Correction of atrioventricular septal defect. Results influenced by Down syndrome?
T. W. Vet and J. Ottenkamp
Department of Pediatric Cardiology, University Hospital Leiden, The Netherlands.
The role of Down syndrome (DS) in the outcome of the surgical correction of
atrioventricular septal defect (AVSD) was investigated by the analysis of
clinical events among 85 patients. Complete AVSD (cAVSD) was present in 49
patients, of whom 36 (73%) had DS. Thirty patients (83%) survived surgical
treatment. Of the 13 patients without DS, 7 (54%) survived. There were four
deaths and one late death. Thirty-six patients had partial AVSD (pAVSD), 5
(14%) of whom had DS; all 5 patients are still alive. Thirty (97%) of the
31 patients with pAVSD without DS survived the operation. There were two
late deaths. Preoperative selection did not account for the favorable
results in children with DS. All early postoperative deaths in cAVSD
occurred in children younger than 2 years; low body weight for age seemed
to be an important factor in adverse outcome. A large number of patients
without DS had other serious congenital malformations. In view of the
relatively favorable results in patients with DS, it does not seem
warranted to maintain a conservative diagnostic and therapeutic approach of
AVSD solely because of concomitant DS.