Prothrombin gene 20210 G-A mutation in Turkish patients with thrombosis.
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Biomedical subjects
Publications and source records attributed to A Celiker.
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BACKGROUND: Experience with pediatric use of intravenous amiodarone is limited. In this study, our experiences with intravenous amiodarone in children with acute life-threatening or chronic tachyarrhythmias are reviewed. METHODS AND RESULTS: Twelve patients, with a mean age of 3.4 +/- 3.1 years, range 9 months-10 years (two with incessant ventricular tachycardia, one with ectopic atrial tachycardia, two with atrioventricular re-entrant tachycardia (three episodes), four with postoperative or congenital junctional ectopic tachycardia, two with bradycardia-tachycardia syndrome, one with atrial tachycardia) were treated with intravenous amiodarone during 13 tachycardia episodes. Left ventricular systolic functions were depressed in six patients. In 11 patients, a median of two drugs (range one-four), including adenosine infusion and in five cases direct current cardioversion were tried without success prior to intravenous amiodarone. The loading dose of amiodarone was 5 mg/kg in all episodes, infused over 1 h. Maintenance infusion was required in 12 episodes. In 10 episodes (77%), amiodarone was considered effective, in one (7.6%) partially effective (junctional ectopic tachycardia) and in two (15.4%) ineffective (sick sinus syndrome, atrial tachycardia). Therapeutic effect was obtained in a median period of 30 h (range 1-103 h). The mean effective maintenance dose was 10 +/- 4.7 micrograms/kg per min (range 5-15 micrograms/kg per min). In one patient, mild hypotension, and in three patients cellulitis occurred, but none of them necessitated termination of treatment. CONCLUSIONS: Intravenous amiodarone is found to be an effective and safe antiarrhythmic agent for children with acute life-threatening and chronic tachyarrhythmias and depressed left ventricular systolic functions.
Rate adaptive pacemakers are used to achieve a better cardiac performance during exercise by increasing the heart rate and cardiac output. The ideal rate adaptive sensor should be able to mimic sinus node modulation under various degrees of exercise and other metabolic needs. Minute ventilation sensing has proven to be one of the most accurate sensor systems. In this study, alterations in sinus rhythm and pacing rates during daily life conditions in 11 children (median age 11 years, range 6-14 years) with minute ventilation single chamber pacemakers were investigated. Correlation of sinus rhythm with pacing rates was assessed. ECG records were obtained from 24-hour Holter monitoring. Average rates of five consecutive P waves and pace waves were determined every half hour. The average of the two values was then used to determine hourly rates. Correlation coefficients between the sinus rhythm and pacing rates were calculated. In nine patients, pacing rates correlated well to sinus rhythm (range 0.6793-0.9558, P < 0.001 and P < 0.05), whereas in two cases correlation was not sufficient (P > 0.05). Most of the patients, in whom rate response factor (RRF) measurements during peak exercise by treadmill with chronotropic assessment exercise protocol were performed and pacemakers were programmed to these parameters, had more appropriate ventricular rates compared to spontaneous sinus rates. In these patients mean RRF value was 15.3 +/- 2.7 (range 12-20, median 15). This study shows that during daily activities minute ventilation rate adaptive pacemakers can achieve pacing rates well correlated to sinus rhythm that reflects the physiological heart rate in children.
Aortic root abscess, aneurysm of sinus Valsalva, severe aortic valve insufficiency, and a fragile aortic wall caused by infective endocarditis were found in a 10-year-old child. Aortic valve replacement was intended as a preoperative strategy, but one of modifications of Cabrol had to be used urgently because of aortic root rupture between right atrial and aortic connection just after pericardiotomy.
Thirteen carnitine-deficient children (mean age, 16.1 +/- 2.56 years) on a three-times-weekly hemodialysis program for at least 1 year, and 11 healthy age matched children were involved in the study. All the patients had stable blood pressure and hemoglobin (Hb) levels with a maintenance dose of erythropoetin and none were digitalized. The total carnitine (TC) and free carnitine (FC) plasma levels were sampled prior to hemodialysis (HD) before and after 3 months of carnitine supplementation. A free carnitine (FC) to acylcarnitine (AC) ratio less than 4 was defined as carnitine deficiency. Intravenous L-carnitine was injected at a dose of 20-40 mg/kg three times weekly at the end of each dialysis session for a 3-month period. Echocardiographic examination was performed the day following HD, before and after carnitine treatment. Systolic and diastolic functions of the left ventricle, including the ejection fraction, were measured. Almost all the parameters were significantly different in controls and hemodialyzed patients. In carnitine-deficient hemodialyzed patients, 3 months of L-carnitine supplementation resulted in a significant increase in blood carnitine levels and the FC/AC ratio, but this was not associated with any significant improvement of cardiac function. Furthermore no significant changes were observed in plasma triglycerides, total cholesterol or other lipoprotein parameters before or after carnitine supplementation. Although there was a moderate increase in mean hematocrit (Hct) and Hb levels, these also did not reach statistically significant levels. These results suggest that the 3 months of carnitine supplementation is not sufficient to ameliorate cardiac function or increase Hb levels in children.
