Table 1.
Down syndrome | 22q11 deletion | Heterotaxy syndrome | Turner syndrome | |||||
---|---|---|---|---|---|---|---|---|
Lesion/operation | Early mortality | LOS | Early mortality | LOS | Early mortality | LOS | Early mortality | LOS |
All cardiac surgery | Low37–39,71,* | Low71 | – | – | High129,130 | High130 | – | – |
Septal defects | ||||||||
AVSD | Medium37,39–46,48,** | Low42 | – | – | – | – | – | – |
VSD | Low37,41,* | High39,65 | – | – | – | – | – | – |
SV lesions | ||||||||
Stage 1 palliation | High37,39 | – | – | – | High129 | – | High25 | – |
Stage 2 palliation | High37,39 | High39 | – | – | – | – | – | – |
Stage 3 palliation | Medium37,39,50,51 | High51 | – | – | Medium124,129 | – | – | – |
Conotruncal defects | ||||||||
Collective | Low31,33 | – | Medium31,33,88 | Low88 | – | – | – | – |
TOF | Low32,37,39,41,49 | High39 | Low32,86 | – | – | – | – | – |
PA-VSD | – | – | High91–93 | – | – | – | – | – |
IAA or PTA | – | – | Low87 | High87 | – | – | – | – |
Other | ||||||||
CoA | Low37 | – | – | – | – | Low160 | High160 | |
Cardiac transplantation | – | – | – | – | Medium137,138 | – | – | – |
TAPVR | – | – | – | – | Low134 | – | – | – |
AVSD = atrioventricular septal defect; CoA = coarctation of the aorta; IAA = interrupted aortic arch; LOS = length of stay (in-hospital); PA-VSD = pulmonary atresia with ventricular septal defect; PTA = persistent truncus arteriosus; SV = single ventricle; TAPVR = total anomalous pulmonary venous return; TOF = tetralogy of Fallot; and VSD = ventricular septal defect
Classification of risk for poor early post-operative outcomes relative to patients without the syndromic diagnosis (high: studies reviewed only demonstrating increased mortality or LOS, medium: studies demonstrating increased or no difference in mortality or LOS, low: studies demonstrating no difference in mortality or LOS)
Some studies reported decreased mortality
Studies reported increased mortality, decreased mortality, and no difference in mortality