Compleet atrioventriculair septumdefect bij kinderen met het syndroom van Down: goede resultaten van chirurgische correctie op steeds jongere leeftijd

To evaluate the results of cardiosurgical treatment of children with Down’s syndrome and a complete atrioventricular septal defect (cAVSD).

Design

Retrospective.

Method

Data were collected from the records of all patients with Down’s syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, the Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality > 30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years.

Results

The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration of follow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19) died. The mortality within 30 days after the operation decreased from 0-38 in the period 1980-1989 via 0-30 in the period 1990-1999 to 0 in 2000-2003. The percentage of reoperations related to the correction of cAVSD (14; 14/98) did not seem to increase. Of the correction-related reoperations, 14 (2/14) were followed by a second reoperation.

Conclusion

In the period 1980-2003, children with Down’s syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0 in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down’s syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.

Ned Tijdschr Geneeskd 2005;149:589-93