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Tetralogy of Fallot (ToF) occurs in about 4 births/1000/year and represents about one tenth of all congenital heart diseases. Nowadays 86% of patients reach adulthood with corrective surgery. Before the 1980s, these patients were treated only with "surgical palliation", which consisted in the creation of a systemic to pulmonary artery shunt or a pulmonary valvulotomy, whereas after the introduction of extracorporeal circulation, corrective surgery is performed electively between 3 and 6 months of life. After repair patients during their life may develop hemodynamic lesions, including right ventricular outflow tract dysfunction, and arrhythmias which can occur in over 30% of cases. It is estimated that these patients present a risk of sudden death of 0.2%/year. Therefore, for the prevention and treatment of arrhythmic events, a periodic follow-up in specialized centres for adult congenital heart disease is mandatory, because most often arrhythmias are triggered by the presence of hemodynamic lesions, first of all pulmonary regurgitation.

At present, syncope is still a matter of great concern and anxiety for both parents and children. Neurocardiogenic syncope is the most common type of syncope in children with an incidence of 70%. It usually has a benign etiology, with rapid loss of consciousness and spontaneous resolution. In case of neurocardiogenic syncope, it is important to assess the emotional and/or adaptive capacity of the patient. From a psychological point of view, the presence of a traumatic area of development has been reported in pediatric patients with syncope. This area is characterized by symptoms of anxiety and/or depression, self-withdrawal, somatic complaints and dissociation. Neurocardiogenic syncope in pediatric age can be the expression of a psychosomatic disorder underlying psychopathological vulnerability.

A new transcatheter ablation method has recently made its appearance in leading electrophysiology laboratories: cryoablation. In contrast with radiofrequency, this method produces a permanent lesion due to cell necrosis, caused by application of very low temperature chilling to the tip of special ablation catheters placed against the area of the heart causing arrhythmia. The benefit of this system over radiofrequency ablation is its ability to find the most suitable site for ablation through transitory electrical paralysis of the heart tissue in contact with the catheter tip, chilled to -30 degrees C (cryomapping). If the site is suitable, the tissue causing the arrhythmia loses its excitability. Moreover, the stability of the catheter tip, which adheres to the myocardium, enables arrhythmia inducibility through programmed atrial stimulation to be assessed without dislodging the catheter. A permanent lesion is created only subsequently, with further chilling to even lower temperatures (cryoablation). In the last 3 years the success rate of cryoablation in pediatric patients has increased and long-term complications have not been described yet. We therefore consider that cryoablation should be considered the treatment of choice for atrioventricular nodal reentrant tachycardia and parahissian, anteroseptal and right midseptal accessory pathway atrioventricular reentrant tachycardia in school-age children. In fact cryoablation is thus "made to measure" for pediatric patients because if an atrioventricular block is observed, stopping the procedure is quickly followed by the restoration of normal atrioventricular conduction.

Ventricular pacing, typically initiated from a right ventricular apical electrode, inherently causes abnormal biventricular activation, decreases left ventricular function, and may cause histopathological changes. Data regarding cardiac resynchronization therapy in pediatric patients are limited. Some authors evaluated the potential of improving paced left ventricular function by a septal electrode implant site. For this reason the role of pacing is not clear in children. In isolated congenital atrioventricular block, cardiac pacing represents a satisfactory treatment. However, recent evidence has demonstrated that a subset of patients with isolated congenital complete atrioventricular block develops dilated cardiomyopathy. Patients with congenital heart diseases after surgery represent a unique and expanding population. Some will require pacemaker or implantable defibrillator therapy. They pose technical and management challenges not encountered in other groups receiving pacing, and the complication and reintervention rates specific to this population are not well defined. Moreover, the small patient size, structural cardiac abnormalities, and growth may complicate pediatric pacemaker management. Better knowledge of risk factors for lead failure in these patients may help improve future outcomes. In this review we analyzed many of these problems.

Introduction: Ventricular tachycardia is the most common diagnosis among children with exercise or emotion-related syncope. The aim of the study was to assess the prognosis and the clinical course of children, without long QT syndrome, with exercise-related ventricular tachycardia and apparently normal heart., Methods: Since January 1984, we studied 14 children (mean age 9.7 +/- 3.8 years) with exercise-related ventricular tachycardia, and, at the initial detection, without demonstrable heart disease, as assessed by clinical evaluation, electrocardiogram and mono-bidimensional echocardiogram. Six patients had exercise related syncope, 1 exercise intolerance and 7 were asymptomatic. All patients underwent exercise testing on treadmill and 24-hour ECG monitoring. All patients were treated with antiarrhythmic drugs. All patients were tested by exercise testing and 24 hour ECG monitoring, alternatively, every six months, during therapy or after complete wash-out of the drug used., Results: Ventricular tachycardia, detected by exercise testing and Holter monitoring, was polymorphic in 4 symptomatic patients, monomorphic with left bundle branch block morphology and inferior axis in the others. The mean rate of monomorphic ventricular tachycardia was 224 +/- 35 bpm in the symptomatic children and 180 +/- 33 bpm in the others (p = 0.03). Successful chronic treatment was achieved with beta-blockers in 5 cases, propafenone in 6, sotalol in 2 and flecainide plus metoprolol in 1. During follow-up (6 +/- 2.7 years), 1 patient with polymorphic ventricular tachycardia, on therapy, suddenly died during exercise, the other symptomatic patients had no variation of their arrhythmia on routine tests, except 1 who, after propafenone was stopped, had ventricular fibrillation during exercise testing. Among the 7 asymptomatic patients, 5 had no evidence of ventricular tachycardia on routine test after 2 +/- 3 years; the others had no variation of their treatment., Conclusion: Exercise related ventricular tachycardia in children seems: 1) to have more severe prognosis if symptomatic or polymorphic; 2) mostly to arise from right ventricle; 3) to be successfully treated by antiarrhythmic drugs with beta-blocking activity.

