PEDIATRICS Vol. 108 No. 1 July 2001, pp. 217

Age at Operation for Children With Atrioventricular Canal

To the Editor.

We read the interesting paper by Chang et al.¹

Among 177 patients with atrioventricular canal (AVC), the authors report on a significantly younger mean age at surgery for children with Down syndrome compared with patients without trisomy 21. In the "Discussion" section the authors explain their data on the basis of the effort to prevent the early occurrence of permanent pulmonary vascular changes in children with Down syndrome.² Unfortunately, the authors do not report how many of their patients presented the complete or the partial forms of AVC. This data could be useful in understanding the reason for early surgery in children with Down syndrome because it is well-known that complete AVC is prevalent in Down syndrome patients and partial AVC is prevalent in children without Down syndrome.³

In our recent experience (1996-1999) with 144 children undergoing primary repair of AVC, 100 presented a complete form (68% Down syndrome, mean age at surgery: 8.3 months) and 44 present a partial form (38% Down syndrome, mean age at surgery: 25 months).

Different ages at surgery for complete and partial AVC were also reported in the largest surgical series. Hanley et al⁴ reviewed the results of surgical repair of complete AVC, reporting a high percentage of children with Down syndrome (77%) and a median age of their patients of 6.8 months.

On the contrary, El-Najdawi et al⁵ reported surgical results of partial AVC, and, in the last 20 years, they noticed a minority of patients with Down syndrome (5%), with a median age of their cases of 7.2 years.

In conclusion, early surgery for patients with AVC and Down syndrome is justified not only by the attempt to prevent pulmonary vascular changes but also by different prevalence of the anatomic forms. Children with Down syndrome have a prevalence of complete AVC associated with early congestive heart failure and need early repair.

On the contrary, children with AVC without Down syndrome have a prevalence of partial AVC rarely associated with early congestive heart failure,⁶ and its surgical correction can usually be performed after the second year of life.

Bruno Marino, MD
Silvia Anaclerio, MD
Roberto Di Donato, MD
Department of Pediatric Cardiology and Cardiac Surgery
Bambino Gesù HospitalP.zza S. Onofrio 4
00165 Rome, Italy

REFERENCES

1. Chang RKR, Chen AY, Klitzner TS Factors associated with age at operation for children with congenital heart disease. Pediatrics. 2000; 105:1073-1081 [Abstract/Free Full Text]
2. Yamaki S, Yasui H, Kado H, Pulmonary vascular disease and operative indications in complete atrioventricular canal defects in early infancy. J Thorac Cardiovascular Surg. 1993; 106:398-405 [Abstract]
3. Marino B, Vairo U, Corno A, Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome. Am J Dis Child. 1990; 144:1120-1122 [Abstract/Free Full Text]
4. Hanley FL, Fenton KN, Jonas RA, Surgical repair of complete atrioventricular canal defect in infancy: twenty-years trends. J Thorac Cardiovasc Surg. 1993; 106:387-394 [Abstract]
5. El-Najdawi EK, Driscoll D, Puga FJ, Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovas Surg. 2000; 119:880-890 [Abstract/Free Full Text]
6. Giamberti A, Marino B, Di Carlo D, Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options. Ann Thorac Surg. 1996; 62:151-154 [Abstract/Free Full Text]

In Reply.

We appreciate the important comments from Marino et al regarding our article on age at operation for children with congenital heart disease.¹ We agree that children with the complete form of atrioventricular canal (AVC) defect have different pathophysiology from children with partial AVC and therefore require surgical repair at different ages. In our study, all patients with AVC were selected based on International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) principal diagnosis code of 745.69, which is used for coding the complete form of AVC. We eliminated all patients with ICD-9-CM principal diagnosis code of 745.61 for ostium primum defect, which indicates partial AVC. Therefore, we believe that our series of 177 children with AVC represents only the complete form AVC.

In the "Results" section we reported that 95 children with AVC (or 54%) had a diagnosis of Down syndrome. The proportion of children with AVC and Down syndrome in our series is comparable to the experience of Marino et al (68%), and their ages at operation are also similar (mean age of 10 months and 8.3 months, respectively). We share the view of Marino et al that it is important to make the distinction between complete and partial forms of AVC. Because our series included only patients with complete form AVC, we feel that our argument is valid that children with Down syndrome and AVC be operated on earlier to prevent irreversible pulmonary vascular changes.

Ruey-Kang R. Chang, MD, MPH
Division of Cardiology
Department of Pediatrics
Harbor-UCLA Medical Center
Torrance, CA 90509

Thomas S. Klitzner, MD, PhD
Department of Pediatrics
UCLA School of Medicine
Los Angeles, CA

REFERENCE

1. Chang R-KR, Chen AY, Klitzner TS Factors associated with age at operation for children with congenital heart disease. Pediatrics. 2000; 105:1073-1081