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J Thorac Cardiovasc Surg 1994;107:1368-1369
© 1994 Mosby, Inc.

LETTERS TO THE EDITOR

Atrioventricular canal with Down syndrome or normal chromosomes: Distinct prognosis with surgical management?

Dipartimento di Cardiologia Pediatrica
Ospedale Bambino Gesù
Rome, Italy

To the Editor:

In their interesting article, Rizzoli and colleagues ¹ suggest that Down syndrome is not an incremental risk factor per se in the surgical treatment of atrioventricular canal (AVC) Similar data had been reported by Schneider and coworkers and by Vet and Ottenkamp, whereas in a recent study Morris and colleagues reached opposite conclusions (Table I).

On the basis of our clinical practice, we would agree with Rizzoli's conclusions that some characteristics specific to patients with Down syndrome, such as higher pulmonary vascular resistance, abnormal airways and lung parenchyma, and perhaps altered ventricular performance, may require special care. However, they do not seem to increase the operative risk of AVC over that observed in patients with AVC and normal chromosomal patterns.

In our opinion, a more valuable prognostic factor may be obtained from the anatomic studies of AVC in relation to Down syndrome. Our previous work on AVC malformations revealed that patients with and without Down syndrome diverge in prevalence of associated left-sided anomalies, including additional malformations of the left component of the common atrioventricular valve and left ventricular hypoplasia, which typically affect children without Down syndrome. ^2-4 Our limited experience with reoperation for left atrioventricular valve dysfunction after AVC repair indeed suggests a strong association of this event with the variable "normal chromosomal asset." Conversely, patients with Down syndrome show a more uniform morphologic type of AVC, with tetralogy of Fallot being the only prevalent associated lesion. ³

These differential anatomic features, which were authoritatively confirmed in a large epidemiologic study, ⁵ do not emerge in the article by Rizzoli and colleagues. This lack may simply descend from exclusion from analysis of patients with major associated anomalies, because the article describes the results of definitive repair of AVC.

Although it is not within our capabilities to provide a definitive answer to the diatribe that revolves around the surgical treatment of patients with Down syndrome who have AVC, we wish to reemphasize the concept that the anatomic patterns of AVC significantly differ in children with trisomy 21 and in those with normal chromosomal patterns and that the diversity is disadvantageous to the latter group. Cardiologists and surgeons should, therefore, be aware that complex anatomic types of AVC are more common in patients with normal chromosomes; these forms carry a less favorable early and mid-term prognosis. In this respect the presence of Down syndrome must not be considered an incremental risk factor for early death after repair of AVC.

References

1. Rizzoli G, Mazzucco A, Maizza F, et al. Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects? J THORAC CARDIOVASC SURG 1992;104:945-53.[Abstract]
   
2. De Biase L, Di Ciommo V, Ballerini L, Bevilacqua M, Marcelletti C, Marino B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down's syndrome. J THORAC CARDIOVASC SURG 1986;91:467-9.[Abstract]
   
3. Marino B, Vairo U, Corno A, et al. Atrioventricular canal in Down syndrome: prevalence of associated cardiac malformations compared with patients without Down syndrome. Am J Dis Child 1990;144:1120-2.[Abstract]
   
4. Marino B. Atrioventricular septal defect—anatomic characteristics in patients with and without Down's syndrome. Cardiol Young 1992;2:308-10.
   
5. Carmi R, Boughman JA, Ferencz C. Endocardial cushion defect: further studies of "isolated" versus "syndromic" occurrence. Am J Med Genet 1992;43:569-75.[Medline]
   

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