Exclusion of HRAS from long QT locus.
Explore the source record for details and available documents.
Chromosome Mapping↗
Biomedical subjects
I Denjoy
Publications and source records attributed to I Denjoy.
[Long term course of catecholaminergic polymorphic ventricular tachycardia in children. Apropos of 20 cases with an 8 year-follow-up].
BACKGROUND: Primary ventricular arrhythmias are rarely seen in children. Some of them have a poor prognosis; they should be diagnosed because adequate treatment can prevent sudden death. POPULATION AND METHODS: Twenty children (11 male, nine female), aged 3 to 16 years (mean: 7.7 +/- 4), with apparently normal hearts and normal QTc intervals were referred for stress or emotion-induced syncope. Primary ventricular arrhythmia, consisting of isolated polymorphic ventricular extrasystoles followed by salvos eventually degenerating into ventricular fibrillation, was reproducibly induced by physical exertion. The syncopal events and "torsades de pointe" disappeared with beta-blocking therapy. A total of four syncopal events and two sudden deaths occurred during a mean follow-up of 8 years, probably due to discontinuation of treatment. DISCUSSION: Fifty-four-cases of stress-induced severe polymorphous ventricular arrhythmia have been reported in the literature. There were four sudden deaths in 37 patients on beta-blocking therapy, and ten sudden deaths in 21 untreated patients. CONCLUSION: Clinically close to the congenital long QT syndrome, this primary ventricular arrhythmia must be looked for in cases of stress or emotion-induced syncope. The diagnosis relies on Holter monitoring and a stress test. Life-long beta blocker therapy is required.
Adolescent↗
[Effect of atropine therapy on sudden infant death. A multicenter survey of 7851 children at risk].
The results of a multicentre inquiry started in 1988 in reference centres of sudden infant death are presented. This study concerns the sudden and unexplained mortality of infants under 1 year of age who were treated with atropinics for an alleged risk of sudden death. The 7,851 infants involved were divided into 2,605 siblings, 1,067 premature babies and 4,179 infants who experienced malaises. Only one of the 2,034 infants treated with atropinics (385 siblings, 435 prematures, 1,214 with malaise) died, as opposed to 27 deaths among the 5,817 infants who where not treated (10 deaths among 2,220 siblings, 6 among 632 premature and 11 among 2,965 infants with malaise); P = 0.005. These results are encouraging, but they suffer from the limitations and biases inherent in all large inquiries. They certainly do not allow us to conclude without reservation that vagal hyperreflectivity is the mechanism responsible for sudden infant death and that atropinics must be systematically given to all infants at risk. Wide and randomized prospective studies are highly desirable in this particular field.
Drug Evaluation↗
Flecainide acetate in the prevention of paroxysmal atrial fibrillation: a nine-month follow-up of more than 500 patients.
In order to assess efficacy, safety, and long-term tolerance of flecainide for the prevention of paroxysmal atrial fibrillation (PAF), 944 patients (555 male) were enrolled in an open multicenter study. All patients had had greater than or equal to 1 episodes of atrial fibrillation and were in sinus rhythm at the time of entry. The mean age was 65.3 +/- 11 years, and 43% of patients had no detectable heart disease. The mean daily dose of flecainide was 190 +/- 34 mg. Clinical examination, electrocardiogram (ECG) and 24-hour Holter monitoring were performed at entry into the study and on months 3, 6, and 9. Of the patients, 189 were lost to follow-up. Of the remaining 755 patients, 562 (74%) continued the treatment during the 9-month period and 193 (26%) dropped out. A total of 84 adverse effects were reported in 7.6% enrolled patients and in 9% of patients during follow-up, with treatment interruption in 50% of the cases. There were only 3 minor cardiovascular side effects, all leading to treatment discontinuation. No deaths in patients with recurrent PAF and no proarrhythmic events were reported. Flecainide appears to be effective in preventing PAF, with 65% of patients being arrhythmia-free after 9 months of treatment at a mean daily dose of 200 mg. Side effects were common, but clinically significant adverse events were infrequent.
Atrial Fibrillation↗
Flecainide in quinidine-resistant atrial fibrillation.
