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J Thorac Cardiovasc Surg 2008;135:230
© 2008 The American Association for Thoracic Surgery

Letter to the Editor

Better surgical prognosis for patients with Down syndrome

^a Pediatric Cardiology, Department of Pediatrics, University of Rome "La Sapienza", Rome, Italy
^b Pediatric Cardiology and Cardiac Surgery, Policlinico "S.Orsola-Malpighi", Bologna, Italy

To the Editor:

In a recent issue, we read the interesting paper by Seifert et al. on risk of death during hospitalization for pediatric cardiac surgery.¹ In their multicentric study, the authors unequivocally demonstrated that Down syndrome is associated with a significant lower mortality after cardiac surgery for congenital heart defects, affirming that "the effect of Down syndrome on immediate outcome is not clear."¹

Although previous studies have suggested that patients with this syndrome have an increased risk of mortality after repair of ventricular septal defect or atrioventricular canal,¹ recent papers have shown that the presence of Down syndrome did not increase the surgical mortality in children with ventricular septal defect² or with tetralogy of Fallot.³ Moreover, in patients with partial⁴ and complete⁵ atrioventricular canal (very frequent heart defects in children with trisomy 21), the Down syndrome is associated with significantly lower mortality and morbidity after cardiac surgery.

These surgical results are probably due to the peculiar anatomic cardiac pattern of patients with atrioventricular canal and Down syndrome, including a lower prevalence of left-sided obstructions, right ventricular dominance, and additional anomalies of the mitral valve.⁶ We can suggest that the effect of best results on children with atrioventricular canal^4,5 could influence all surgical results in patients with Down syndrome.¹

In conclusion, in spite of the tendency to infections and early pulmonary hypertension in children with Down syndrome, cardiac surgery not only is not contraindicated, as previously suggested, but can be performed with very good results, as clearly shown by Seifert et al.¹

References

1. Seifert HA, Howard DL, Silbert JH, Jobes DR. Female gender increases the risk of death during hospitalization for pediatric cardiac surgery. J Thorac Cardiovasc Surg 2007;133:668-675.[Abstract/Free Full Text]
   
2. Knott-Craig CJ, Elkins RC, Ramakrishnan K, Hartnett DA, Lane MM, Overholt ED, et al. Associated atrial septal defects increase perioperative morbidity after ventricular septal defect repair in infancy. Ann Thorac Surg 1995;59:573-578.[Abstract/Free Full Text]
   
3. Michielon G, Marino B, Formigari R, Gargiulo G, Picchio F, Digilio MC, et al. Genetic syndromes and outcome after surgical correction of tetralogy of Fallot. Ann Thorac Surg 2006;81:968-975.[Abstract/Free Full Text]
   
4. Giamberti A, Marino B, di Carlo D, Iorio FS, Formigari R, de Zorzi A, et al. 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]
   
5. Formigari R, Di Donato RM, Gargiulo G, Di Carlo D, Feltri C, Picchio FM, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down’s syndrome. Ann Thorac Surg 2004;78:666-672discussion 672. Review.[Abstract/Free Full Text]
   
6. 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;91:467-469.[Abstract]
   

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Roberto Formigari Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Saffirio, C. Articles by Formigari, R. Search for Related Content PubMed Articles by Saffirio, C. Articles by Formigari, R.