Valvar heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), the aortic valve being most commonly affected. Reported cases with symptomatic mitral involvement are rare. We describe a 13-year-old boy with seronegative, polyarticular onset of JRA in whom mitral and aortic valve insufficiency was diagnosed by clinical and laboratory investigations. Two-dimensional and continuous-wave Doppler echocardiography confirmed mild pericardial effusion with moderate mitral and mild aortic insufficiency. Cardiac assessment and echocardiographic follow-up are recommended in all patients with JRA.

Adolescent↗

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Parameters of iron deficiency in children with cyanotic congenital heart disease.

A group of 67 children with cyanotic congenital heart disease (CCHD) were studied, and 35 were given iron treatment according to a regimen that gives iron to patients with a hematocrit (Hct) below 60%. The patients were categorized as iron-deficient and iron-sufficient according to their transferrin saturation and ferritin values. The pretreatment hemoglobin (Hb) and Hct values of the groups were similar. The mean Hct was nearly three times as much as the mean Hb in the iron-sufficient group and more than three times as much as the Hb in the iron-deficient group. Excessive erythrocytosis in the iron-deficient group was impressive. Mean corpuscular volume (MCV) values were below 72.7 fl in all of the iron-deficient patients. After treatment the Hb, Hct, transferrin saturation, and ferritin increased significantly in both groups, with the increments greater in the iron-deficient group. Increments in the erythrocyte (RBC) count were significant in the iron-sufficient group but insignificant in the iron-deficient one. Increments of MCV in the iron-deficient group were significant but insignificant in the iron-sufficient group. Our study demonstrated that prediction of Hb, RBC count, and MCV, measurements of which are easy and inexpensive and require little blood, can suffice for the diagnosis of iron deficiency in patients with CCHD without altering systemic perfusion.

Anemia, Hypochromic↗

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Vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: a rare cause of respiratory distress in newborn infants.

Vascular ring, in which the trachea and esophagus are completely surrounded by vascular structures, is one of the causes of respiratory distress in children. Right aortic arch with aberrant left subclavian artery is a common aortic arch anomaly; however, respiratory distress due to vascular ring is seldom associated with this anomaly. We report herein a newborn infant treated surgically because of severe respiratory distress caused by vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. As laryngomalacia was first thought to be the reason for respiratory distress, we suggest that patients with respiratory distress diagnosed with laryngomalacia be evaluated for possible vascular ring.

Abnormalities, Multiple↗

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The angiocardiographic analysis of 73 patients with double-outlet right ventricle.

The purpose of this study was to determine the cardiac anatomy of patients with double-outlet right ventricle by angiocardiography. A total of 73 patients between the ages of one day and 11 years were examined. The aorta was on the right side of the pulmonary artery in 23 cases (32%), right anterior in 20 (27%) and right posterior in 17 cases (23%). Pulmonary stenosis was found in 53 patients (73%) and subaortic stenosis in six cases. Ventricular septal defect (VSD) was subaortic in 39 cases (52%), remote type in 17 (23%), doubly committed in 10 (13%), and subpulmonic in 9 (12%). Double VSD was noted in two patients. Pulmonary hypertension was more frequent in subpulmonic ventricular septal defect (78%). The most common associated anomalies were atrial septal defect (34%), anomalous coronary arteries (12%) and endocardial cushion defect (10%). Aortic root angiography was not satisfactory in half of the cases with coronary arterial anomaly. In conclusion, double-outlet right ventricle is a complex anomaly, all of whose cardiac features can be successfully demonstrated in detail by echocardiography and angiocardiography. However, in order to determine the anatomy of the coronary arteries, selective coronary angiography may be necessary in some patients.

Angiocardiography↗

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Adenosine-sensitive right ventricular tachycardia in children.

Right ventricular outflow tachycardia is a distinct subgroup of idiopathic ventricular tachycardia (VT) with characteristic clinical and electrophysiologic properties. This study was planned to evaluate the effects of adenosine on idiopathic right ventricular tachycardia in children, and to assess the long-term efficacy of verapamil treatment. Diagnostic tests including electrocardiogram, chest roentgenogram, echocardiogram, exercise stress test and 24-hour ambulatory electrocardiographic monitoring were performed in each patient. Adenosine was administered in increasing amounts until an effective dose or a maximal dose of 300 micrograms/kg was reached. In adenosine-sensitive patients verapamil was given orally (3-10 mg/kg/day) for long-term suppression of arrhythmia. Seven patients with a mean age of 10.2 +/- 4.7 years were enrolled in the study. There were five male and two female patients. Six patients had VT, and one patient had frequent ventricular ectopic bests. Arrhythmia originated from the right ventricle in all patients. Adenosine was effective in terminating the arrhythmia in all patients, with a mean effective dose of 183 +/- 75 micrograms/kg. Favorable long-term results (mean follow-up period 17 +/- 8 months) were obtained with verapamil treatment at an average dose of 6.3 +/- 2.2 mg/kg. In conclusion, adenosine can be used effectively for termination of VT originating from the right ventricular outflow tract in children. In these patients verapamil may be considered as the drug of choice for long-term therapy.

