The vertebrate left-right axis is specified during embryogenesis by a transient organ: the left-right organizer (LRO). Species including fish, amphibians, rodents and humans deploy motile cilia in the LRO to break bilateral symmetry, while reptiles, birds, even-toed mammals and cetaceans are believed to have LROs without motile cilia. We searched for genes whose loss during vertebrate evolution follows this pattern and identified five genes encoding extracellular proteins, including a putative protease with hitherto unknown functions that we named ciliated left-right organizer metallopeptide (CIROP).
View Article and Find Full Text PDFObjective: To assess the feasibility, safety, and efficiency of ductal stenting in pulmonary atresia with intact ventricular septum or critical pulmonary stenosis after balloon pulmonary valvuloplasty.
Background: Ductal stenting in pulmonary atresia with intact ventricular septum is a re-emerging and promising technique. There is little data available on its outcomes after establishing prograde pulmonary blood flow.
We present a case in which transthoracic echocardiography was the first diagnostic tool to suspect mediastinal Hodgkin's lymphoma by revealing a change in the hemodynamic of left pulmonary artery flow, and it was used as a follow-up method for monitoring treatment efficacy by demonstrating a normalization of pulmonary artery hemodynamics.
View Article and Find Full Text PDFObjectives: The main aim of this study is to evaluate the significance of several clinical and laboratory findings presented by a large number of pediatric patients who were suspected to have acute rheumatic fever (ARF) by physicians who ordered echocardiogram for confirmation and for avoiding overuse of unnecessary antibiotics. These suspecting reasons include elevated ASO titers and recurrent tonsillitis. Another aim is to stress the role of Doppler echocardiogram in confirming the diagnosis of ARF by detecting rheumatic heart disease (RHD) in suspected cases.
View Article and Find Full Text PDFObjective: 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 dysmorphic syndrome and possible damage to the coronary ostia, the means of diagnosis and outcome of these patients.
Materials And Methods: 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.
Results: SVAS were found in 14 patients (0.
Coarctation of the aorta in neonates and small infants is, in most cases, a severe congenital malformation and requires a rapid surgical treatment or interventional catheterization. To our knowledge, and so far, no cases of coarctation of the aorta have spontaneously improved. We report three cases of coarctation diagnosed in young infants and considered as moderate forms on physical exam and/or ultrasound study which evolved to partial or complete regression without any treatment.
View Article and Find Full Text PDFObjective: Study various parameters encountered in the linear insertion of the atrioventricular valves (LIAVV) in patients with and without Down's syndrome (DS).
Material: A group of patients (n = 6240) aged from 0 to 16 years with and without congenital heart disease (CHD) registered over 6 years (1.07.
The significant amount of waste generated from construction demolition has become a chronic problem in many developing countries. Using data obtained from demolition contractors and various other sources, this paper proposes a framework for proper handling of construction demolition waste (CDW) to serve as a decision support tool in countries suffering from the lack of national CDW management guidelines. The framework is then demonstrated through a case study in the city of Beirut, Lebanon, and a sensitivity analysis is carried out to examine the economic feasibility of developing a recycling facility.
View Article and Find Full Text PDFUnlabelled: Percutaneous closure of the patent ductus is the gold standard therapy. Our aim was to analyze our failures between 2001 and 2010.
Methods: All patients over 5 kg benefited from a transcatheter attempt at duct closure.
Objective: Describe a mechanical method of perforation-dilatation of the pulmonary valve in pulmonary atresia with intact interventricular septum (PA-IVS), with or without stenting the patent ductus arteriosus (PDA) and medium-term results.
Method: Since 2007, all patients with PA-IVS and a right ventricle adequate for biventricular repair, benefited from a transcatheter attempt to perforate-dilate the valve with or without stenting the PDA, and were included in this retrospective study.
Technique: A catheter was percutaneously introduced through the femoral vein and positionned in front of the atretic pulmonary valve.
Objectives: To study the incidence, etiology, clinical signs and treatment of children with mitral valve disease in Lebanon.