Background: The aim of this study was to assess the prognosis and the clinical course of incessant idiopathic ventricular tachycardia (VT) with left bundle branch morphology, in a group of 14 children consecutively evaluated in our institutions between 1983 and 1994., Methods: Mean age of these patients was 8 +/- 2 years, and they were followed for 56 +/- 32 months. In all the children, VT was present for more than 80% of the day, in non sustained runs. The mean rate of VT was of 142 +/- 30 beats/min. Four children were symptomatic. The evaluation of these children was obtained by treadmill stress test (12/14), cardiac catheterization (13/14), electrophysiologic study (10/14), nuclear magnetic resonance (7/14), and serial echocardiographic and Holter examinations., Results: Right ventricular dysplasia was suspected in 2 cases on the basis of nuclear magnetic resonance and angiographic data. In both these cases VT was not suppressed by exercise. Medical treatment was started in 9/14 patients (69%), it was completely effective in 4 (44%) and partially effective in 2 further cases (22%). Thirteen out of fourteen patients had a regular follow-up. No patient died. All patients remained asymptomatic and in 6 (46%) VT disappeared. In 4 of these last 6 patients therapy has been previously effective. Two children, one with right ventricle dysplasia, continue antiarrhythmic treatment., Conclusions: Incessant VT is well tolerated in pediatric age. The frequency of spontaneous remissions is relatively high (46% of cases). In few patients this arrhythmia is associated with evident cardiac abnormalities. In our opinion, medical treatment is necessary in symptomatic patients and in those with heart abnormalities.

Objectives: The purpose of this study was to evaluate the efficacy and safety of radiofrequency ablation of supraventricular tachycardias due to a reentry circuit in a group of closely followed-up pediatric patients., Patients: Forty-six pediatric patients (mean age 12.6 years, range 3-18) underwent a total of 48 radiofrequency ablation procedures for reciprocating supraventricular tachycardia. Thirty-nine had an orthodromic reentry tachycardia, 6 an atrioventricular node reentry tachycardia and 1 a permanent junctional reciprocating tachycardia., Methods: To ablate the overt accessory pathways, the Kent potential (K) was identified and then the shortest A-K, and delta wave-K intervals were measured. The shortest V-A interval was identified for those concealed. For the left-sided accessory pathways, we used the retrograde transaortic approach in 10 procedures and the transseptal approach in 18. Atrioventricular node reentry tachycardia was ablated at the site where the slow-pathway electrogram was recorded. Follow-up clinical data, electrocardiogram, 24-hours Holter monitoring and transesophageal atrial stimulation were obtained and evaluated., Results: Early: We performed a total of 48 procedures. Among 41 procedures performed in the 39 patients with accessory pathway, 38 were successful (92,6%). Mean fluoroscopy time was 31 +/- 18 min. For left sided accessory pathways, mean fluoroscopy time of procedures with retrograde approach was 45 +/- 10 min and with transseptal 23 +/- 12 min (p < 0.01). Ablation of slow-pathway in patients with intranodal reentry tachycardia was successful in 3/6 cases (50%). The patient with permanent junctional reciprocating tachycardia was successfully ablated. Late: All patients are alive and none was lost during the follow-up after a mean time of 12.9 months (range 5-33). Success at last follow-up was 100% in patients with an accessory pathway and 33% in patients with atrioventricular node reentry. In patient with permanent junctional reciprocating tachycardia, the arrhythmia appeared again one month after the procedure, thus a second successful attempt was performed., Complications: Major complications (6,5%) including a right femoral vein thrombosis (one patient), an hematoma without pulse loss (one patient) and a non-sustained monomorphic ventricular tachycardia (one patient)., Conclusions: Our data show high efficacy of radiofrequency ablation in pediatric patients with accessory pathways. The risks are low at follow-up evaluation, but might be helpful a longer-term follow-up in order to evaluate the risk of a long fluoroscopy time and the arrhythmogenic effect of the scar.

