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[Linear insertion of atrioventricular valves in children with and without Down's syndrome]
Insights
Linear insertion of the atrioventricular valves (LIAVV) is more common in males and children with Down
Area of Science:
- Cardiovascular Medicine
- Pediatric Cardiology
- Genetics
Context:
- A cohort of 6240 pediatric patients (0-16 years) with and without congenital heart disease (CHD) was analyzed.
- Data was collected over six years (2005-2011) from the Lebanese National Registry of Pediatric and Congenital Heart Disease.
- Patients were categorized into two groups: those with Down's syndrome (DS) and those without.
Purpose:
- To investigate the parameters of linear insertion of the atrioventricular valves (LIAVV).
- To compare the incidence and characteristics of LIAVV in pediatric patients with and without Down's syndrome (DS).
- To identify associated cardiac anomalies and sex distribution in LIAVV cases.
Summary:
- LIAVV was identified in 20% of pediatric patients with DS (31/155) and 0.2% without DS (14/5995), with a highly significant p-value (<0.00001).
- A marked male predominance was observed for LIAVV, particularly in isolated cases (86% males).
- Family history of DS was noted in some children without DS presenting with isolated LIAVV.
Impact:
- This study highlights the increased prevalence of LIAVV in pediatric patients with Down's syndrome.
- Findings emphasize the importance of considering LIAVV in male pediatric patients, especially those with DS or a family history of DS.
- The research contributes to understanding the spectrum of congenital heart disease in pediatric populations.
Objective:
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 (1.07.2005-30.06.2011) in the National Registry of the Pediatric and Congenital Heart Disease of the Society of Cardiology in Lebanon.
Method:
Children were divided in two groups; Group I: children with DS and group II: children without DS. In the two groups, the frequency of LIAVV whether isolated or associated with other CHD (excluding the atrioventricular canal), the distribution boy-girl and the various associated cardiac anomalies were studied. We used nonparametric tests for comparing the two groups. The p value < 0.05 was considered significant.
Results:
Group I consists of 155 patients including 31 (20%) with LIAVV, isolated in 22 cases (14.2%) and associated with CHD in 9 cases (5.8%). Group II was composed of 5995 patients; the incidence of LIAVV was 0.2% (14 patients) with apvalue < 0.00001. Regarding sex, there was a marked male predominance: respectively 78% and 56% in groups I and II. In patients with isolated LIAVV, 86% of patients were males. Family history of DS is found in 3/7 children without T21 with isolated LIAVV. A patient, in whom an atrial septal defect ostium primum type with LIAVV was diagnosed during the first month of life, has seen his septal defect closed spontaneously five months later.
Conclusion:
Encountered predominantly among a male population, the isolated LIAVV is less frequent in children without DS but often with family history of Down's syndrom.
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