Frequency of the severe combined immunodeficiency disease gene among horses in Morocco.

Severe combined immunodeficiency disease (SCID) of horses is an autosomal, recessive hereditary disease occurring among Arabian or crossbred Arabian horses. The genetic defect responsible was previously identified as a 5-base pair deletion in the gene encoding the catalytic subunit of the DNA dependant protein kinase (DNA-PKcs). This study was carried out to determine the frequency of SCID and identify horses carrying the gene for SCID among Arabian and Arabian crossbred stallions and mares in Morocco using a DNA-based test. Twenty-one horses were SCID carriers: 14 (7%) Arabians, 6 (4%) Arab-Barbs and one (33%) Anglo-Arab. After analysing their genealogy, 3 imported stallions were identified that disseminated the mutant gene of DNA-PKcs in Morocco.

Influence of clinical and hemodynamic characteristics on signal-averaged electrocardiogram in postoperative tetralogy of Fallot.

This study examines the relation between signal-averaged electrocardiographic measurements and the occurrence of spontaneous ventricular arrhythmias in 86 patients with a postoperative right bundle branch block after repair of tetralogy of Fallot; special attention was given to the influence of age, body surface area and right ventricular systolic pressure on signal-averaged electrocardiograms. Twenty-eight of the 86 patients had significant ventricular arrhythmias on 24-hour ambulatory monitoring. A positive linear correlation was found between filtered QRS duration and age at evaluation or body surface area (r = 0.45, p = 0.00001; r = 0.54, p < 0.00001, respectively) and between amplitude of the last 40 ms of the filtered QRS and right ventricular systolic pressure (r = 0.48, p < 0.001). A negative linear correlation was found between amplitude of the last 40 ms of the filtered QRS and age at evaluation or body surface area (r = -0.27, p = 0.01; r = -0.34, p = 0.002, respectively). When the age of the patients or the body surface area was considered with an analysis of covariance, the presence of ventricular arrhythmias was associated with a higher amplitude of the last 40 ms of the filtered QRS. In addition, an amplitude of the last 40 ms of the filtered QRS > 170 microV had an excellent sensitivity (100%) and a good specificity (88%) for identifying patients with both right ventricular systolic hypertension and spontaneous ventricular arrhythmia. Thus, adjustment of signal-averaged parameters for age at evaluation and body size is mandatory when studying postoperative tetralogy of Fallot.

[Influence of age and body surface on the signal averaged high amplification ECG after complete repair of tetralogy of Fallot]. / Influence de l'âge et de la surface corporelle sur le signal moyenné de l'ECG haute amplification après réparation complète des tétralogies de Fallot.

The aim of this study was to assess the influence of age and body surface area on the signal averaged ECG after complete repair of Tetralogy of Fallot. Fifty eight patients operated for Tetralogy of Fallot in whom the postoperative ECG showed right bundle branch block without any significant arrhythmia on Holter monitoring or exercise stress testing underwent signal averaged electrocardiography. A very significant relationship was observed between age and body surface area and the duration of the filtered QRS (r = 0.45, p = 0.0004 and r = 0.49, p = 0.00009 respectively) or the average voltage of the last 40 milliseconds (r = -0.49, p less than 0.03 and r = -0.31, p less than 0.02 respectively). Therefore, signal averaged ECG in patients operated for Tetralogy of Fallot varies with age and morphology. An adjustment of the parameters of the signal averaged ECG with respect to clinical characteristics is necessary before assessing the value of this technique for identifying patients at risk of developing ventricular arrhythmias.

[Holter monitoring, exercise test and atropine test in isolated congenital atrioventricular block in children]. / Evaluation par enregistrement Holter, épreuve d'effort et test à l'atropine des blocs auriculo-ventriculaires congénitaux isolés de l'enfant.

Twenty-four patients with isolated congenital heart block were investigated by 24-hour Holter monitoring at an average age of 9.3 +/- 5.5 years. Six patients were symptomatic and 18 were asymptomatic. Eight asymptomatic patients underwent exercise stress tests and an atropine test was performed in 10 asymptomatic patients to evaluate the capacity to accelerate the heart rate. The symptomatic patients were older than the asymptomatic patients. None of the parameters which analyse ventricular rate were significantly different in the two groups of patients. Significant ventricular arrhythmias (Lown Grade 2 or over) were recorded in 1 symptomatic and 3 asymptomatic patients. The incidence of these ventricular arrhythmias increased with age and degree of bradycardia. The percentage increase in ventricular rate after atropine correlated with what was observed on effort (r = 0.95, p = 0.01) but there was no relationship between the ventricular rates during these two tests and those recorded on Holter monitoring. The results of this series of children with isolated congenital heart block show the Holter parameters cannot distinguish symptomatic from asymptomatic patients. The exercise stress and atropine tests gave very similar results but their prognostic value has not yet been established.

[Infectious endocarditis of the tricuspid valve in patients with endocardial pacemakers]. / Endocardites infectieuses tricuspidiennes sur sonde de stimulation permanente endocavitaire.

Right heart endocarditis in patients with permanent cardiac pacing catheters is uncommon but not an exceptionally rare complication. Two dimensional echocardiography provides diagnostic and prognostic information. The authors report two cases which illustrate the fact that many workers recommend explanation of the pacing catheter and that tricuspidectomy with or without valve replacement may sometimes be necessary.

[Incidence and factors favouring ventricular arrhythmia after surgical repair of tetralogy of Fallot]. / Incidence et facteurs favorisant les arythmies ventriculaires après réparation de tétralogie de Fallot.

The prevalence and factors favorising postoperative ventricular arrhythmias in 62 patients undergoing surgical repair of tetralogy of Fallot between 1971 and 1982 were analysed. Nineteen patients (31 %) had significant ventricular arrhythmias (Lown greater than or equal to 2) on Holter monitoring or exercise stress testing. The arrhythmia could only be recorded after stress testing in 5 patients. The patient's age at operation, the duration of follow-up and age at evaluation were significantly greater in the group with ventricular arrhythmias. However, age at operation was closely related to the other two parameters and a discriminating analysis showed that the age at operation was not a favorising factor for the occurrence of ventricular arrhythmias (p = 0.23), in contrast to the duration of follow-up (p = 0.0015) and age at evaluation (p = 0.0007). No relationship was found between ventricular arrhythmias and the following factors: previous anastomosis, outflow patch, necessity of a ventriculotomy or reoperation, presence of residual ventricular septal defect, postoperative systolic right ventricular pressure, severity of intraventricular conduction defects, and effort tolerance. These results suggest that after surgical repair of tetralogy of Fallot, the incidence of ventricular arrhythmias increases with time. The main problem is to identify patients with a high risk of sudden death, i.e. those with high degree ventricular arrhythmias and poor haemodynamic results of repair.