July 1990

Atrioventricular Canal Without Down Syndrome Associated With Additional Cardiac and Noncardiac Anomalies

Author Affiliations

Dipartmento Medico-Chirurgico Instituto Di Ricerca Scientifica Ospedale Pediatrico Bambino Gesu Piazza Sant 'Onofrio 40165 Rome Italy

Am J Dis Child. 1990;144(7):752. doi:10.1001/archpedi.1990.02150310018015

Sir.—We read the interesting article by Vet and Ottenkamp1 regarding the surgical correction of atrioventricular septal defect in relation to the presence of Down syndrome (DS) in the November 1989 issue of AJDC. This study confirms our previous observations on the prevalence of left-sided obstructive lesions in children with atrioventricular canal (AVC) without DS.2 Moreover, Vet and Ottenkamp report that "a large proportion of children without Down syndrome had other serious congenital anomalies." Our experience is in agreement with this observation. In particular, we

Two patients with "non-Down atrioventricular canal syndrome" with hypertelorism, epicanthus, and depressed nasal bridge. found a prevalence of phenotypic anomalies in "non-Down patients" with AVC.3 Comparing 75 children with AVC and 50 children with other types of congenital heart disease (all 125 with normal chromosomes), we found two or more phenotypic anomalies in 35 patients with AVC (46.6%) and only in 6

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