Flecainide (100 mg twice daily) was used for prevention of paroxysmal atrial fibrillation (PAF) in 52 patients with frequent symptomatic attacks that were resistant or intolerant to quinidine (600-900 mg/day). Underlying heart disease was present in only 8 cases and left ventricular ejection fraction was always greater than 30%. No patient had had a myocardial infarction. Vagally induced PAF was clinically documented in 35 patients. Amiodarone, previously used and ineffective, was combined with flecainide in 33 patients. After 1-5.8 years of follow-up, complete disappearance of PAF was observed in 38 patients (73%). The success rate was slightly higher in patients with vagally induced PAF (p = 0.07). Extracardiac side effects necessitated withdrawal in only 3 cases. Permanent pacemaker was needed in 7 patients on amiodarone and flecainide because of excessive sinus bradycardia. Two patients, with previously documented atrial flutter, experienced presyncopal episodes of atrial flutter with 1:1 atrioventricular (AV) conduction and wide QRS complex. No death occurred during the follow-up. In this series, quinidine proved to be unsuccessful in 46 patients and it was withdrawn in 6. We concluded that flecainide is efficient and well tolerated for long-term prevention of PAF in patients resistant to quinidine. The possibility of 1:1 AV conduction during atrial flutter may suggest the use of verapamil or beta blockers in combination with flecainide in patients with previously documented atrial flutter.
Amiodarone↗
[Value of signal-averaged electrocardiogram in ventricular arrhythmia without apparent heart disease].
Signal-averaged electrocardiography was performed in 132 subjects with various ventricular arrhythmias without clinically apparent heart disease and compared with the results obtained in a series of 45 normal subjects. The latter enabled definition of the following criteria of normality: QRS duration after averaging < 113 ms; Simson vector of the last 40 ms (RMS40) > or = 17 microV; low amplitude signal duration over 40 microV < 38 ms for a high pass bidirectional filter of 40-300 Hz. Complementary investigations (echo or angiography), performed in all cases, showed underlying abnormalities in 26 patients: 13 right ventricular dysplasias, 7 cardiomyopathies, 3 mitral valve prolapses and 3 minor congenital heart defects. These investigations were normal in the remaining 106 subjects. Of the 26 patients with cardiac disease, 15 had 3 criteria of positivity for late ventricular potentials, 6 had 2 criteria and the other 5 had no criteria of late ventricular potentials. Therefore, 81% of cases with cardiac disease had at least 2 diagnostic criteria of late ventricular potentials whereas only 4% of those without cardiac disease, and in the control group, had criteria of positivity (p < 0.001). Using these criteria, the predictive value of signal-averaged electrocardiography for the detection of underlying cardiac disease was good when 2 criteria are required for diagnosis of late ventricular potentials: sensitivity 81%, specificity 96%; predictive value of a positive test 78%, predictive value of a negative test 97%. Signal-averaged electrocardiography is therefore a good non-invasive method of diagnosing underlying cardiac disease in patients with ventricular arrhythmias without clinically apparent heart disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Long-term follow-up after sustained monomorphic ventricular tachycardia: Causes, pump failure, and empiric antiarrhythmic therapy that modify survival.
We analyzed the actuarial cardiac mortality rate of 295 consecutive patients with sustained monomorphic ventricular tachycardia who were referred to us between 1978 and 1988. Patients were divided into four groups: group I of 156 patients with coronary disease, group II of 55 patients with nonischemic left ventricular disease, group III of 65 patients with right ventricular disease, and group IV of 19 patients without detectable heart disease. Patients were treated empirically according to a prospective schema: (1) class I antiarrhythmic drugs, (2) in case of recurrence of ventricular tachycardia: amiodarone or beta-blockers, (3) in case of recurrence of ventricular tachycardia: drug combinations or surgery. The mean follow-up duration was 61 +/- 40 months after the first occurrence of ventricular tachycardia, and the clinical outcome was known in 67.5% of patients at 5 years. The actuarial mortality rates were considerably higher in groups I and II compared with those in groups III and IV (p less than 0.01). The mortality rate was slightly higher in group I than in group II (p less than 0.05). In groups I and II, actuarial mortality rate were much higher when left ventricular ejection fraction was less than 0.30 (p less than 0.01). Comparisons between treatments showed no difference in actuarial mortality rates in patients with ventricular tachycardia and left ventricular ejection fraction greater than 0.30.(ABSTRACT TRUNCATED AT 250 WORDS)
Actuarial Analysis↗
[Use of flecainide in the preventive treatment of tachycardia crisis of Wolff-Parkinson-White syndrome in 4 newborn infants].
Four neonates with Wolff-Parkinson-White syndrome developed supraventricular tachycardia and received flecainide orally after reduction of the arrhythmia. The initial doses were 5 mg/kg/day. They are increased according to clinical response and flecainide concentrations, with 5 and 10 mg/kg/24 h respectively, were in the therapeutic range of 200 to 1000 micrograms/l. Flecainide was effective and well tolerated in the 4 cases. Therefore, this drug may be an alternative to digoxine to prevent supraventricular tachycardias in the neonatal period.
Flecainide↗
[Life expectancy after a first attack of sustained monomorphic ventricular tachycardia. Apropos of 295 patients with a follow-up of more than 5 years].
