group 1 and one in Group 2. One patient from each group had moderate left AV valve regurgitation postoperatively, but none from either group had left ventricular outflow obstruction.ConclusionsThe “Australian” technique is a simpler method requiring shorter aortic cross-clamping and total bypass times with good clinical and functional results. The early postoperative results are as encouraging as those achieved by the traditional single patch technique; however, long-term follow-up results are required to establish the efficacy of this simplified technique.

Echocardiographic findings in children with Marfan syndrome

Background The typical cardiac manifestations of Marfan syndrome are aortic regurgitation with progressive dilatation of the aortic root, which may cause dissection and rupture of the ascending aorta, mitral valve prolapse and mitral valve regurgitation. In this study, we aimed to show echocardiographic findings in 11 patients with Marfan syndrome. Methods Diagnosis of Marfan syndrome was based on the Ghent criteria. All patients had a full echocardiographic evaluation. During the evaluation, we investigated the presence of mitral valve prolapse, mitral valve regurgitation, tricuspid valve prolapse, dilatation of the aortic root, and aortic regurgitation. Results Eleven patients were diagnosed as Marfan syndrome (seven male, four female, age 4–14 years). All had mitral valve prolapse (nine with mitral valve regurgitation). Among these 11 patients, seven had accompanying tricuspid valve prolapse, six had dilatation of the aortic root and two had aortic regurgitation. Conclusion Eleven patients in our clinic were diagnosed as Marfan syndrome since they had distinct characteristics of marfanoid phenotype. Echocardiographic evaluation of these patients showed marked heart valve involvement. In Marfan syndrome, it is known that the aortic valve is affected following mitral valve involvement. In our experience, aortic root dilatation is less common. However, particular attention should be given to following up aortic root status with non-invasive echocardiography to institute measures to prevent complications.

Fibrillin-1 gene intron 56 polymorphism in Turkish children with mitral valve prolapse.

OBJECTIVE Mitral valvar prolapse is the most common anomaly of the mitral valve apparatus throughout childhood. Fibrillin is one of the structural components of the elastin-associated microfibrils found in the mitral valve. A case-controlled study has performed to investigate the relationship between fibrillin 1 gene intron 56 polymorphism and risk of mitral valvar prolapse in Turkish children. PATIENTS AND METHODS A total of 77 patients with mitral valvar prolapse diagnosed by clinical evaluation and echocardiography and 89 normal children of same age and sex were studied. The fibrillin-1 gene intron 56 polymorphism was identified by the polymerase chain reaction-based restriction analysis. RESULTS There was a significant difference in the distribution of fibrillin-1 gene intron 56 genotypes (p = 0.0001) and allelic frequency (p = 0.0001) between the cases and the controls. CONCLUSIONS Patients with mitral valvar prolapse have higher frequencies of fibrillin-1 gene intron 56 GC genotypes. Healthy children have higher frequencies of fibrillin-1 gene intron 56 CC genotypes. We speculate that the higher frequency of fibrillin-1 gene intron 56 G-allele increases the risk of mitral valvar prolapse.

Evaluation of Dysrhythmia in Children with Muscular Dystrophy

Myocardial involvement and dysrhythmia are common findings with muscular dystrophy and are among the leading causes of death. The authors evaluated rhythm and conduc tion abnormalities in children with muscular dystrophy by electrocardiography, signal- averaged electrocardiography, and Holter monitoring. Twenty-nine patients (mean age, 8 years) and 29 healthy control subjects were included in the study. Sixty-two percent of patients had electrocardiographic abnormalities defined as deep Q waves in V 6, tall R waves in V1, and QRS axis deviation. The cardiomyopathy index was significantly greater in the patient group whereas QT and QTc dispersion values showed no significant difference. Holter monitoring revealed premature atrial and ventricular contractions more frequently than normal. However all were classified as Lown I and II. Mean heart rate was significantly higher in the patient group. The elec trocardiograms of 41% of the patients showed late potentials. No relationship with these changes and cardiac function was observed. During the study, one patient died whose cardiomyopathy index was longer and had late potentials detected with signal- averaged electrocardiography. In conclusion, standard electrocardiography, cardiomyopathy index, signal-averaged electrocardiography, and Holter monitoring are valuable and reliable monitoring methods in children with muscular dystrophy.