In this study, the first study performed in pediatric patients, we assessed the safety and efficacy of a steroid-eluting active fixation ventricular electrode in 18 children. Our study shows that steroid-eluting active fixation leads are safe and effective in children, and suggests that these leads with their easy implantation and low chronic threshold values may be considered as the first choice in this age group.
UNLABELLED: To assess the efficacy of a serotonin re-uptake inhibitor, sertraline hydrochloride, in preventing recurrent neurocardiogenic syncope, we studied 15 patients (10 female; mean age 12.9 +/- 2 years) with positive head-upright tilt test and resistant to standard pharmacotherapy, atenolol or disopyramide. The patients were given 50 mg oral sertraline hydrochloride daily for 6 weeks. Intolerance to the drug was seen in 3 patients and 2 had syncopal episodes during the therapy. A head-upright tilt table test was then repeated in 10 patients. Six were tilt negative and asymptomatic over a mean follow up period of 7 +/- 3 months while four remained tilt positive: two experienced marked hypotension and bradycardia, characterized as mixed type syncope, and two had cardiac asystole, lasting > 10 s, during tilting, thereby exhibiting a cardio-inhibitory response. CONCLUSION: Sertraline hydrochloride may be useful in preventing recurrent neurocardiogenic syncope resistant to standard pharmacotherapy but careful clinical studies are essential before such a treatment strategy can be recommended since serious asystole could develop.
OBJECTIVE: To determine the efficacy of transcatheter closure of patent arterial ducts using controlled-release coils. DESIGN: Transcatheter closure of patent arterial ducts was attempted in 52 patients using controlled-release coils. SETTING: For the study, four tertiary paediatric cardiology units were used, two of which were in the U.K., one in Italy and one in Turkey. PATIENTS: The 52 patients ranged in age between 3-5 months and 61 years (median 3-5 years), and weighted between 4.5 kg and 62 kg. The duct diameters were 1 mm to 6.5 mm. RESULTS: In four patients the ducts were too large for safe release of the coils. In the remaining 48, one coil was inserted in 33 patients, two coils in nine, three coils in four and four coils in two patients. Immediately at the end of the procedure, the duct was completely occluded in 26/47 (55%) patients. Haemolysis occurred in one patient, in whom the coil was removed by a snare catheter and a large umbrella device was implanted with resolution of the haemolysis. Coil embolization to the pulmonary artery occurred in five (10%) patients. All were easily retrieved and replaced by larger coils. At the latest follow-up by colour Doppler echocardiography, the duct was completely occluded in 44/47 (94%) patients. CONCLUSIONS: Transcatheter closure of patent arterial ducts by controlled-release coils is effective and safe. Even when more than one coil is inserted the technique is still less cumbersome and considerably cheaper than transcatheter umbrella closure.
Tachyarrhythmias are common rhythm disturbances in infants and children. Despite the availability of diagnostic criteria arrhythmias are sometimes commonly misdiagnosed. Recent reports suggest that an endogenous purine nucleoside, adenosine, has a diagnostic effect in narrow QRS complex tachycardias, in addition to terminating supraventricular tachycardia involving the atrioventricular node. This report reviews the authors' experience with the use of adenosine for diagnosis of narrow and wide complex tachyarrhythmias in children. Adenosine was administered to 43 patients with several types of tachyarrhythmias (mean age, 8.3 +/- 5.24 years). Nineteen patients had structural or acquired heart disease. Of the 43 patients there were 28 (65%) several different types of narrow QRS complex tachycardia and 14 (33%) ventricular arrhythmias. One patient (2%) had long QT. Adenosine terminated supraventricular tachycardia, in 11 of 12 patients (92%), ventricular tachycardia in five of eight patients (63%), and transiently terminated premature ventricular contractions in two of six patients (33%). The diagnostic ability of adenosine was perfect in eight supraventricular tachycardia. In these eight cases the tachycardia mechanism was unclear before the administration of adenosine, which demonstrated three cases of sinus tachycardia, three of atrial flutter, one of atrial fibrillation and one of atrial fibrilloflutter. Confirmation of the primary diagnosis by adenosine was perfect in five tachyarrhythmias including three cases of atrial flutter, one of atrial fibrillation and one of ectopic atrial tachycardia. The average effective dose of adenosine was 212 micrograms/kg (range, 100-400 micrograms/kg). There were no serious side-effects except transient dyspnea, chest pain and flushing. These findings demonstrate adenosine to be helpful and safe in the diagnosis of tachyarrhythmias.
