Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative complications?
OBJECTIVE
Children with congenital heart disease and genetic disorders may be at increased risk for postoperative mortality and morbidity compared with children with congenital heart disease alone. The aim of the present study was to determine differences in postcardiopulmonary bypass outcome between these two groups.


METHODS
Prospective cohort study.


METHODS
Tertiary university children's hospital.


METHODS
We enrolled 211 infants (<1 yr) who underwent bypass surgery for congenital heart disease. Data on perioperative course were compared between infants with and without genetic disorders. Univariate analysis was followed by regression analysis to control for confounders.


METHODS
None.


RESULTS
We enrolled 148 infants without and 63 infants with a genetic disorder. The majority of infants with genetic disorders had trisomy 21 (n = 32), six had microdeletion 22q11, and 25 had other genetic disorders. There was no significant difference in mortality between infants with and without genetic disorders. An underlying genetic disorder was an independent risk factor for renal insufficiency (p = .003) and reintubation (p = .02). Trisomy 21 was an independent risk factor for chylothorax (p = .01) and sepsis (p = .05). The length of hospital stay was longer in infants with genetic disorders other than trisomy 21 compared with infants with trisomy 21 (p = .009).


CONCLUSIONS
Infants with congenital heart disease and genetic disorders are not at increased risk for postoperative mortality. However, a genetic disorder is a risk factor for reintubation and renal insufficiency, whereas infants with trisomy 21 have a higher risk of chylothorax and sepsis. Intensive care providers need to be aware of these differences in morbidity to improve management decisions and parental counseling.
Pediatric Intensive Care and Neonatology, University Children's Hospital, Zurich, Switzerland.
Carsten
C
Doell
Vera
V
Bernet
Luciano
L
Molinari
Ingrid
I
Beck
Christian
C
Balmer
Beatrice
B
Latal
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000209:etiology*; D011446:Prospective Studies; D018570:Risk Assessment; D013557:Switzerland