Patients And Methods: This is a retrospective study on a period of 9 years (between May 1, 1999 and April 30, 2008), including a group of children with congenital and acquired heart disease, consecutively enrolled in the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology.
Results: 2586 new patients aged 0-18 years were recorded.
Objectives: Analyze the possible role that can play parental smoking during early pregnancy on the occurrence of different types of congenital heart defects (CHD).
Materials And Methods: Over a period of 4.5 years (between January 1, 2006 and June 30, 2010), data on all children who had an echocardiography were collected and entered in the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology, Beirut.
We hereby review liver transplantation for homozygous familial hypercholesterolemia and report the case of a 14-year-old girl presenting with severe bilateral coronary ostial stenosis and tight supra-valvular aortic narrowing 10 years after liver transplantation. Despite normalization of the lipids after liver transplantation, the patient showed evidence of severe cardiac atherosclerosis 10 years later and died of apparent sepsis.
View Article and Find Full Text PDFObjectives: Analyze the demographic and clinical characteristics of complete atrioventricular septal defect (AVSD), its association with Down's syndrome, with other cardiac and extra-cardiac anomalies, and finally the impact of consanguineous marriages on the incidence of AVSD.
Patients & Methods: The sample consisted of 2195 consecutive patients with congenital heart defect, entered in the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology, Beirut, between Jan 1999 and Dec 2007. 120 patients with AVSD were analyzed.
Objective: Study the new Amplatzer Duct Occluder II (ADO II). Limitations were encountered with the preexisting devices in nonconical ducts, large ducts, or in small infants. These include failure, residual shunts, protrusion, migration, and vascular damage.
View Article and Find Full Text PDFBackground: Despite the availability of effective devices, percutaneous closure of patent ductus arteriosus (PDA) can be challenging in some situations.
Aim: To describe our initial experience of percutaneous PDA closure.
Methods: Between 2001 and 2007, 73 consecutive patients aged 3 months to 70 years underwent transcatheter PDA closure.
Objectives: To describe the characteristics and outcome of children with an isolated silent patent ductus arteriosus (SPDA), with comparison to non-silent ducts.
Patients And Methods: 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.
Objective: Analyze the role of parental consanguinity and/or maternal age on the occurrence of congenital cardiac anomalies in infants with Down's syndrome (DS).
Material And Methods: A large group of patients with or without congenital heart disease, registered between May 1st, 1999 and June 30th, 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.
Objectives: To explore the association of congenital heart disease and malformations of the gastrointestinal tract/abdominal wall.
Patients And Methods: Over seven years, 105 neonates presenting with congenital gastrointestinal/abdominal wall anomalies were explored for associated congenital heart disease. Sex, consanguinity, type of cardiac anomaly, type of gastrointestinal/abdominal wall anomaly, and chromosomal anomalies were all analyzed in order to find predisposing factors for this association.
Aims: To test on a large cohort whether parental consanguinity varies among different types of congenitally malformed hearts.
Methods And Results: Between 1 May, 1999, and 28 February, 2006, a large cohort of 1585 newly diagnosed cases with non-syndromic congenitally malformed heart was enrolled at the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology, Beirut. Another group, made up of 1979 cases referred to the National Register of Paediatric and Congenital Heart Disease, but free of any malformation, and with a rate of consanguinity similar to a recent survey made by UNICEF in Lebanon, was used for the purposes of control.
Objectives: To study the different indications behind performance of a first Doppler echocardiography (DE) in children, to assess the results and their impact on the residency teaching program in Lebanon.
Material And Methods: Retrospective study over a 40-month period, from March 1st, 2002, through June 30, 2005. Patients referred for the first DE were examined in different medical centers in Beirut, Mount-Lebanon and South Lebanon.
Objectives: We evaluated our immediate and midterm results of balloon dilation of critical valvular aortic stenosis in 15 consecutive neonates.
Methods: Balloon dilatation was attempted in 15 neonates at a mean age of 14 days. Three patients (20%) had associated left heart hypoplasia.