[Circular shunting of blood: a complication of neonatal Ebstein anomaly]. / Le shunt circulaire: complication de la maladie d'Ebstein de forme néonatale.

We report a severe neonatal presentation of Ebstein anomaly with homodynamic aggravation at birth attributed to patent ductus arteriosus and circular shunt. Prenatal diagnosis of Ebstein anomaly of the tricuspid valve with functional pulmonary atresia was made at 37 weeks gestation by fetal two- and three-dimensional echocardiography. A cesarean section was performed in view of poor fetal tolerance. The newborn was born with hydrops and multivisceral failure. A post-natal echocardiography demonstrated a left-to-right shunt across the patent ductus arteriosus and functional pulmonary atresia with severe pulmonary insufficiency with absent forward flow. This created a circular shunt, where blood flowed through the ductus to the pulmonary arteries, retrograde through the pulmonary artery and Ebstein valve, across the patent foramen ovale and out the aorta. Prostaglandin E1 infusion was stopped, resulting in clinical and echocardiographic improvement. The management of the phenomenon of a circular shunt across a patent ductus arteriosus with Ebstein malformation involves promoting early ductal closure by stopping prostaglandin therapy.

[Morgagni hernia causing cardiac tamponade]. / Tamponnade néonatale par hernie de Morgagni.

Morgagni hernia is a rare malformation (3% of diaphragmatic hernias). This hernia is usually asymptomatic in children. We report on a case revealed by an unusual complication. Severe cyanosis was due to right-to-left atrial shunt through the foramen ovale assessed by 2D echocardiography. Diagnosis of the Morgagni hernia was made with CT scan. The intrathoracic liver compressed the right chambers of the heart causing tamponade. Cardiac compression was reversed after surgery and replacement of the liver in the abdomen. Six months after the surgery, the infant was symptom-free with normal size right chambers of the heart.

[Fetal myocarditis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block with good outcome]. / Myocardite fœtale sans trouble conductif chez une femme lupique avec anticorps anti-SSA/Ro et anti-SSB/La d'évolution favorable.

We report a case of fetal myocarditis without conductive abnormality in a pregnant woman with anti-Ro/La antibodies. Fetal echocardiography showed myocarditis with ventricular and valvular hyperechogenicity, which was confirmed by postnatal transthoracic echography. Treatment with dexamethasone (4 mg/day) was started in the 22nd week of gestation. The outcome was good, with the child remaining asymptomatic 2 years later. This observation describes one of the rare forms of fetal myocarditis with favorable outcome.

[Candida albicans endocarditis after pulmonary artery banding]. / Endocardite àCandida albicans sur cerclage de l'artère pulmonaire.

Endocarditis is uncommon in infants and is exceptionally related to Candida albicans on pulmonary banding. We report on a case in a 7-month-old infant who had pulmonary artery banding for a ventricular septal defect and who presented with candidal endocarditis. Banding was chosen because of the patient's poor trophic and unstable status, which could be risky for surgery involving extracorporeal circulation. A few weeks after the banding, the patient developed systemic Candida infection, which was treated successfully. At 7 months, cardiac failure appeared without fever or inflammatory signs. Cardiac echography showed that the banding was not protective as well as a hyperechogenic image on the pulmonary bifurcation. The angioscan showed a hypodense thrombus. Emergency surgery was performed consisting of pulmonary artery exploration, thrombectomy, and ventricular septal defect closure. The exploration showed a pulmonary artery perforation caused by the infected pseudoaneurysm and the migration of the banding into the pulmonary artery. The anatomopathologic analysis of the vegetation identified multisensitive Candida albicans. After surgery and prolonged antifungal treatment, progression was satisfactory.

Videoconference pediatric and congenital cardiology consultations: a new application in telemedicine.