Adenosine↗

PubMedDate not supplied

Analysis of 2017 Holter records in pediatric patients.

Holter monitoring (HM) is widely used in arrhythmic disorders of adult patients; however, studies in the pediatric age group are limited. This study aims to determine the value of HM in diagnosis and treatment of disorders related to arrhythmias in pediatric patients. We examined 2,017 Holter records of 1,500 children who applied to our institution between November 1994 and October 1998. The age ranged from 0-24 years (52% male, 48% female). The indications for HM were screening for arrhythmic symptoms (palpitation, chest pain, syncope) in 67 percent, monitoring dysrhythmic therapy in 17 percent, postoperative control in five percent, and pacemaker control in four percent. Palpitation is the leading presenting symptom, with more frequent findings of supraventricular extrasystole (SVE), supraventricular tachycardia (SVT), ventricular extrasystole (VE) and complete heart block (CHB) when compared to other symptoms. Only 5.3 percent of the patients had arrhythmic symptoms during monitoring and asymptomatic patients had more frequent arrhythmias. SVT, VE, and CHB are more frequent findings in the abnormal heart with previous cardiac operations. The diagnostic yield is low with arrhythmic symptoms in the pediatric age group; however, HM enables cumulative evaluation of heart rhythm and rhythm variability, which is important in diagnosing silent arrhythmias in high risk groups (abnormal heart, postoperative heart).

Adolescent↗

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Congenital sick sinus syndrome with breath holding and severe syncope episodes during infancy. A case report.

Sick sinus syndrome is a rare cause of bradycardia in children without structural heart disease. A case of profound sinus bradycardia, sinus arrest with junctional escape, and pauses in a two-year-old infant with breath-holding and syncope episodes is presented. As a result of these clinical symptoms and electrocardiographic findings, the patient with sick sinus syndrome underwent implantation of transvenous ventricular pacemaker. He has been well and asymptomatic since the insertion of the pacemaker. In the differential diagnosis of an infant with breath-holding and syncope episodes, when these symptoms in particular cannot be explained by other common reasons, sick sinus syndrome should be kept in mind. This case also illustrates the importance of electrocardiographic studies for the diagnosis.

Apnea↗

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Echocardiographic findings in endomyocardial fibrosis.

An 18-month-old infant diagnosed as having endomyocardial fibrosis by echocardiography is presented. Most patients with endomyocardial fibrosis reported in the literature are either older children or adults. To our knowledge, our patient was the youngest ever to have been reported. Echocardiographic studies showed obliteration of the left ventricular apex and increased echo reflectance at the left ventricular endocardium and subendocardium. The left atrium and right ventricle were significantly enlarged. Doppler echocardiography showed minimal mitral, but significant tricuspid regurgitation. In regard to the contribution of echocardiography in the diagnosis, we recommend this method for suspected cases. Contrary to the other patients reported, there was no thickening of the atrioventricular valves. Mitral valve insufficiency was related to the restriction of the ventricular filling rather than to valve involvement occurring with the disease.

Cineangiography↗

PubMedDate not supplied

DRB1, DQA1, DQB1 genes in Turkish children with rheumatic fever.

OBJECTIVES: Several studies have suggested that genetic susceptibility to rheumatic fever (RF) may be linked to HLA Class II alleles. The purpose of this study was to examine the association between HLA Class II genes and RF in Turkish children. METHODS: DNA typing HLA Class II genes (DRB1, DQA1, DQB1) were performed in 55 children with RF and 50 healthy unrelated controls using sequence specific primers (SSP). RESULTS: The frequency of the HLA DQA1*03 (OR: 0.462, p < 0.05) allele was significantly decreased in the patient group. Also, the frequency of the combination of DRB1*04 and DQA1*03 allele (OR: 0.42, p < 0.01) was more significantly decreased in the patient group. Differences in frequencies of the DRB1 and DQB1 alleles between groups were not significant. CONCLUSIONS: Our data indicate that the HLA DQA1*03 allele may be a protecting factor in Turkish children with RF. Our results also suggest that the combination of the DRB1*04 and DQA1*03 alleles may be a stronger protective factor than the DQA1*03 allele alone.

