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Biomedical subjects

A Celiker

Publications and source records attributed to A Celiker.

At least 73 records · Page 4Linked to original sources

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↗

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↗

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↗

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↗

Neonatal lupus syndrome: report of a case.

Neonatal lupus syndrome (NLS) is characterized by the presence of cutaneous lupus, congenital heart block or both associated with maternal autoantibodies anti-Ro (SS-A) and anti-La (SS-B). NLS is responsible for more than 80 percent of cases with isolated congenital complete heart block. This report describes a neonate with third-degree atrioventricular block who was born to an anti-Ro (SS-A) positive mother. The dermatological and hematological manifestations of NLS were absent. The infant's clinical condition was good during the two months of follow-up with expectant management.

Adult↗

Absent pulmonary valve syndrome with agenesis of the left pulmonary artery.

Absent pulmonary valve syndrome is represented by rudimentary nodules without identifiable leaflets, and it is commonly associated with a ventricular septal defect and tetralogy of Fallot. Unilateral absence of a pulmonary artery is also a rare congenital anomaly. The following is a case report of a one-day-old newborn with cyanosis. The physical examination revealed a to-and-fro murmur and no expansion of the left hemithorax. Echocardiography revealed tetralogy of Fallot, absent pulmonary valve, and enlarged main and right pulmonary arteries. The catheterization and angiography confirmed the diagnosis and absence of the left pulmonary artery.

Cyanosis↗

Ambulatory electrocardiographic monitoring in healthy newborn infants.

Twenty-four hour ambulatory electrocardiographic monitoring is a useful method to document dysrhythmias and to assess treatment response. Various studies have been done in the pediatric age-group to determine normal heart rate values. In this study we determined the heart rate and rhythm patterns of 25 healthy newborn infants whose ages ranged between 3-10 days (mean 6.5 days). There were 15 males and 10 females. The maximum heart rate in these infants was 175-231 beats/min (207 +/- 14), minimum heart rate 60-121 beats/min (93 +/- 16) and the average heart rate was 130-161 beats/min (143 +/- 9). Five infants (20%) demonstrated marked sinus dysrhythmia, seven infants (28%) had ventricular premature contractions, two infants (8%) had supraventricular premature contractions, and five infants (20%) showed junctional rhythm disturbance. The sinus pause did not exceed 1.2 sec and there was no evidence of atrioventricular conduction disorders, or supraventricular-ventricular tachycardia attacks. Our results were consistent with previous studies carried out in newborn infants. Dysrhythmias were detected during 24-hour ambulatory electrocardiographic monitoring in our study group. Since they were generally benign, they need no treatment.

Arrhythmia, Sinus↗

Familial sick sinus syndrome in two siblings.

Two siblings with sinus node disease are presented. The patients were severely affected and required permanent pacing. A cardiac electrophysiologic study was conducted in Case 2, which revealed an atrioventricular conduction disturbance in addition to sinus node dysfunction. The parents and other siblings showed no evidence of sinus node disorder. The occurrence of this disease in these siblings suggests an autosomal dominant mode of inheritance with variable penetrance.

Bundle of His↗

Long-term results of patients with congenital complete atrioventricular block.

Congenital complete atrioventricular block is an uncommon disorder with a prognosis which is usually favorable. The disorder is sometimes associated with syncope, sudden death or cardiac pacing. It is difficult to determine the patients at risk of sudden death. In this retrospective study, hospital records of children who had been admitted to the Hacettepe University Children's Hospital between 1970-1990 for evaluation of complete heart block were examined. The study population, consisting of 39 children, 27 males and 12 females, had electrocardiograms consistent with complete heart block. These patients, diagnosed as having congenital complete A-V block, had an otherwise normally structured heart, and were followed up for a period of from one month to 15 years. Age at diagnosis ranged from 27 days to 17 years (mean: 86.9 +/- 48 months). Of the 14 patients with symptoms (five with syncope, eight with exercise intolerance and one with presyncope), nine were paced electively and have done well. Clinical and laboratory features of the asymptomatic and symptomatic groups were compared to evaluate potential risk factors.

Adolescent↗

"Acquired" subvalvular aortic stenosis after repair of several congenital cardiac defects.