Head-up tilt testing with or without isoproterenol is extensively used in the evaluation of patients with unexplained syncope. However, sensitivity and specificity of tilt protocols with and without isoproterenol have not been clarified in children, due to lack of age matched control subjects. This study was designed to assess and to compare the sensitivity and specificity of tilting alone and tilting in conjunction with isoproterenol. Thirty children with unexplained syncope (group I) and 15 age-matched control subjects (control group I) underwent successive 60 degrees head-up tilts for 10 minutes during infusions of 0.02, 0.04, and 0.06 microgram/kg/min of isoproterenol, after a baseline tilt to 60 degrees for 25 minutes. Also, 35 children (group II) with unexplained syncope and 15 healthy control subjects (control group II) were evaluated by head-up tilt to 60 degrees for 45 minutes without an infusion of isoproterenol. In response to tilt protocol with graded isoproterenol, 23 (76.6%) of the patients in group I and 2 of the 15 (13.3%) control subjects developed syncope. Accordingly, the sensitivity of tilt testing with isoproterenol was 76.6%, and its specificity was 86.7%. Tilt testing without isoproterenol was positive in 17 (48.5%) of the patients in group II but in only 1 of the 15 (6.6%) control subjects. Thus, sensitivity and specificity of tilt testing without isoproterenol were 48.5% and 93.4%, respectively. The mean heart rate and systolic blood pressure decreased significantly (P < 0.001) in all tilt positive patients during syncope. In conclusion, the head-up tilt test is a valuable diagnostic test in the evaluation of children with unexplained syncope, and isoproterenol is likely to increase the sensitivity of the test without decreasing its specificity.
UNLABELLED: Dual sensor pacemakers were developed to obtain more appropriate responses to activity. We evaluated ten children with dual sensor pacemakers in different sensor blending circumstances using exercise testing to assess which ratio was optimal. Ten patients with several bradydysrhythmias (ages 6-16 years; mean 10.1 years) were included in the study. Eight patients had VVIR pacemakers (Vitatron Topaz), models and two patients had VDD pacemakers implanted via the transvenous route. All patients were in a paced rhythm (98.5% pacing). Accurate T wave sensing ranged from 81%-100%; mean 92%, median 95%. Voluntary exercise testing with a CAEP protocol was performed using a treadmill with the pacemaker in VVIR mode. Medium activity threshold with three sensor blending ratios (QT = ACT, QT > ACT, and QT < ACT) were done in all patients. The mean duration of exercise was not statistically different among the three sensor blending ratios. After 90 seconds of exercise, the mean pacing rate had increased by 12%, 3%, and 5%, respectively, in the three groups. At maximal exercise, the increases were 45%, 42%, and 54%. Mean HRs during exercise in each of the three ratios were not significantly different, although we found a statistically significant increase in HR during the first two stages of rest period in the QT = ACT sensor blending ratio compared to the QT > ACT ratio. No difference was observed after the second stage. IN CONCLUSION: (1) there is no difference between the QT = ACT, QT < ACT, and QT > ACT sensor blending ratios; and (2) each child has to be evaluated by exercise testing to program a correct sensor blending ratio.
The efficacy of a new single-chamber, rate-responsive pacemaker that utilizes information from two sensors, activity and stimulus to T wave, was evaluated in 15 children during a mean follow-up period of 10.3 +/- 3.3 months (range 5-16 months). There were 10 males and five females, with a mean age of 5.9 +/- 3.8 years (range 9 months-16 years). The indication for pacing was high grade atrioventricular block in 10 (eight postoperative, two congenital), and sinus node dysfunction in five patients. In endocardial implants the mean T wave amplitude was 2.48 +/- 0.7 mV, and mean T wave sensing 91 +/- 6.3%, whereas in epicardial implants T wave amplitude and sensing were inadequate. Each patient underwent 24-h Holter monitoring and 10 performed a graded treadmill test in three sensor-blending modes (Stimulus-T = Activity, Stimulus-T > Activity, Stimulus-T < Activity), using the chronotropic assessment exercise protocol. Sensor cross-checking was analysed by continuous tapping over the pacemaker. Holter monitoring demonstrated that pacing rate variations were closely related to daily activity. At the initial phases of exercise testing, the mean percentage of increase in pacing rate was significantly lower in Stimulus-T > Activity mode, when compared to Stimulus-T = Activity (P < 0.01); however, the initial disparity among the three modes disappeared halfway through the exercise and similar heart rate changes were observed thereafter. Continuous tapping over the pacemaker in Stimulus-T = Activity mode caused an initial increase in pacing rate, and inappropriate responses were quickly corrected by sensor cross-checking. Rate modulation with a single-chamber, dual-sensor pacemaker is adequate and safe in children, and may offer significant advantages over single-sensor devices in endocardial implants.
