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Pediatric Cardiology 04/2012; 28(5):412-413. · 1.30 Impact Factor
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ABSTRACT: A prenatally sonographically diagnosed conotruncal anomaly with mosaic type trisomy 21 and 22q11.2 microdeletion/DiGeorge syndrome: We report a prenatally sonographically diagnosed conotruncal and urogenital anomaly. Postnatally, the patient presented with seizures, hypocalcemia, hypoparathyroidism and thymic aplasia and diagnosed as DiGeorge syndrome. Echocardiography showed malalignment VSD, supravalvular pulmonary stenosis and overriding aorta. Chromosome and FISH studies showed the association of mosaic type trisomy 21 and 22q11.2 microdeletion. The present patient is the second case of mosaic type of Down syndrome associated with 22q11.2 microdeletion. In addition the patient also had clinical and laboratory features of DiGeorge syndrome.
Genetic counseling (Geneva, Switzerland) 01/2009; 20(4):373-7. · 0.50 Impact Factor
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Y Alanay,
F Unal,
G Turanli,
M Alikaşifoğlu, D Alehan,
U Akyol,
E Belgin,
C Sener,
D Aktaş,
K Boduroğlu,
E Utine,
B Volkan-Salanci,
S Ozusta,
A Genç,
F Başar,
S Sevinç,
E Tunçbilek
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ABSTRACT: Fragile X syndrome (FXS) is the most common inherited form of intellectual disability. Since the identification of the responsible gene (FMR1) and its protein (FMRP), there has been enormous progress in both clinical and pathogenetic research on the neurobehavioural aspects of the condition. However, studies regarding other medical problems anticipated in individuals with FXS are limited. A multidisciplinary study evaluating various causes of morbidity in the same group has not been published yet.
Twenty-four boys with FXS full mutation were recruited out of a larger group of 103 diagnosed in one centre over the past 10 years. Ear nose and throat, eye and cardiac examinations were performed in addition to routine cognitive, behavioural, neurological and speech and language assessments.
The average IQ score was 49.8 +/- 20 (range 25-90). There were four patients (18%) with IQ above 70. Using DSM-IV, attention deficit hyperactivity disorder was diagnosed in five boys out of 22 examined (23%), while 32% were diagnosed with pervasive developmental disorder. The seizure frequency was 17%. A psychiatric disorder was diagnosed in six out of eight boys with electroencephalogram abnormalities (75%). Minimal conductive hearing loss was found in five (5/22) patients. There was significant delay in both expressive and receptive language skills. Ocular findings were refractive errors (13%) and strabismus (4.4%). Mitral valve prolapsus (MVP) was observed in 3/22 (13.7%) patients and aortic annulus dilatation was present in 2/22 (9%) patients.
Frequency of psychiatric diagnoses made with DSM-IV were in parallel to those reported in the literature. Comorbidity of seizures and psychiatric disorders was noteworthy. The percentage of 'high-functioning' full mutation males supports the previous observations. Ear nose and throat and eye examination revealed remarkably lower prevalence of abnormal findings than reported. MVP was slightly less frequent compared with the single study in the literature. Age at the time of examination had an effect on the outcome of cardiac evaluation. These findings will guide us in future management of the group of patients followed in our institution. The protocol applied provides an applicable outline for multidisciplinary institutional settings dealing with individuals with FXS.
Journal of Intellectual Disability Research 03/2007; 51(Pt 2):151-61. · 1.88 Impact Factor
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Y. Alanay,
F. Ünal,
G. Turanlı,
M. Alikaşifoğlu, D. Alehan,
U. Akyol,
E. Belgin,
C. Şener,
D. Aktaş,
K. Boduroğlu,
E. Utine,
B. Volkan-Salancı,
Ş. Özusta,
A. Genç,
F. Başar,
Ş. Sevinç,
E. Tunçbilek
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ABSTRACT: Background Fragile X syndrome (FXS) is the most common inherited form of intellectual disability. Since the identification of the responsible gene (FMR1) and its protein (FMRP), there has been enormous progress in both clinical and pathogenetic research on the neurobehavioural aspects of the condition. However, studies regarding other medical problems anticipated in individuals with FXS are limited. A multidisciplinary study evaluating various causes of morbidity in the same group has not been published yet.Methods Twenty-four boys with FXS full mutation were recruited out of a larger group of 103 diagnosed in one centre over the past 10 years. Ear nose and throat, eye and cardiac examinations were performed in addition to routine cognitive, behavioural, neurological and speech and language assessments.Results The average IQ score was 49.8 ± 20 (range 25–90). There were four patients (18%) with IQ above 70. Using DSM-IV, attention deficit hyperactivity disorder was diagnosed in five boys out of 22 examined (23%), while 32% were diagnosed with pervasive developmental disorder. The seizure frequency was 17%. A psychiatric disorder was diagnosed in six out of eight boys with electroencephalogram abnormalities (75%). Minimal conductive hearing loss was found in five (5/22) patients. There was significant delay in both expressive and receptive language skills. Ocular findings were refractive errors (13%) and strabismus (4.4%). Mitral valve prolapsus (MVP) was observed in 3/22 (13.7%) patients and aortic annulus dilatation was present in 2/22 (9%) patients.Conclusions Frequency of psychiatric diagnoses made with DSM-IV were in parallel to those reported in the literature. Comorbidity of seizures and psychiatric disorders was noteworthy. The percentage of ‘high-functioning’ full mutation males supports the previous observations. Ear nose and throat and eye examination revealed remarkably lower prevalence of abnormal findings than reported. MVP was slightly less frequent compared with the single study in the literature. Age at the time of examination had an effect on the outcome of cardiac evaluation. These findings will guide us in future management of the group of patients followed in our institution. The protocol applied provides an applicable outline for multidisciplinary institutional settings dealing with individuals with FXS.