Discrete subvalvular aortic stenosis is a progressive lesion. In this report we presented nine patients who had no significant left ventricular-aortic obstruction at initial cardiac catheterization or echocardiographic examination, but later developed significant subvalvular aortic stenosis. Associated lesions included ventricular septal defect in three, patent ductus arteriosus in two, aorticopulmonary window in one, tetralogy of Fallot in one, supramitral membrane in one, and ventricular septal defect and patent ductus arteriosus in one case. Nine patients were diagnosed with subvalvular aortic stenosis 18 months to eight years after surgical correction. Eight of the patients required surgery for subvalvular obstruction. In conclusion, discrete subaortic stenosis is a rare, late complication of the surgical repair of several congenital heart defects. It is a progressive lesion after surgery; therefore these patients require careful follow-up.

Aortic Stenosis, Subvalvular↗

Transcatheter embolization of a pulmonary collateral vessel in a child with tricuspid atresia: case report.

When a significant systemic-pulmonary arterial collateral connection occurs in children with cyanotic congenital heart disease, they may present surgical difficulties making operative management undesirable. Transcatheter vessel occlusion can be a life-saving procedure or an important adjunct in reducing blood loss during surgery. This report describes the selective obliteration of a large systemic pulmonary arterial collateral vessel in a child with tricuspid atresia before Fontan operation. A transcatheter wire coil embolus technique was used, and no complications or errors in placement of the coils occurred.

Aorta, Thoracic↗

Blade atrial septostomy: experience with 18 patients.

Blade atrial septostomy was performed in 18 patients, including 14 with transposition of the great arteries, three with tricuspid atresia and one with severe mitral stenosis and ventricular septal defect. The patients' ages ranged from 25 to 210 days (mean: 108 +/- 64 days), and weights ranged from 3.0 to 6.0 kg. Following blade septostomy, partial arterial pressure of oxygen (PaO2) increased from an average of 22 +/- 3.3 mmHg to 38.9 +/- 7.6 mmHg (p < 0.001), and prompt clinical improvement was observed in the majority of patients. Significantly decreased atrial pressures (from 8.16 +/- 6.1 to 3.93 +/- 2.2 mmHg, p < 0.01) and significantly enlarged inter-atrial openings (from 1.6 +/- 0.9 to 7.5 +/- 1.8 mm, p < 0.001) were achieved with successful blade atrial septostomy. Blade atrial septostomy is an effective and life-saving procedure in patients who require nonrestrictive inter-atrial communication.

Echocardiography↗

Radiofrequency catheter ablation of accessory pathways and modification of atrioventricular node in children and adolescents.

Catheter ablation of an accessory pathway and atrioventricular node modification using 550 kHz radiofrequency was attempted in 23 children and adolescents between five and 19 years of age (mean = 15.7 years). Fifteen children had accessory-pathway-mediated tachycardia and eight had atrioventricular node reentrant tachycardia. Accessory pathways were present in ten children. Two patients had associated congenital heart disease. Symptoms included disabling palpitations and episodes of syncope. Ablation was attempted from the right and left sides of the heart. Single-catheter technique was used in seven patients. Eleven of the 15 patients with accessory pathways were treated completely. Two patients had recurrences, and one of them died after the arrhythmia surgery. There were two failures. Two patients with incomplete interruption of the accessory pathways were followed up clinically. There were three cases with temporary systemic embolization and one with severe pain related to radiofrequency energy application. Atrioventricular node modification was done by fast-pathway ablation in six, and by slow-pathway ablation in two patients. Two patients with fast-pathway ablation had recurrences of clinical arrhythmia, and one of them underwent a successful second session. There were no complications associated with the procedure in this group of patients. Radiofrequency catheter application was initially successful in 21 (84%) out of 25 procedures, and ultimately curative in 16 (69%) out of 23 patients. There were some serious complications which resolved in the immediate post-procedure period. Radiofrequency catheter ablation appears to be a safe and successful method for the management of supraventricular tachycardia secondary to accessory pathways or atrioventricular node reentrant tachycardia in children and adolescents.

Adolescent↗

Short- and intermediate-term efficacy of amiodarone in infants and children with cardiac arrhythmia.

