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Résumé Introduction : La maladie d'Ebstein (ME) est une cardiopathie congénitale rare et hétérogène touchant la valve tricuspide et le ventricule droit. Peu d'études ont analysé les caractéristiques électrocardiographiques de cette maladie. Objectif : Décrire les aspects électrocardiographiques observés dans la maladie d'Ebstein. Méthodes : Nous avons mené une étude descriptive à recrutement rétrospectif colligeant 26 patients suivis pour ME. Résultats : L'âge moyen de découverte de la ME était de 103.5±99 mois [0-31 ans]. Le diagnostic de ME est le plus souvent fait entre 5 et 10 ans. Nous avons noté une hypertrophie auriculaire droite chez 11 patients (42%), une hypertrophie ventriculaire droite chez la moitié des patients. Une déviation axiale droite a été notée chez 11 patients (42%). Huit patients (30%) avaient des QRS larges de durée ≥ 120 ms. Sept parmi ces 8 patients (27%) avaient un aspect fragmenté des QRS. Un bloc de branche droit a été noté chez 22 patients (84%), complet dans sept cas (27%). Un aspect de préexcitation a été retrouvé chez 6 patients (23%). La voie accessoire était de localisation postéro-septale droite dans tous les cas. Les troubles de rythme ont été notés chez 9 patients (34%). Il s'agit d'une tachycardie jonctionnelle chez 3 patients (11%), un flutter auriculaire chez 4 patients (15%) et une fibrillation auriculaire chez 2 patients (7%). Un bloc auriculo-ventriculaire de 2ème degré a été objectivé chez un patient, de type Mobitz I. Deux cas de troubles du rythme post-opératoires ont été enregistrés, il s'agit d'une fibrillation auriculaire paroxystique et d'une tachycardie jonctionnelle en rapport avec un syndrome de Wolf Parkinson White (WPW). Conclusion : L'ECG de surface dans la ME est souvent pathologique avec prédominance de troubles de rythme en rapport avec un syndrome de WPW.

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Introduction A diagnosis of ductal infective endocarditis (DIE) usually relies on the clinical histories of infectious syndrome, results of blood cultures and echocardiography. It remains a therapeutic challenge. The prognosis is still poor. Is the prevention of infective endarteritis a valid reason to always close the isolated silent PDA in childhood? The question is not yet eluded [1] , [2] , [3] , [4] . We present the case of six years old girl with DIE developed after dental procedure. The patient was managed by combined medical and early surgery with favorable evolution. Case report A six year old girl was admitted to our service with tachypnea, fatigue and fever lasting for seven days. The patient had dental procedures few days earlier. The physical examination revealed a temperature at 40 °C and a left subclavian murmur. The acute phase marker levels were significantly elevated and β-hemolytic group A streptococcus was grown in his blood culture. Echocardiography revealed vegetations with the diameter of 10 mm on the left side of the PDA ( Fig. 1 ) with turbulent flow in the left pulmonary artery. Treatment with ceftriaxone 200 mg/kg/day and gentamycin 5 mg/kg/day was started then readapted according to antibiogram. The operation was performed on day 15 of antibiotherapy ( Fig. 2 a and b), whom was continued for 4 weeks. The fever did not return and the clinical course of the patient was stable; the final echocardiogram revealed the absence of left ventricular dilatation with a correct systolic function, normal pulmonary pressure. She was discharged four weeks after the surgical cardiac procedure. Conclusions Infective endarteritis (IE) complicating PDA is very rare nowadays and the risk is very low especially in the industrialized world. Indeed, standardized management by a skilled multidisciplinary team with expertise in imaging, surgery and intensive care has proven to decrease mortality [3] , [4] , [5] , [6] , [7] . Primary prevention is vital. Even though it was not clear if the closure of a small PDA was beneficial, routine closure of any PDA in children and young adults appeared reasonable. The reasons for this recommendation included the risk of infective endocarditis and low-or-zero morbidity from closure, especially when using a transcatheter device [8] , [9] .