BACKGROUND: The relative rarity of congenital heart disease gives it an orphean disease status, requiring specialised centres. The present maturity of information technology allows telemedicine to be integrated into current medical practice. We report our experience of telemedicine between the cardiology department at St Pierre Hospital on the island of Réunion and the pediatric cardiology department at the teaching hospital in Toulouse. AIMS: The aims of this work were to 1. verify the technical feasibility of transmitting echocardiographic images, 2. determine an optimal therapeutic strategy for each patient, and 3. deliver precise information live to patients and their families. METHODS: Five pediatric cardiology videoconference consultation sessions were transmitted between April 2006 and May 2007. The videoconference equipment, POLYCOM VSX 7000 (R), was used to relay information between the two centres, using six high-debit digital telephone lines, allowing a transfer rate of 384 kbits/s and an image frequency of 25 frames per second. The echocardiographic equipment at St Pierre Hospital was connected to the videoconference equipment by an S-VHS video output. The transmitted sources alternated between the echographic video output and the signal from a video camera, with continuous audio transmission. RESULTS: The telemedicine meeting was made up of three main elements: 1. a consultation with real-time echocardiographic acquisition and transmission, 2. a discussion between medical colleagues, and 3. a discussion with the family. Five videoconference consultation sessions were organised between April 2006 and May 2007. 22 patients were involved (median age 3 years, age range 7 days to 48 years). Heart disease was congenital in 20 patients, and acquired in 2 patients. The aim of the telemedicine consultation was to specify: 1. medical treatment in 7 patients, and 2. an indication for surgery or interventional catheterisation in 15 patients. There was no significant change in diagnosis, but in 2 patients with complex heart disease some anatomical clarifications were made. For 3 patients, the videoconference discussion was essential to get the extremely reticent families to accept the indication for surgery. CONCLUSION: This is the first experience in France of telemedicine consultation for pediatric and congenital cardiology. These videoconferences allowed patients in the south of Réunion to benefit from a specialist opinion on optimal therapeutic strategy, with no delay or need to travel a long distance.

[Evaluation of left ventricular diastolic function in children by doppler tissue imaging]. / Evaluation de la fonction diastolique par Doppler tissulaire chez l'enfant.

Left ventricular diastolic function may change at an early stage in cardiac disease. It is often difficult to assess in daily practice. The use of Doppler tissue imaging at the annulus has been validated in adults. This method is little used in paediatrics and the physiological norms have not been established in children. Forty three children aged 7 days to 241 months were referred for a cardiological opinion with normal echocardiogrammes were included. Myocardial velocities were measured by Doppler tissue imaging of the left and right ventricular walls at different moments of the cardiac cycle in the apical 4-chamber view. A complete study was possible in 39 cases (91%). Doppler tissue imaging was not performed in one case and was incomplete on the right ventricle in 3 children. The median of the lateral mitral tissue E wave (Ea) was 16.3 cm/s and that of the right ventricle was 15.8 cm/s with a tissue Ea/Aa ratio of 2.6 and 1.6 respectively. The median of the tissue S waves was 8.8 cm/s for the left ventricular lateral wall and 13.3 cm/s for the right ventricular lateral wall. The E/Ea ratio of the left ventricular lateral wall was 5.9. Although the velocities of the left ventricular lateral wall were not related to the children's' age or size, the correlations between the E/Ea ratio and age and size were statistically significant. The myocardial velocities of the neonate were characteristic and different to those of the older paediatric population (slower Ea and S waves, faster Aa with a higher E/Ea ratio). The authors conclude that Doppler tissue imaging is feasible in clinical paediatric cardiology. Comparative studies with populations with cardiac disease are necessary to determine pathological values.

[Transposition of the great arteries with pulmonary valvular stenosis and intact interventricular septum. Discussion of management strategies with respect to one case]. / Transposition des gros vaisseaux avec sténose valvulaire pulmonaire à septum interventriculaire intact. Discussion des enjeux thérapeutiques à propos d'un cas.

The authors report the case of a neonate with transposition of the great arteries (TGA) with pulmonary stenosis (PS) and intact Interventricular septum. The child was born at full term without a prenatal diagnosis with cyanosis developing at the 12th hour. After emergency atrioseptostomy, detransposition was performed on the 6th day. Commissurotomy and shaving of the much thickened bicuspid pulmonary valve was also performed. The postoperative course was complicated by a non-compliant left ventricle. After two months, myocardial ischaemia and persistent obstruction of the outflow tract led to severe cardiac failure and the death of the child. TGA with PS and intact interventricular septum is a very rare cardiac malformation. The prognosis is closely related to the anatomy of the pulmonary valve and the impact on the left ventricle. The therapeutic options are complex and are discussed in relation to this case.

[Arrhythmia follow-up of children and adolescents with neuromuscular diseases]. / Surveillance rythmologique de l'enfant et l'adolescent avec maladie neuro-musculaire.