The antiarrhythmic effect of amiodarone was examined in this retrospective study in a group of 20 patients with a mean age of 8.5 +/- 6.7 years (range 42 days to 20 years, median 9 years). Five patients with atrial flutter, one patient with atrial fibrillation, two patients with an intermediate rhythm between atrial flutter and atrial fibrillation, four patients with chaotic atrial tachycardia, three patients with atrioventricular reentry tachycardia, two patients with junctional ectopic tachycardia, and three patients with ventricular arrhythmias were treated with amiodarone. The mean duration of therapy was 9.1 +/- 12.3 months (range 1 month to 4 years). Before amiodarone treatment, 18 patients had been unresponsive to various antiarrhythmic drugs (range 1-8, median 2). Two patients received amiodarone as an initial therapy. It was administered orally at a dose of 10 mg/kg once per day for 10 days and then decreased to 5 mg/kg once per day. Amiodarone was effective in 16 patients (80%). Side effects occurred in three patients, including thyroid dysfunction, elevation of liver enzymes, and keratopathy. All side effects disappeared upon cessation of the therapy. We recommend amiodarone for the treatment of childhood arrhythmias, especially for the refractory types.

Administration, Oral↗

Bicuspid aortic valve and coarctation of aorta.

Bicuspid aortic valve (BAV) and coarctation of aorta (COA) are frequently seen together. It is believed that these malformations result from a single developmental diathesis. A case is presented of COA, aortic stenosis and aneurysm of the ascending aorta corrected by patch aortoplasty and commisurotomy. An aneurysm at the site of the coarctation repair can develop as late as 20 to 25 years after surgery. Almost all of the aneurysms described have occurred in patients undergoing patch aortoplasty. We do not recommend this technique except in special cases.

Aortic Aneurysm↗

Incidence and severity of arrhythmias and conduction disturbance after repair of tetralogy of Fallot.

Ventricular and supraventricular arrhythmias and conduction disturbances were evaluated by routine electrocardiography and 24-hour ambulatory monitoring in 31 patients who underwent correction of tetralogy of Fallot. The interval from operation to the study was 1 month to 14 years (mean 4.8 +/- 2.8). Complete right bundle branch block occurred in 22 (71%) patients and incomplete right bundle branch block in 9 (29%) patients. Bifascicular block with right bundle branch block and left axis deviation (LAD) occurred in one patient. Two patients had second degree type II atrioventricular block. Twenty-four-hour ambulatory electrocardiographic monitoring was performed in all patients and they were divided in two groups according to the frequency of ventricular arrhythmias (Lown classification). Group 1 included the 23 patients who had no arrhythmia or rare ventricular arrhythmias (Lown grade 0-1). Group 2 was comprised of eight patients (26%) with significant ventricular arrhythmias (Lown grade 2-5). Twelve patients (39%) had supraventricular arrhythmias, three patients rare supraventricular tachycardia attacks, and seven patients occasional supraventricular ectopies. One patient had bradycardia-tachycardia attacks and one patient had junctional tachycardia. There was no correlation between age at the time of surgery and ventricular arrhythmias. Of the patients who had ventricular and supraventricular arrhythmias of various degrees on ambulatory monitoring, two had significant arrhythmias on routine electrocardiogram. Symptoms were rare in these patients. In conclusion, both supraventricular and ventricular arrhythmias were found in considerable frequency in our patients. As ventricular arrhythmias may be the cause of sudden death and supraventricular arrhythmias are a main cause of morbidity, it is important to evaluate ventricular and supraventricular arrhythmias by ambulatory monitoring in patients who have undergone correction of tetralogy of Fallot.

Adolescent↗

Vasovagal syncope: asystole provoked by head-up tilt testing under sertraline therapy.

Syncope is defined as a sudden transient loss of consciousness. Vasovagally mediated hypotension and bradycardia are believed common, yet difficult to diagnose, causes of syncope in healthy children and adolescents. These episodes are often both sudden and sporadic in nature and, if recurrent and severe (malignant vasovagal syncope), can be a source of morbidity and possibly mortality. Head-up tilt testing has emerged as a useful investigation in patients who are thought to have recurrent vasovagal syncope with systemic hypotension, bradycardia, or both, and it has been suggested as a potential method to test for vasovagal episodes. Sertraline hydrochloride, a serotonin reuptake inhibitor, has been reported to be effective in preventing the vasovagal syncopal episodes in children and adults. Here, two cases of recurrent, unexplained syncope are presented. Both were under sertraline therapy and underwent provocative head-up tilt testing that resulted in asystole.

1-Naphthylamine↗