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[Complete atrioventricular septal defect. Anatomo-functional correlation between patients with and without Down's
L M Ferrín1, E Atik, N M Ikari
1Instituto do Coração do Hospital das Clínicas, FMUSP.
Arquivos Brasileiros De Cardiologia
|July 1, 1997
Summary
Children with Down syndrome and complete atrioventricular canal (CAVC) show pulmonary vascular hyperreactivity, while those without Down syndrome exhibit more cardiac insufficiency signs. This impacts their clinical course and surgical outcomes.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Genetics
Background:
- Complete atrioventricular canal (CAVC) is a congenital heart defect.
- Down syndrome is associated with an increased incidence of congenital heart defects, including CAVC.
- Understanding the clinical differences between children with and without Down syndrome who have CAVC is crucial for management.
Purpose of the Study:
- To compare the clinical course, causes, and symptom onset in children with complete atrioventricular canal (CAVC) with and without Down syndrome.
- To evaluate differences in functional class, clinical repercussions, and anatomical variations.
Main Methods:
- Retrospective review of 80 patients under 2 years of age with CAVC.
- Patients were divided into two groups: Group I (GI) with Down syndrome (n=55) and Group II (GII) without Down syndrome (n=25).
- Evaluated age at symptom onset, functional class (NYHA), clinical repercussions (congestive heart failure, pulmonary hypertension), and anatomical variations.
Main Results:
- Mean age at symptom onset was similar in both groups (50 +/- 75 days).
- Higher rates of severe functional class (III-IV NYHA) were observed in the group without Down syndrome (76%) compared to the group with Down syndrome (56.5%).
- Children with Down syndrome showed higher prevalence of pulmonary hypertension (38% vs 16%) and elevated mean pulmonary arterial pressure (68% vs 35%), suggesting pulmonary vascular hyperreactivity. Conversely, the group without Down syndrome had more signs of congestive heart failure (84% vs 62%) and more severe valvar lesions (38% vs 8%).
Conclusions:
- Children with Down syndrome and CAVC exhibit a predominance of pulmonary vascular hyperreactivity.
- Children with CAVC and normal genetics present with more signs of cardiac insufficiency.
- These differences influence the clinical course and may necessitate tailored management strategies.