Analysis of actuarial cardiac mortality of 295 patients after a first attack of sustained monomorphic ventricular tachycardia followed up for an average of 5.1 years showed that: the aetiology was the main prognostic factor. Patients with a normal left ventricle had a zero 5 year mortality rate (normal heart) or 3% (arrhythmogenic right ventricular dysplasia) compared with 24% in patients with cardiomyopathy and 40% with myocardial infarction: p less than 0.01. Similar results were obtained if sudden death was considered; in patients with left-ventricular disease, the role of pump function assessed by the ejection fraction was essential: the 5 year mortality was 14.5% (cardiomyopathy) and 30% (myocardial infarction) if this was greater than 0.3 compared to 43% and 51% respectively when less than 0.3 (p less than 0.01); the heart rate of spontaneous ventricular tachycardia and that of tachycardia induced during endocavitary investigation had no influence on the mortality. However, the mortality following a first tachycardia with syncope was higher than that following a well tolerated tachycardia (p less than 0.5 if the ejection fraction less than or equal to 0.3 and p less than 0.001 if greater than 0.3).(ABSTRACT TRUNCATED AT 250 WORDS)
Actuarial Analysis↗
[Our experience with flecainide acetate in resistant arrhythmias in children. Apropos of 35 cases].
Thirty-five patients aged 6 days to 18 years (average 7.5 +/- 5.2 years) were treated for an average period of 16 months (range 8 days to 50 months) with flecainide acetate at an average dose of 4.8 +/- 1.4 mg/kg (2.9 to 10 mg/kg) or 130 +/- 30.5 mg/m2 administered twice daily. The cardiac arrhythmia was a resistant paroxysmal junctional tachycardia due to a Wolff-Parkinson-White syndrome in 27 cases, intranodal reentry in 6 cases and a chronic reciprocating rhythm in 2 cases. Treatment was successful with complete suppression of the tachycardia in 24 cases. Partial success with a good clinical result was obtained in 4 cases and there were 7 failures, 6 due to inefficacy of the drug, and 1 because of an extracardiac secondary effect. One case of incessant junctional tachycardia was observed in a 9 month old child in whom the preexcitation disappeared. Atrioventricular preexcitation persisted in 20 out of 24 cases. The duration of the non-preexcited QRS complexes increased significantly from 73.6 +/- 13.8 to 82.2 +/- 15.2 ms; n = 14, p less than 0.01. The minimal effective plasma concentration was 347 +/- 147 ng/ml. The plasma concentration/dose ratio of children over 4 years of age was the same as in adults. It was significantly higher in babies and infants suggesting a progressive acquisition of the capacity to metabolise flecainide during the first year of life. In conclusion, flecainide acetate was easy to use with respect to administration and follow-up, and seems to be a drug of choice for the treatment of junctional tachycardia in children.
Adolescent↗
Flecainide acetate dose-concentration relationship in cardiac arrhythmias: influence of heart failure and amiodarone.
The trough concentration-dose (C/D) ratio of flecainide was prospectively studied in 78 patients with various cardiac arrhythmias. After the removal of two outlier values, no influence of body weight on C/D ratio was evidenced. Coadministration of amiodarone, and, moreover, the presence of heart failure increase the C/D ratio, from 2.01 +/- 0.78 to 2.55 +/- 0.37 and 2.9 +/- 1.19 ng/ml/mg, respectively (p less than 0.001 by two-factor analysis of variance). The presence of both heart failure and amiodarone therapy increases the C/D ratio to 3.88 +/- 1.07 ng/ml/mg. A single loading oral dose (30 mg/kg) of amiodarone increased C/D measured at the sixth hour in nine patients from 2.27 +/- 0.50 to 2.57 +/- 0.73 ng/ml/mg (p less than 0.05). The trough C/D ratio increased more during chronic treatment from 2.03 +/- 0.86 to 2.92 +/- 1.32 ng/ml/mg (p less than 0.05). Thus, a dosage reduction of flecainide (of 50% in some cases) is mandatory, in case of heart failure or the combination with amiodarone therapy, to obtain a plasma level of the drug that is similar to those observed in patients with a normal heart and without amiodarone therapy. The flecainide-amiodarone interaction seems time dependent.
Adult↗
[Signal-averaged electrocardiography in right ventricular dysplasia].