Much progress has been made over the last few years in understanding and classifying neuromuscular diseases. The heart is frequently affected but often in a dissociated manner with respect to the neuromuscular signs although it has a significant impact on the prognosis. In children and adolescents, the dystrophinopathies, especially Duchenne's muscular dystrophy, are the principal problems but the mild arrhythmic events observed seem to be related to left ventricular dysfunction. On the other hand, in myotonic dystrophies (Steinert's disease), ventricular arrhythmias or conduction defects may appear at an early stage of the disease with serious consequences justifying appropriate follow-up and invasive preventive measures. Emery Dreifuss X-linked dystrophy and other laminopathies are rare conditions but are associated with sudden death and cardiomyopathies of the young adult. Specialised cardiological follow-up is justified in childhood from the time of diagnosis. Medication or implantable electric devices may be justified before the end of the second decade of life. Progressive infra-hisian conduction defects have also been reported in Kearns-Sayre oculo-pharyngeal myopathy. Prospective studies are required at this age to determine the natural history of these pathologies that are probably under diagnosed. The present recommendations, which are based mainly on data from adult series, could then be adapted for younger patients.

[Very large cardiac tumor revealed by ventricular arrhythmia in a 18-month-old infant]. / Volumineuse tumeur cardiaque révélée par un trouble du rythme ventriculaire chez un nourrisson de 18 mois.

Cardiac tumors are rare in childhood and can be revealed by arrhythmias. We report the observation of an 18-month-old infant who had an episode of ventricular tachycardia (VT) which resulted in a large intramyocardic tumour diagnosis evocating a left ventricular fibroma. A treatment by amiodarone allowed a stable reduction of the VT. The presence of an intracardiac obstruction or uncontrollable arrhythmias would lead to a surgical resection.

[New approach to perimembranous ventricular septal defect by real-time 3D echocardiography]. / Nouvelle approche des communications interventriculaires périmembraneuses par échocardiographie 3D temps réel.

Perimembranous ventricular septal defects (VSD) have a close anatomical relationship to the aortic and tricuspid valve which may make percutaneous closure difficult. The authors' objective was to study the anatomy of perimembranous VSD by real-time 3D echocardiography. Fourteen patients aged 1 day to 3 years (mean 3 months) underwent transthoracic 2D and 3D echocardiography. Two patients (> 10 kg) underwent closure of the perimembranous VSD with an Amplatzer occluder. The other 12 patients were operated (surgical closure in 11 and circling suture with resorbable thread in 1 patient). The 3D echocardiography showed variations in the geometry of the perimembranous VSD (eccentric, oval, cleft). The maximal diameter of the septal defect by 3D echocardiography was on average 2 mm greater than the diameter by 2D echocardiography. The 3D echo enabled visualisation of the close relationship of the septal tricuspid leaflet, the aortic valve and the perimembranous VSD, showing the mechanism of its closure and the associated aortic regurgitant lesions. The profile of the prosthesis implanted percutaneously was well shown in the different 3D views. The authors conclude that real-time 3D echocardiography is a technique which allows accurate anatomic description of perimembranous VSD. As a complement to classical 2D methods, it gives a better appreciation of the maximal diameter of the septal defect and of its relationship to the aortic and tricuspid valves.

[Unusual outcome of mitral valve infectious endocarditis in a premature infant]. / Evolution inhabituelle d'une endocardite infectieuse mitrale chez un prématuré.

The authors report a case of Staphylococcus Aureus infectious endocarditis in a premature baby. Echocardiography on the 8th day of life showed a large vegetation on the anterior mitral leaflet without significant regurgitation. Intravenous antibiotics and platelet antiaggregant therapy were given. At three weeks of age the vegetation split into two, one part attached to the foramen ovale and the other to the anterior mitral leaflet. At one month, the development of severe mitral regurgitation led to surgical valvuloplasty in a 2 kg neonate, the vegetation on the foramen ovale having spontaneously fragmented. The ablation of the mitral vegetation associated with commissuroplasty reduced the mitral regurgitation. At 3 months after surgery, the child was asymptomatic with a minimal mitral regurgitation.

[A rare association of double discordance with aortic arch anomalies: value of multislice CT scan]. / Association exceptionnelle d'une double discordance avec anomalie des arcs aortiques: apport du scanner multicoupe.

The authors report the case of a 14 month old infant admitted for intermittent stridor. Routine echocardiography showed atrioventricular and ventriculo-arterial discordance with an intact septum and situs solitus with levocardia. Multislice CT scan showed tracheal compression due to a Neuhauser anomaly of the aortic arches. The paucity of symptoms led to surgical abstention.