Atrioventricular Septal Defect Prognosis for Patients With Down Syndrome

Abstract

We read with interest the article by Patel et al. [8] entitled Early Outcomes and Prognostic Factors for Left Atrioventricular Valve Replacement after Primary Atrioventricular Septal Defect Repair in a recent issue of your journal. In their cohort of patients with previously repaired atrioventricular septal defect (AVSD), the authors identified Down syndrome (DS) as an important predictor of in-hospital mortality after left atrioventricular valve (LAVV) replacement. Surprisingly, the study contains no possible explanations for these data. Moreover the authors wrote that ‘‘the association between DS and survival after surgical interventions for AVSD remains unclear’’ [8]. In our opinion this sentence is not accurate and in this form could be misleading. In the recent literature, only one study of a cohort from the period 1958 to 1997 reported that infants with complete AVSD and DS at surgical correction had a higher mortality rate than infants without DS [9]. However, as previously noted [7], the reported mortality difference was primarily in the earlier years of the study [9] when infants with AVSD and DS probably received unequal cardiac care [7]. All recent contributions have clearly demonstrated that with the current level of care, DS is not a risk factor for primary surgical correction of complete AVSD [1–7]. Moreover, some studies have even suggested that DS in patients with complete AVSD may be considered a protective factor for surgical correction [1, 3, 5] and for long-term follow-up experience [1, 2] due to the more favorable cardiac anatomy. These are fundamental and established data for correct counseling in relation to fetuses, neonates, and children with DS and AVSD. For patients with DS, the higher mortality rate at valve replacement after primary correction of AVSD is new and interesting information [8], and this topic deserves further studies, additional data, and adequate explanations.