The authors report their experience of signal-averaged electrocardiography (SAE) in arrhythmogenic right ventricular dysplasia (ARVD) in a study comparing the results in 40 control subjects, 21 patients with ARVD with episodes of sustained monomorphic ventricular tachycardia (VT) (Group I), 7 patients with ARVD without sustained VT (Group II) and 10 asymptomatic members of the families of patients in Group I (Group III). There were significant differences in the results of SAE in the time domain between the control group and Group I, patients with "classical" ARVD. The RMS 40 (root mean square of the last 40 ms of the QRS complex) was the most sensitive parameter but the specificity was less than the averaged QRS width and the low amplitude signal duration. The results in Group II were more varied, some patients having clearly abnormal SAE and others strictly normal recordings. In Group III, the investigation was normal. Spectral analysis after Fourier transformation did not show significant differences between the 3 groups. A difference was observed between the diffuse and localised forms of ARVD in Groups I and II: the localised forms had a shorter QRS width and low amplitude signal duration than the diffuse forms. Also, there was a relationship in Group I between age and duration of QRS and delayed potential which increased and with the RMS 40 or 50 which decreased. This is an argument in favour of the progressive nature of ARVD with respect to time.
Adult↗
[Esophageal pacing in children. 38 consecutive cases].
On the occasion of a preliminary series of 38 cases, the authors review the esophageal pacing technique and its main indications. On the therapeutic level, the esophageal lead may be successfully used to decrease supraventricular tachycardias due to reentry (typical or atypical flutter, reciprocating nodal tachycardia with or without WPW). As a means of investigation, esophageal pacing is overall useful to diagnose undocumented paroxysmal tachycardia fits (palpitations), to evaluate the refractory stage of an accessory pathway (WPW) or to assess the refractory stage of antiarrhythmia medications. This investigation may also be used to assess the sinusal function, the atrioventricular conduction (Wenckebach point) and the spontaneous rhythm of atrioventricular blocks after pacemaker insertion. Due to the technical improvements achieved, esophageal pacing may be used presently in pediatric units taking care of children with arrhythmias.
Adolescent↗
[Late potentials and arrhythmogenic right ventricular dysplasia].
The authors report on their experience in investigating late potentials (LPs) in arrhythmogenic right ventricular dysplasia (ARVD), by comparing 35 patients with ARVD with 40 control subjects. The values of QRS and RMS40 and the duration of the LP showed a marked difference between the control group and the ARVDs, particularly if sustained VT was present. In this case, 68 per cent of patients were found to have LPs (with at least 2 out of 3 parameters abnormal) versus 50 per cent in the ARVDs without sustained VT and 5 per cent in the controls. There was a difference between the diffuse forms (10 per cent) and the localized forms (25 per cent) of ARVD: the LPs were markedly more obvious in the diffuse forms. Likewise, there was a clear relationship between age and the criteria for LPs in ARVD with sustained VT. This is an argument in favour of the evolutive character of the disease, which could be observed directly in a few cases in which the examinations were repeated over the course of time.
Adult↗
[Cardiac stimulators].
Explore the source record for details and available documents.
Arrhythmias, Cardiac↗
Database management of haemodynamic data.
Explore the source record for details and available documents.
Database Management Systems↗
Cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly.
The aim of the study was to evaluate cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly. Eleven children with a mean age of 9.6 years were prospectively studied by spirometry, cardiopulmonary exercise testing (bicycle ergometer n = 8, treadmill test n = 3), and contrast echocardiography. A right-to-left atrial shunt was detected by contrast echocardiography in 7 children (group 1), whereas no shunt was found in 4 (group 2). VO2 max was decreased [84.5 (SD = 16.8)] and was strongly correlated to oxygen saturation in group 1 (p < 0.0001). Oxygen saturation at peak uptake was significantly decreased compared to baseline [97.4 (SD = 2.0) vs 90% (SD = 9.5%), p = 0.02] and was significantly lower in group 1 than in group 2 [85.7 (2.2) vs 98.2% (SD = 1.2%), p = 0. 03]. Oxygen desaturation was related to a right-to-left atrial shunt (p = 0.01). Decreased VO2 max was also correlated to the small size of the left ventricle (p = 0.05). We concluded that decreased exercise tolerance in children with asymptomatic Ebstein's anomaly is related to a right-to-left atrial shunt and to a small left ventricle. In case of poor exercise tolerance, a contrast echocardiography should be performed to detect an atrial septal defect.
Adolescent↗
Heterozygous mutation in the pore of potassium channel gene KvLQT1 causes an apparently normal phenotype in long QT syndrome.
Mutations in KvLQT1, a gene encoding a potassium channel, cause both the recessive Jervell and Lange-Nielsen (JLN) syndrome and the dominant Romano-Ward (RW) syndrome. These diseases are characterised by a prolonged QT interval on the ECG, syncopes and sudden death due to cardiac arrhythmias. The JLN syndrome is also associated with a congenital bilateral deafness. We report here a novel missense mutation, W305S, in the pore region of KvLQT1 identified by PCR-SSCP analysis in two consanguineous JLN families. In contrast to several missense mutations found in the same region of KvLQT1 in RW patients which are associated with severe cardiac phenotypes, the W305S mutation is responsible for an apparently normal phenotype in heterozygous JLN carriers.
Consanguinity↗