Introduction Interrupted aortic arch (IAA) is a rare congenital ductal-dependent heart disease. It commonly presents in infancy following spontaneous closure of the patent ductus arteriosus (PDA) [1] , [2] . Few cases have been reported in pediatric age [3] . We present the case of 13 years old boy with IAA and a large ventricular septal defect (VSD), a restrictive PDA and an Eisenmenger syndrome. Case report A thirteen years old Tunisian boy presented to our consultation with malaise, headache and weakness of his legs. He reported reduced exercise tolerance since two years. There was no family history of congenital heart disease or sudden unexpected death ( Fig. 1 , Fig. 2 ). Physical examinations noted a generalized cyanosis. Radial pulses were normal but his femoral pulses were weak. The systolic pressure difference between the upper and lower limbs was 40 mmHg. Transthoracic echocardiography noted a conal VSD with a posterior malalignment of the conal septum with right to left shunt. The short axis examination showed a large pulmonary tract and a restrictive PDA with an increased velocity of 3.3 m/s and right to left shunt. The high left parasternal examination with a slightly rightward angulation of the transducer demonstrated a complete interruption of aortic arch. Computed tomography revealed an IAA between the left common carotid artery and the left subclavian artery (type B) and a PDA (3.3 mm) between the left pulmonary artery (LPA) and the descending aorta. Pulmonary arterial hypertension was confirmed in cathlab with mean pulmonary arterial pressure (mPAP) at 100 mmHg. Following multidisciplinary team discussion, on regard of patient health state, Eisenmenger syndrome, the restrictive PDA and the contraindication to performing surgery, we discuss the percutaneous stenting of the PDA. Conclusion Interrupted aortic arch is a rare congenital abnormality that presents in infancy. We presented the case of 13 years old boy with IAA, Eisenmenger syndrome and a restrictive PDA. Following multidisciplinary team discussion, giving the restrictive pattern of the PDA and the Eisenmenger syndrome, we discuss a percutaneous of the PDA.

This is a retrospective study including 47 patients with isthmic coarctation (CoA) diagnosed in ante et postnatal périod, hospitalized in pediatric cardiology departement of la Rabta Hospital-Tunisia during the period from 2000 to 2017.They were 36 girls and 11 boys with an average age of 14 days. The diagnosis of CoA was suspected during the anténatalperiod in eight cases. In postnatal period heart failure was observed in (38.5%), abolition of femoral pulse (74%), a tension asymetry was found in all patients. Antenatal echocardiography suspected indicators of fetal coarctation especially ventriculo-arterial asymmetryechocardiography confirmed the diagnosis of CoA ,The ejection fraction was impaired in 31% of the cases.The abnormalities associated with coarctation were dominated by the patent ductus arteriosus (68%), atrial septal defect (55.5%), aortic hypoplasia, bicuspidia in respectively in 34% and 31% coarctation syndrome in (23.4%). Prostaglandin wasneccessary in (89.3%). forty two patients were operated with good immédiaterésults. the early postoperative mortality was 12.5%. In the long term, we deplored two late deaths, six cases of recoarctation with a follow-up of 14 months treated by percutaneous angioplasty with a result considered good in all cases.Coarctation of the aorta in neonates is a special form of aorticcoarctaion that differs from the child and adult forms in clinical, echocardiographic, and therapeutic characteristics . His clinical diagnosis must be early, if possible in antenatal , to asssure optimal management.

The common arterial trunk is a heart disease in witch a unique arterial trunk, with a unique ventriculo-arterial valve, exits from the ventricular mass and supply directly the coronary, the aortic and also the pulmonary arterial circulations. Its anatomic repair is now possible but necessitates the use of conduit in pulmonary position.To evaluate the incidence and the causes of late reinterventions after repair of common arterial trunk.We retrospectively study the outcome of 17 patients who underwent repair of common arterial trunk between January 1983 and December 2006. The 15 early survivors were followed during a median period of 7 years (range 10 months and 13 years).Nine reinterventions were necessary in 8 patients. Only one conduit reintervention was necessary in the 8 patients. Freedom from conduit reintervention was 73% at 5 years and 33% at 10 years. Three reinterventions were performed in 2 patients for severe truncal valve incompetence, including repair in 2 cases and replacement in one case. Freedom from truncal valve reintervention was 67% at 10 years.Late reinterventions are inevitable after repair of common arterial trunk. The most common reasons are conduit stenosis and truncal valve incompetence.

Between 1995 and 2002, 5 patients with a mean age of 32 years have been treated for atrial flutter in the Department of Paediatric Cardiology at Hospital La Rabta. In all cases, diagnosis was established on surface electrocardiogram (12 leads. All patients had a normal heart. The 3 infants were in heart failure and In the 2 neonates, the arrhythmia was well tolerated despite a very fast ventricular rate. Reduction of the first episode of atrial flutter was possible by cardioversion in 3 patients, 2 of them had a good impregnation with Amiodarone. The two neonates died at 8th and 10th day of life. In the long term follow-up, on infant presented two recurrent episodes at 4 months x 2 years. Atrial flutter, when diagnosed in neonatal period is a rare but serious rhythm disturbance. The mechanism of activation of this rhythm disturbance remains unknown in the foetus, neonate and infant.