Introduction: The aim of the study was the evaluation of the usefulness of transesophageal atrial pacing in predicting chronic oral treatment efficacy of symptomatic reciprocating supraventricular tachycardia in infants and in avoiding the risk of very dangerous recurrences at home., Methods: We studied 13 infants (11 males, 2 females, mean age 43 +/- 31 days) with symptomatic reciprocating supraventricular tachycardia and no structural heart disease. All patients had chronic oral therapy, using the drug effective in acute i.v. somministration. Each patient was discharged when supraventricular tachycardia was not inducible with transesophageal atrial pacing after 5 half-lives of the drug used in chronic oral treatment. All patients, every 6 months, were retested with transesophageal atrial pacing alternatively during chronic oral therapy and after complete wash out. Oral therapy was stopped in each patient when supraventricular tachycardia was not inducible after the wash out., Results: The number of oral treatments tested for each patient were 2 +/- 1 (range 1-5). The number of transesophageal studies performed for each patient were 4 +/- 2 (range 3-7). No patient had symptomatic episodes of supraventricular tachycardia or needed to change therapy during the follow-up. The oral treatment was stopped after the twelfth month of life in 8 patients and after the twenty-fourth in 2 others without recurrences., Conclusion: Transesophageal atrial pacing seems to be useful in predicting accurately and rapidly the oral treatment efficacy of supraventricular tachycardia in infants. Our protocol seems to be effective to avoid dangerous recurrences of tachycardia and to decide when we can stop therapy without risk.

The aim of this paper is to define the problems that arise in the clinical evaluation of drugs for the treatment of vertigo. Among these are the objective criteria used in defining vertigo and those used in evaluating efficacy of the drugs. The resulting protocol for a clinical study of vestibular drugs is a document that clarifies the debated points in the field, and above all furnishes guidelines for establishing uniformity in clinical studies. This, therefore, may become the reference protocol in Italy for clinical evaluations of drugs for the treatment of vertigo.

The parents of 151 children operated on for congenital heart disease have answered some questions about the scholastic, extrascholastic and physical activity of their children. Approximately 94% of the children are at the correct school level for their age. Extrascholastic activities of the city children is the same as children living in the country. During their free time 22% of the child population engages in physical activity, whereas 78% of the child population engages in physical activity at school. Among the parents, 61% think the activity of their children is normal, and 27% think it is too active. Our results demonstrate that the children operated on for congenital heart disease have a normal life during scholastic and free time, whereas, the introduction of these children to sport activities is anomalous and insufficient.

The need for a more complete protocol for bicycle ergometer exercise testing in children in our country, induced the Authors to apply the F.W. James protocol to healthy Italian children and then compare the results with a study on a similar american sample population. A total of 102 children, 67 males and 35 females, age range 4.4 - 17 years (mean 10.3 yrs), were examined following the F.W. James protocol. The Authors report the anthropometric and ergometric data obtained, the comparison with James' results and the linear correlation coefficients. Normal reference data were obtained. There were no significant differences between the results obtained from the Italian and American groups, and the correlation coefficients were satisfactory. The results will enable us to benefit from a simple and complete protocol which can be applied to healthy and sick children alike in our country.

Before Mustard's operation 27 children, from 2 days to 11 months old, with simple or complex transposition of the great vessels, underwent an electrocardiogram at rest. After operation, all the children underwent a standard electrocardiogram and 24 hours continuous Holter monitoring. Successive electrocardiograms at rest were recorded in all patients, and eleven children were successfully examined by dynamic electrocardiogram. The electrocardiogram at rest showed a sinusal rhythm in 66.7 of the patients; during the follow-up, in 33.3% non sinusal rhythm was recorded. The continuous 24 hours dynamic electrocardiogram, immediately after surgery, revealed 25.9% of the patients with non-sinusal rhythm. After 7 months, the Holter monitoring showed, in one case, a severe sinus node disfunction. Our data and those of the literature confirm the importance to follow these patients during a long period of time, because it was found that, even bedately, can develop serious arrhythmias.

The aim of the study was to assess the diagnostic efficacy of ambulatory electrocardiographic monitoring in children with a previous history of fainting and/or syncope. Sixty-one children, from 2 months to 17 years of age, all at the time undergoing different physical examinations because of previous syncopal episodes were examined. All the children had a normal electrocardiogram at rest and all underwent 24 hour electrocardiographic monitoring (Holter). The ECG was normal in 48 patients. Eight children had several dysrhythmias, classified as "borderline" and five children showed a clearly pathological trend. These results are even more significant considering that standard electrocardiogram results are negative. These data underline the diagnostic importance of the dynamic electrocardiogram particularly in cases of very frequently repeated symptoms.

The diagnostic role of carcinoembryonic antigen (CEA) in ascitic fluid was evaluated in order to assess whether or not ascitic haemorrhage is related to cancer. A total of 30 patients with ascitic haemorrhage were analysed (17 had cirrhosis, 5 non-metastasised cancers and 8 metastasised cancers). CEA radioimmunoassay was performed in serum and ascitic fluid. The results showed that CEA in the ascitic fluid highlighted a clear distinction between the average values of the 3 groups examined serum assay did not. Results were respectively: cirrhotic patients 6.11, non-metastasised cancers 38 and metastasised cancers 204.12. Ascitic fluid measurement revealed considerable variation, especially between the first and third groups examined (p = .00000004). Although CEA must be regarded as an aspecific laboratory test for malignancy, these results suggest that CEA determination in the ascitic fluid may be useful for screening patients with ascitic haemorrhage.