[Parental smoking during early pregnancy and congenital heart defects]. / Tabagisme parental en début de grossesse et cardio- pathies congénitales.

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. Patients were divided into two groups: group I without CHD, and group II with CHD. Variables recorded were: age of mother, place of residence, parental smoking, the type of CHD, the sex of the child and consanguinity of the parents. We used nonparametric tests for comparing the two populations. The p value was considered significant when inferior to 0.05. RESULTS: Group I consisted of 2466 patients and group II of 793 patients. Only valvar pulmonary stenosis (VPS) was highly associated with mothers who smoked 20 cigarettes or more per day (p = 0.03). When the mother and father consumed at least 20 cigarettes per day, VPS and coarctation of the aorta were associated with a significant p-value (0.03 and 0.02 respectively). CONCLUSION: Parental smoking in early pregnancy can be considered a risk factor for valvar pulmonary stenosis and coarctation of the aorta. Cessation of smoking by the parents before pregnancy could reduce the risk of their occurrence.

[Epidemiology of mitral valve disease in pediatrics: a Lebanese study]. / Epidémiologie des pathologies mitrales en pédiatrie: étude libanaise.

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. Among them, 128 (4.9%) had mitral disease. The causes were congenital in 65% of the cases (28% were associated with other congenital heart anomalies, especially a double outlet right ventricle) and acquired in 35% of the cases (91% were secondary to rheumatic fever). The diagnosis of the disease was fortuitous in 24% of the cases, and a murmur was discovered on auscultation in 53% of the cases. The majority of children showed mitral regurgitation (86%). The regurgitation was severe in 12% of the cases. Half of the children received medical or surgical treatment. The mortality of this disease reached 2%. CONCLUSION: Congenital anomalies of the mitral valve are the most common cause of mitral regurgitation in children. Rheumatic disease remains another important cause of mitral disease in Lebanon. A high index of suspicion must exist, since the vast majority of diseases are found fortuitously or during simple auscultation.

[Atrioventricular septal defect characteristics in infants with and without Down's syndrome: a Lebanese study]. / Caractéristiques du canal atrioventriculaire chez les enfants avec et sans syndrome de Down: étude libanaise.

OBJECTIVES: 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. The gathered data included age, sex, type of AVSD, mother's age, 1st and 2nd degree cousins, and other associated cardiac or extra-cardiac anomalies. RESULTS: AVSD was diagnosed in 5.5% of all patients with congenital heart disease, with 81.7% (n = 98) being complete AVSD. Male sex was predominant (58%). More than half (57.5%) were also diagnosed with Down's syndrome. The mean maternal age was 30.4 years (+/- 4.7 years) and consanguinity found in 16.7% of the cases. Cardiac and extra-cardiac anomalies (all in the esophagus and intestine) were associated in 15% and 6.7% respectively. Complete AVSD was significantly associated with Down's syndrome: 94% of patients with Down's syndrome had a complete AVSD. Digestive anomalies were also significantly more frequent with Down's syndrome (10% versus 2%, p = 0.02). Other cardiac anomalies, however, were less frequent with Down's syndrome (33% versus 7.7%, p = 0.02). CONCLUSION: Down's syndrome is more frequently associated with isolated and complete AVSD. Other anomalies may complicate the management of these patients. The cause of this probable genetic anomaly is still debated.

[Congenital heart disease, maternal age and parental consanguinity in children with Down's syndrome]. / Cardiopathie congenitale, age maternel et consanguinite parentale chez les enfants avec syndrome de Down.

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. 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. RESULTS: 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. CONCLUSION: 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.