Journal of Intellectual Disability Research 01/2007; 51(2):151 - 161. · 1.88 Impact Factor
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Pediatric Cardiology 01/2007; 28(5):418-419. · 1.30 Impact Factor
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ABSTRACT: Although active fixation ventricular leads seem to have advantages over passive fixation leads, this study compares the follow-up results of active and passive fixation leads in children. We evaluated the implantation and follow-up data of 41 children with active (Accufix II DEC, group 1) (n = 20) or passive (Membrane E, group 2) (n = 21) fixation, steroid-eluting ventricular leads. All but one of the patients in group 1 completed the 12-month follow-up. The mean follow-up period in group 2 was 10.4 +/- 2.9 months (range 3-12 months, median 12 months). In both groups the mean pacing threshold was measured as 0.51 +/- 0.09 V versus 0.48 +/- 0.15 V (P > 0.05) at 0.5-ms pulse width, mean R wave amplitude as 9.9 +/- 2.5 mV versus 9.4 +/- 3.2 mV (P > 0.05), and mean impedance as 557 +/- 92 omega versus 664 +/- 160 omega (P < 0.05), respectively, at implantation. After the first week of pacing, mean threshold values in group 1 were significantly lower than those of group 2 (P < 0.01 and P < 0.05, respectively). During the follow-up period, lead impedance measurements did not show a significant difference between the two groups. In one patient from group 1, the lead (by unscrewing) was removed easily because of pacemaker pocket infection. No lead dislodgement or helix deformation occurred in group 1. Nevertheless, in one patient from group 2, the lead was extracted at 4-month postimplantation because of lead displacement. We conclude that the steroid-eluting active fixation lead (Accufix II DEC) have advantages of easier implantation and lower acute and chronic stimulation thresholds compared to the passive fixation lead (Membrane E). Therefore, Accufix II DEC is superior to Membrane E, and it is a better first choice in children with an implanted single chamber ventricular pacemaker.
Pacing and Clinical Electrophysiology 08/2000; 23(8):1245-9. · 1.35 Impact Factor
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ABSTRACT: 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.
Pacing and Clinical Electrophysiology 12/1998; 21(11 Pt 1):2100-4. · 1.35 Impact Factor
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ABSTRACT: This prospective study was performed to assess the effects of 1 year of angiotensin-converting enzyme inhibition with captopril in 20 children (mean age 14.3+/-2.3 years) with asymptomatic chronic aortic regurgitation.
At 12 months patients receiving captopril had a significant reduction in left ventricular end-diastolic and end-systolic dimensions (57+/-9.3 vs 51+/-9.5 mm, p < 0.001; 35.4+/-6.1 vs 32+/-6.8 mm, p < 0.001), end-diastolic and end-systolic volume indexes (111+/-36 vs 94+/-29 ml/m2, p < 0.001; 35+/-13 vs 30+/-12 ml/m2, p < 0.001, respectively), and mass index (138+/-37 vs 109+/-32 gm/m2, p < 0.0001) determined by two-dimensional echocardiography. Meridian (p < 0.01) and circumferential (p < 0.0001) wall stresses also decreased significantly with therapy. Significant reduction (27.8%, p < 0.0001) was achieved in regurgitant fraction with captopril.
These data show that the long-term therapy with angiotensin-converting enzyme inhibitors is able to reverse left ventricular dilation and hypertrophy and suggest that such therapy has the potential to favorably influence the natural history of the disease in children.
American Heart Journal 05/1998; 135(4):598-603. · 4.65 Impact Factor
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ABSTRACT: 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.
Acta paediatrica Japonica; Overseas edition 11/1997; 39(5):570-7.
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ABSTRACT: 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.
European Journal of Pediatrics 10/1997; 156(10):747-50. · 1.88 Impact Factor
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ABSTRACT: Among our 20 families with LGMD2, 10 were documented to have muscle-specific calcium-activated neutral protease 3 (calpain-3) deficiency. Consanguinity was present in all. The current ages of the index cases were between 12 and 23 years, and there were additional nine members affected. Clinically, the patients showed mild courses; none of the cases below age 30 lost autonomy so far. The dystrophy is mainly proximal and atrophic with calf enlargement and scapular wasting in some. In three cases walking was delayed. Creatine kinase levels were at least 10 times elevated. All obligate carriers had normal creatine kinase levels. Five families shared the same 551 delA frameshift mutation. In four of these families there was the same core haplotype, whereas one was distinct suggesting an independent origin. Calpain-3 deficiency in general is a mild muscular dystrophy during childhood.
