Congenital Heart Surgery Outcomes in Down Syndrome: Analysis of a National Clinical Database

Abstract

Background Contemporary outcomes data in Down Syndrome (DS) patients undergoing congenital heart surgery are limited. We describe patient characteristics and post-operative morbidity and mortality in a large multi-center cohort. Patients and Methods This retrospective cohort study utilized The Society of Thoracic Surgeons Congenital Heart Surgery Database to compare patient characteristics and postoperative outcomes in DS and non-DS patients (0–18y) undergoing surgery from 2000–2008. Primary analysis focused on the most common surgeries performed in DS patients. Results A total of 45,579 patients (n=4,350 DS, 41,229 non-DS) were included: median age 7m [interquartile range (IQR) 47d–4yr]; 56% male. DS patients were younger at surgery, with the exception of those undergoing tetralogy of Fallot (TOF) and atrioventricular septal defect (AVSD) repair. Mortality did not differ significantly in DS vs. non-DS patients. Length of stay was prolonged in DS patients undergoing atrial septal defect [4d (IQR 3–5d) vs. 3d (IQR 2–4d), p<0.0001] and ventricular septal defect (VSD) closure [5d (IQR 4–8d) vs. 4d (IQR 3–6d), p<0.0001], and TOF repair [7d (IQR 5–10d) vs. 6d (IQR 5–9d), p<0.001], and was associated with post-operative respiratory and infectious complications. DS patients undergoing VSD closure also had a higher rate of heart block requiring pacemaker placement (2.9% vs. 0.8%, p<0.0001). Conclusion In this large contemporary cohort undergoing congenital heart surgery, DS does not confer a significant mortality risk for the most common operations performed in this population; however post-operative morbidity remains common.

Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease

“…[11][12][13]23 Surprisingly, little is known about the outcomes of children with DS after surgical correction of congenital cardiac defects other than an AVSD. 24 An enhanced understanding of the association between DS and survival across a broad spectrum of lesions may improve our ability to optimize the care of these infants and children.…”

Atrioventricular Septal Defect Prognosis for Patients With Down Syndrome

“…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][2][3][4][5][6][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.…”

El niño con Síndrome de Down en la Unidad de Cuidados Intensivos

“…Esta temática se encuentra fuera del objetivo de esta actualización, no obstante, el lector puede encontrar revisiones sobre esta materia 9,36,37 .…”

“…El riesgo de mortalidad por sepsis en estos pacientes aumenta un 30% tras corrección de factores de confusión 8 . Asimismo, las complicaciones respiratorias e infecciosas post-cardiocirugía son más comunes resultando en estadías más prolongadas en la unidad de cuidados intensivos (UCI) 9 .…”