Supravalvular aortic stenosis with and without coronary lesions in pediatrics

This study aims to analyze several parameters concerning the supravalvular aortic stenosis [SVAS] in children such as age of diagnosis, place of residence, the existence of a metabolic disorder or dysmor-phic syndrome and possible damage to the coronary ostia, the means of diagnosis and outcome of these patients. A large group of patients [2868] with congenital heart disease enrolled between 1 May 1999 and 30 April 2010 at the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology. SVAS were found in 14 patients [0.5%] aged 8.5 months to 15 years. The Williams Beuren syndrome was the most common etiology [6 cases] without ostial stenosis, asymptomatic and not treated, followed by forms without dysmorphic syndrome [5 cases] and without ostial stenosis, one patient was operated because of severe SVAS ; finally, 3 cases of homozygous familial hypercholesterolemia treated differently: a patient had a successful liver transplantation at age of 4.5 years but has developed, despite the normalization of cholesterol level, a SVAS associated with severe ostial lesions 10 years after transplantation, another treated by coronary artery bypass graft surgery and the latter treated medically. SVAS is a very rare disease, but its discovery must lead to search for coronary lesions especially in presence of homozygous familial hypercholesterolemia. The ultrasound monitoring is mandatory and is designed to detect this anomaly and early coronary lesions. The slightest suspicion should carry out more extensive explorations to detect ostial stenosis

silent patent ductus arteriosus

To describe the characteristics and outcome of children with an isolated silent patent ductus arteriosus [SPDA], with comparison to non-silent ducts. Between 1999 and 2004, all consecutive cases of isolated silent and non-silent-patent ductus were recorded at the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology. Patients with a SPDA were followed clinically and by Doppler echocardiography while all non-SPDA were percutaneously or surgically closed. Twenty-four cases of isolated SPDA and 50 cases of isolated non-SPDA ducts were recorded. Male sex was significantly predominant in the silent group [70%]. First-cousin consanguinity rates were not different between both groups, with 20.4% for the silent group versus 22% for the non-silent group. Down's syndrome was associated in three cases of SPDA. No cases of endocarditis were noted during a mean follow-up of 33.3 months. Four patients with a SPDA experienced spontaneous closure at the age of 25,30,36 and 58 months. SPDA is a relatively benign disease. The risk of endocarditis cannot be totally ignored, but the systematic closure of the SPDA is not warranted. Larger series and longer follow-up are needed in order to draw conclusions. Spontaneous closure occurred in four patients with SPDA

Cardiopathie congenitale, age maternel et consanguinite parent ale chez les enfants avec syndrome de down

Analyze the role of parental consanguinity and/or maternal age on the occurrence of congenital cardiac anomalies in infants with Down's syndrome [DS]. A large group of patients with or without congenital heart disease, registered between May 1[st], 1999 and June 30[th], 2006, at the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology. 217 children with DS have been enrolled over this period. Group I: DS with cardiac anomalies [n: 119]. Group II: DS without cardiac anomalies [n: 98]. The consanguinity rate was compared to a study made in 2000 by UNICEF in Lebanon, in which the consanguinity rate was 14.9% of 1st degree cousins and 25% of all degrees together. Congenital cardiac anomalies were found in 54.2% of babies with DS. First degree cousins in both groups I and II was 143% [close to the general population], and it was lower in group I compared to group II [11.8% and 17.3% respectively ; p = 0.205] ; this difference was not statistically significant for all degrees together. Maternal age ranged from 20 to 47 years, with a mean of 29.5 +/- 5.3 years. The risk of congenital cardiac anomaly was greater [p < 0.05] when maternal age was equal or below 32 years. As in the international literature, complete atrioventricular canal and ventricular septal defect were the most frequent cardiac anomalies. In children with DS, the risk of congenital cardiac anomalies was not associated with the parents consanguinity. Maternal age above 32 years seem to be associated with a lesser occurrence of congenital cardiac anomaly in children with DS

Fermeture percutanee des communications interauriculaires par la prothese Amplatzer

Percutaneous closure of an atrial septal detect [ASD] was undertaken in 10 patients aged from 3 to 33 years. The Amplatxer device was introduced intravenously and positioned under fluoroscopic and echographic control. the size of the ASD varied from 13 to 20 mm and that of the devices from 15 to 32 mm. one patient was operated for the removal of an improperly positioned device and closure of the ASD. All the other patients were discharged 24 hours after the procedure with successfull closure of the ASD. Complications were benign: spontaneously regressive arrhythmias [1 case] and migraines [2 cases]. our series reflects the experience of others, showing the faisability and efficiency of the procedure. tridimensionnal or intracardiac echocardiography may improve this technique in the future

Fermeture percutanee du canal arteriel chez l'enfant une premiere serie au Liban

Percutaneous closure of the patent ductus arteriosus [PDA] has become an efficient and safe technique in children. We report the first Lebanese series of 8 children who had their PDA closed percutaneously with a coil or an Amplatxer Duct Occluder. Patients median age was 3.2 years [range 1.2 to 12 years]. The median minimal diameter of the PDA was 2.1 mm [1.2 to 4 mm]. A coil was used in 4 children and an Amplatxer occluder in the 4 other patients. The device was chosen according to the diameter of The PDA The results were excellent in all patients. A trivial shunt present in 3 cases, disappeared within 8 weeks. No complication were noted in this series. Percutaneous closure of the PDA in children, using interventional radiology, is a simple and reproducible technique. It is now the treatment of choice in most pediatric cardiac centers worldwide