Neuropediatrics 09/1997; 28(4):212-6. · 0.94 Impact Factor
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ABSTRACT: 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.
The American Journal of Cardiology 09/1997; 80(3):355-8. · 3.37 Impact Factor
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ABSTRACT: 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.
Pacing and Clinical Electrophysiology 07/1997; 20(7):1769-76. · 1.35 Impact Factor
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ABSTRACT: To determine the efficacy of transcatheter closure of patent arterial ducts using controlled-release coils.
Transcatheter closure of patent arterial ducts was attempted in 52 patients using controlled-release coils.
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.
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.
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.
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.
European Heart Journal 04/1997; 18(3):450-4. · 10.48 Impact Factor
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ABSTRACT: To describe 11 patients with narrowing of the left ventricular outflow tract caused by angular posterior deviation of both the outlet septum and the upper part of trabecular septum, which was diagnosed by cross sectional echocardiography in all and confirmed by angiocardiography in seven.
Four patients had a subaortic systolic pressure gradient ranging from 23 to 70 mm Hg by Doppler echocardiography; cardiac catheterisation showed a significant (60 and 104 mm Hg) systolic pressure gradient in two. In four cases aortic regurgitation and two tricuspid pouches were shown by Doppler echocardiography, angiocardiography, or both. Four cases had a ridge at the angulation point on echocardiographic examination. Three patients were operated on for systolic pressure gradients of the left ventricular outflow tract and one for severe aortic regurgitation. There was proliferation of collagen-rich fibrous tissue in the subendocardial region on histopathological examination of the myectomy material. A ventricular septal defect had been diagnosed previously by contrast echocardiography in one patient; thus ventricular septal defects may close spontaneously over a period of time including fetal life. A subaortic ridge was detected in one patient at follow up.
Deviation of the outlet and trabecular septa should be considered as a cause of ventricular outflow tract obstruction even when no ventricular septal defect is present.
Heart (British Cardiac Society) 04/1997; 77(3):242-6. · 4.22 Impact Factor
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ABSTRACT: 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.
Acta paediatrica Japonica; Overseas edition 11/1996; 38(5):500-5.
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ABSTRACT: In this report we present two cases with unguarded tricuspid orifice, and review the literature. The complete absence of tricuspid valvular structures is a very rare anomaly coexisting with pulmonary atresia and intact ventricular septum, and is termed unguarded tricuspid orifice. Double tricuspid orifices and isolated dextrocardia were distinguishing features of our first case. The second patient of this report, a 5-year-old boy, is the first case in the literature with this malformation of the tricuspid valve as an isolated anomaly, in addition to being the first surviving case in the literature.
International Journal of Cardiology 11/1996; 56(2):125-9. · 7.08 Impact Factor
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ABSTRACT: 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.
European Heart Journal 09/1996; 17(8):1251-5. · 10.48 Impact Factor
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ABSTRACT: Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date.
In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5.7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation.
Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells.
The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges.
Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.
European Heart Journal 07/1996; 17(6):935-9. · 10.48 Impact Factor
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ABSTRACT: Unexplained syncope may cause diagnostic and therapeutic problems in children. The head-up tilt test has been shown to be
a useful tool for investigating unexplained syncope, especially for diagnosis of neurally mediated syncope. In this study
20 patients aged 9–18 years (12.0±2.5 years) with syncope of unknown origin and 10 healthy age-matched children were evaluated
by head-up tilt to 60° for 25 minutes. The test was considered positive if syncope or presyncope developed in association
with hypotension, bradycardia, or both. If tilting alone did not induce symptoms (syncope or presyncope), isoproterenol infusion
was administered with increasing doses (0.02–0.08 μg/kg per minute). During the tilt test, symptoms were elicited in 15 (75%)
of the patients with unexplained syncope but in only one (10%) of the control group (p<0.001). The sensitivity of the test was 75% and its specificity 90%. Three patterns of response to upright tilt were observed
in symptomatic patients: vasodepressor pattern with an abrupt fall in blood pressure in 67%; cardioinhibitory pattern with
profound bradycardia in 6%; and mixed pattern in 27%. In patients with positive head-up tilt, there were sudden decreases
in systolic blood pressure (from 130±15 to 61±33 mmHg) and in mean heart rate (from 147±26 to 90±38 beats per minute) (p<0.001) during symptoms. Treatments with atenolol 25 mg/day has shown complete suppression of syncope in positive responders
during a mean follow-up period of 18±6 months. The head-up tilt test is a noninvasive, sensitive, specific diagnostic tool
for evaluating children with unexplained syncope.
Pediatric Cardiology 04/1996; 17(2):86-90. · 1.30 Impact Factor
