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Ann Thorac Surg. 2003 Feb;75(2):412-21.

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors.

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Department of Paediatric Cardiology, Royal Brompton and Harefield NHS Trust, NHLI, Imperial College, London, United Kingdom.



This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect.


A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%.


The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor (p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1.


The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death.

[Indexed for MEDLINE]

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