Complete atrioventricular septal defect: Impact of Down syndrome on atrioventricular valve morphology and haemodynamics and clinical outcomes - 09/01/21
Résumé |
Background |
Complete atrioventricular septal defect (CAVSD) is a common congenital heart disease among children with Donw syndrome
Purpose |
Investigate the impact of Down syndrome on atrioventricular valve morphology and haemodynamics as well as on outcomes in children with CAVSD.
Methods |
We performed a single-centre, retrospective, analytic study with a prospective follow-up in the Congenital Heart Disease Unit of cardiology department over a 9-year period (from December 2008 to September 2017).We compared 2 groups of patients with CAVSD: Down syndrome patients (group 1) and non-Down syndrome patients (group 2).
Results |
Among the 2778 patients registered in our CHD Unit during the study period, 225 (8%) were diagnosed with CAVSD, 121 (54%) in group 1 and 104 (46%) in group 2. Median age at diagnosis was 5 months [0–17 years] in group 1 versus 5 months [1month–10 years] in group 2. The situs was solitus in all group 1 patients. However, in group 2, the situs was inversus in 4 patients (3.8%) and ambiguous in 17 (16.3%) (P<0.001). Ventricles were unbalanced in 4 patients (3.3%) in group 1 versus 30patients (28.8%)in group 2 (P<0.001). Atrioventricular valve annulus was hypoplastic in 6 patients (5.8%) of group 2 whereas all patients in group 1 had a normally developed atrioventricular valve annulus (P=0.024). Papillary muscles were abnormal in 8 patients (6.6%) in group 1 versus 15 patients (14.4%) of group 2 (P=0.048). Significant preoperative left atrioventricular valve regurgitation was lower in group 1 patients: 11 (9.1%) versus 23 (22.1%) (P=0.02). Median age at surgery was 6 months [1month–7 years] in group 1 versus 7 months [1month–11 years] in group 2 (P=0.023). Mortality rate was lower in group 1; 14 (11.5%) versus 23 (22.1%) (P=0.03).
Conclusion |
Down syndrome patients have a better atrioventricular valve anatomy, fewer preoperative left atrioventricular valve regurgitation and a lower long-term mortality rate after surgery.
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Vol 13 - N° 1
P. 138-139 - janvier 2021 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.