Controlled-release coils have become available recently for the closure of patent ductus arteriosus (PDA). Transcatheter closure of patent arterial ducts was attempted in 13 patients, ranging in age from 5 months to 15 years, mean 4.1 years. Implantation of controlled-release PDA coils was attempted via the femoral artery through 5 Fr catheters in all cases except one, in whom both the femoral arterial and venous routes were used. The procedure was successful in 10 of the 13 patients. In these, the pulmonary artery systolic pressure ranged between 25 and 42 mmHg and the duct diameter varied from 1.5 to 6 mm at its narrowest point. Six of the patients received a single coil. Two coils were inserted in three patients and three coils in one patient. In three patients the ducts were too large for safe release of the coils, despite attempted implantation of up to three coils simultaneously. These coils were easily withdrawn into the catheter. Immediately at the end of the procedure, the duct was completely occluded in nine of the 10 patients, and in one patient there was a small residual flow. The procedure time varied between 35 min and 2.5 h, mean 81 min and the fluoroscopy time varied from 5 to 78 min, mean 25 min. None of the patients experienced hemorrhage, diminished lower extremity pulse, hemolysis or infection. In one patient, a 5 mm coil embolized into the right pulmonary artery soon after release. It was retrieved with a snare, then 8 mm and a 5 mm coil were implanted satisfactorily in the arterial duct. At follow-up by color Doppler echocardiography, the duct was completely occluded in all patients. Transcatheter closure of patent arterial ducts by controlled-release PDA coils is effective and safe. Even when more than one coil is inserted, it is still cheaper than transcatheter umbrella closure. This method is therefore of great value, particularly in less affluent countries.
The disease of pulmonary vascular sling is a rare congenital condition. When it does occur it is often in conjunction with tracheal compression. The authors report herein a newborn with this anomaly presenting with a tracheal and esophageal compression.
The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.
Cardiac hydatid cyst is extremely rare in children. We report a case of a cardiac echinococcal cyst in an 11 year old boy, diagnosed by two-dimensional echocardiography and magnetic resonance imaging (MRI). The cyst was located in the posterior wall of the left ventricle and was excised surgically. Echocardiographic and MRI findings are discussed.
Delta wave and QRS complex polarities have been extensively studied in preexcitation syndromes. However, only limited data exist about ventricular depolarization and repolarization in the setting of maximal preexcitation in relation to the site of insertion of the accessory pathway. Therefore this study was designed to systematically analyze cardiac depolarization and repolarization in patients with maximal preexcitation. We analyzed the polarity of the QRS complex and T wave on the frontal plane on the conventional 12-lead electrocardiogram in 118 patients with maximal preexcitation. Fast atrial pacing was used to provoke maximal ventricular preexcitation. The 32 patients with a left lateral accessory pathway showed right-axis deviation of the QRS complex (110 +/- 20 degrees) with a left-axis deviation of the T-wave axis (-40 +/- 25 degrees). The 54 patients with a posteroseptal accessory pathway had a left axis of the QRS complex (-50 +/- 20 degrees) with a right-axis deviation of the T-wave axis (95 +/- 15 degrees). The 11 patients with a right lateral accessory pathway had a left axis of the QRS complex (-40 +/- 20 degrees) and a right axis of the T wave (110 +/- 10 degrees). In 7 patients with a left anterolateral accessory pathway and 14 patients with a right anteroseptal accessory pathway, the axis of the QRS complex was 50 +/- 25 degrees and 45 +/- 20 degrees, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
A signal averaged ECG is a useful tool for detecting low-amplitude, high frequency electrical potentials in the terminal portion of QRS. There is a close association between abnormal signal averaged ECG and coronary heart disease. In this study we evaluated late potentials and total QRS duration determined by this technique in diabetic children with and without good glycemic control. The signal averaged ECG of 20 children with diabetes mellitus and nine healthy age-matched controls were compared. The total QRS duration was longer in diabetic children than in controls (104.6, and 97.9 ms vs. 82.7 ms, P < 0.0005). Late potentials were not observed in the patient and control groups. The RMS voltage of the QRS complex in the last 40 ms was significantly lower in the diabetic group compared to the control group. There was no significant difference in the signal averaged ECG indexes between the two diabetic groups determined by the glycemic control state. These findings suggested that the children with diabetes mellitus frequently have intraventricular conduction disturbance regardless of glycemic control condition.