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Pediatr Cardiol. 2017 Dec;38(8):1680-1685. doi: 10.1007/s00246-017-1713-7. Epub 2017 Sep 22.

The Impact of 22q11.2 Microdeletion on Cardiac Surgery Postoperative Outcome.

Author information

1
Faculty of Medicine, University of Belgrade, Dr Subotica 8, 11000, Belgrade, Serbia. udkgenetika@udk.bg.ac.rs.
2
Department of Medical Genetics, University Children's Hospital, Tirsova 10, Belgrade, Serbia. udkgenetika@udk.bg.ac.rs.
3
Institute of Molecular Genetics and Genetic Engineering, University of Belgrade, Vojvode Stepe 444a, Belgrade, Serbia.
4
Faculty of Medicine, University of Belgrade, Dr Subotica 8, 11000, Belgrade, Serbia.
5
Department of Cardiology, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
6
Department of Cardiac Surgery, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
7
Department of Pediatric Intensive Care, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
8
Department of Neonatology, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
9
Department of Hematology, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
10
Department of Medical Genetics, University Children's Hospital, Tirsova 10, Belgrade, Serbia.

Abstract

22q11.2 microdeletion is the most common microdeletion in humans. The purpose of this study was to evaluate postoperative outcome in children with 22q11.2 microdeletion who had undergone complete surgical correction of a congenital heart defect. The study included 34 patients who underwent complete correction of conotruncal heart defects. Of these, 17 patients diagnosed with 22q11.2 microdeletion represent the investigated group. Another 17 patients without 22q11.2 microdeletion represent the control group. Investigated and control groups differ significantly for total length of stay in the hospital (average 37.35 and 14.12 days, respectively); length of postoperative stay in the intensive care unit (average 10.82 and 6.76 days, respectively); sepsis (eight and two patients, respectively); administration of antibiotics (15 and seven patients, respectively); duration of antibiotic therapy (average 17.65 and 14.59 days, respectively); occurrence of hypocalcemia (16 and 0 patients, respectively); and initiation of peroral nutrition during the postoperative course (average 10.29 and 3.88 days, respectively). No difference was found for duration of ventilatory support (average 6.12 and 4.24 days, respectively), administration of total parenteral nutrition, and postoperative mortality rate. The study results suggest that genotype of 22q11.2 microdeletion affects postoperative outcome after cardiac surgery. Possible targets for intervention in postoperative intensive care management are prevention and treatment of systemic infections, monitoring, and treatment of hypocalcemias, rational administration of antibiotics and careful planning of nutrition. Consequently, this could shorten patients' intensive care stay and overall duration of hospitalization.

KEYWORDS:

22q11.2 deletion syndrome; Congenital heart defect; Intensive care; Pediatrics; Postoperative care

PMID:
28940032
DOI:
10.1007/s00246-017-1713-7
[Indexed for MEDLINE]

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