Adolescent↗

PubMedDate not supplied

Idiopathic long Q-T syndrome.

The association of Q-T interval prolongation, syncope and sudden death is known as the long Q-T syndrome. The syndrome may be familial, associated with congenital deafness, or idiopathic. The diagnosis is based on the electrocardiographic finding of a prolonged Q-T interval with or without T wave abnormalities. In this article, we present a nine-year-old boy admitted to the Hacettepe University Children's Hospital with complaints of syncopal episodes. The prolonged Q-T syndrome was diagnosed as the cause of the syncopal attacks. In addition, the same syndrome was detected in his father. The prolonged Q-T syndrome should be considered in the differential diagnosis of cases with recurrent, unexplained syncope in the pediatric age group.

Child↗

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Experience with propafenone for the treatment of cardiac arrhythmias in children.

The aim of this prospective study was to evaluate the efficacy of propafenone in suppressing various arrhythmias in a group of children who were patients in the Pediatric Cardiology Unit of Hacettepe University Children's Hospital. Seven of the 13 children (53.8%) with ventricular ectopics, all three children with paroxysmal re-entry atrioventricular tachycardia, and two of the three children with paroxysmal atrial tachycardia were treated successfully with propafenone. The drug did not cause any side effects or arrhythmogenesis. The median dosage of propafenone at discharge was 300 mg/m2/day (range 250-400 mg/m2/day) every eight hours. Our findings confirm the efficacy and safety of propafenone for the treatment of childhood arrhythmias.

Adolescent↗

PubMedDate not supplied

Detection of intracardiac left-to-right shunts in children by contrast echocardiography and the radionuclide technique.

This study was carried out in the Departments of Pediatrics and Nuclear Medicine at Hacettepe University Faculty of Medicine between April 1986 and March 1988 with the aim of detecting left-to-right shunts in 70 patients with isolated ventricular septal (VSD) defect, isolated atrial septal defect (ASD) and patent ductus arteriosus, by using the radionuclide technique together with contrast echocardiography. In some patients, the results were confirmed by both cardiac catheterization and angiocardiography. Most of the data were confirmed during surgery. A good correlation was found between the results of contrast echocardiography and radionuclide angiography. The difference between the QP/QS value and the grade of positive contrast effect was tested statistically and "p" was found to be less than 0.001. Therefore, we conclude that these methods could be applied together to determine the indication for surgery in cases with ASD or VSD.

Adolescent↗

PubMedDate not supplied

Exercise-induced ventricular tachycardia in children.

Two children with exercise-induced tachycardia, one with idiopathic long-QT syndrome, are presented. The patients were evaluated by exercise testing and electrophysiologic study. From the onset of treatment with the beta-blocking agent, pindolol, the patients have been symptom-free. These findings emphasize that children with syncope must be evaluated by ECG, exercise testing, 24-h Holter-monitoring, and finally, electrophysiological study.

Adolescent↗

PubMedDate not supplied

Percutaneous transluminal balloon pulmonary valvuloplasty: immediate and medium-term results.

Ten patients with pulmonary stenosis, (six males and four females) whose ages ranged between five and ten years (mean age 7 years) underwent cardiac catheterization and balloon valvuloplasty. Right ventricular systolic pressure before valvuloplasty ranged from 90 to 200 mm Hg (mean 133.5 +/- 33.3 mm Hg). It fell to 50-90 mm Hg (mean 64.5 +/- 13.8 mm Hg) immediately after the procedure. The peak systolic gradient across the pulmonary valve before valvuloplasty ranged from 70 to 180 mm Hg (mean 114.5 +/- 35.4 mm Hg) and decreased significantly to 30-70 mm Hg (mean 43.0 +/- 13.8 mm Hg) immediately after dilation. Doppler echo studies confirmed these results. At repeat cardiac catheterization in three patients, five to 15 months after valvuloplasty, restenosis was noted in one patient while there was no change in the others. All patients had been followed up by Doppler echocardiography. Patients with isolated valvular pulmonary stenosis can be adequately and safely treated with balloon valvuloplasty which results in excellent immediate and medium-term results.

Catheterization↗