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Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative complications?


Doell, C; Bernet, V; Molinari, L; Beck, I; Balmer, C; Latal, B (2011). Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative complications? Pediatric Critical Care Medicine, 12(5):539-544.

Abstract

BJECTIVES:

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.
DESIGN:

Prospective cohort study.
SETTING:

Tertiary university children's hospital.
PATIENTS:

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.
INTERVENTIONS:

None.
MEASUREMENTS AND MAIN 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.

Abstract

BJECTIVES:

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.
DESIGN:

Prospective cohort study.
SETTING:

Tertiary university children's hospital.
PATIENTS:

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.
INTERVENTIONS:

None.
MEASUREMENTS AND MAIN 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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2011
Deposited On:11 Jan 2012 14:15
Last Modified:05 Apr 2016 15:15
Publisher:Lippincott Wiliams & Wilkins
ISSN:1529-7535
Publisher DOI:https://doi.org/10.1097/PCC.0b013e3181fe4085
PubMed ID:21057364

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