Literature DB >> 21057364

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

Carsten Doell1, Vera Bernet, Luciano Molinari, Ingrid Beck, Christian Balmer, Beatrice Latal.   

Abstract

OBJECTIVES: 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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Mesh:

Year:  2011        PMID: 21057364     DOI: 10.1097/PCC.0b013e3181fe4085

Source DB:  PubMed          Journal:  Pediatr Crit Care Med        ISSN: 1529-7535            Impact factor:   3.624


  8 in total

Review 1.  CHD associated with syndromic diagnoses: peri-operative risk factors and early outcomes.

Authors:  Benjamin J Landis; David S Cooper; Robert B Hinton
Journal:  Cardiol Young       Date:  2015-09-08       Impact factor: 1.093

2.  22q11.2 Deletion Status and Perioperative Outcomes for Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collateral Vessels.

Authors:  Laura Mercer-Rosa; Okan U Elci; Nelangi M Pinto; Ronn E Tanel; Elizabeth Goldmuntz
Journal:  Pediatr Cardiol       Date:  2018-03-08       Impact factor: 1.655

3.  Sepsis in the pediatric cardiac intensive care unit.

Authors:  Derek S Wheeler; Howard E Jeffries; Jerry J Zimmerman; Hector R Wong; Joseph A Carcillo
Journal:  World J Pediatr Congenit Heart Surg       Date:  2011-07-01

4.  22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.

Authors:  Laura Mercer-Rosa; Nelangi Pinto; Wei Yang; Ronn Tanel; Elizabeth Goldmuntz
Journal:  J Thorac Cardiovasc Surg       Date:  2013-01-11       Impact factor: 5.209

5.  Case fatality rate and associated factors in patients with 22q11 microdeletion syndrome: a retrospective cohort study.

Authors:  Gabriela M Repetto; M Luisa Guzmán; Iris Delgado; Hugo Loyola; Mirta Palomares; Guillermo Lay-Son; Cecilia Vial; Felipe Benavides; Karena Espinoza; Patricia Alvarez
Journal:  BMJ Open       Date:  2014-11-06       Impact factor: 2.692

6.  Pattern of congenital heart disease among children presenting to the Uganda Heart Institute, Mulago Hospital: a 7-year review.

Authors:  Judith Namuyonga; Sulaiman Lubega; Twalib Aliku; John Omagino; Craig Sable; Peter Lwabi
Journal:  Afr Health Sci       Date:  2020-06       Impact factor: 0.927

Review 7.  Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease.

Authors:  Benjamin Kelly; Sheyanth Mohanakumar; Vibeke Elisabeth Hjortdal
Journal:  Curr Cardiol Rep       Date:  2020-10-10       Impact factor: 2.931

8.  Beyond the Syndrome: Extensive Congenital Abnormalities in an Infant With Trisomy 21.

Authors:  Jeremy D Ward; Mahesh S Sharma; Matthew F Pizzuto; Vincent J Moylan; Frederic B Askin; David G Kaufman
Journal:  Clin Pathol       Date:  2022-04-22
  8 in total

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