Infection in DiGeorge Following CHD Surgery


We propose a retrospective review of patients with DiGeorge syndrome having undergone cardiac surgery to evaluate the incidence of blood stream and/or surgical site infection. The hypothesis is that we will find an increased number of infections for this sub-group. We will compare the incidence of infection to children of similar age and diagnosis to evaluate for variances in the incidence of infection.

Full Title of Study: “Incidence of Infection in the Patient With DiGeorge Syndrome Following Surgery for Congenital Heart Disease”

Study Type

  • Study Type: Observational
  • Study Design
    • Time Perspective: Retrospective
  • Study Primary Completion Date: March 2008

Detailed Description

DiGeorge syndrome is a common genetic disorder that frequently results in congenital heart defects such as truncus arteriousus, coarctation of the aorta, interrupted aortic arch, tetralogy of Fallot, pulmonary atresia with VSD, and several others. The defect is usually due to a deletion in the long arm of chromosome 22. Approximately 25% of patients with DiGeorge have a congenital heart defect. These patients also have varying degrees of thymic hypoplasia with associated T cell dysfunction. They are at increased risk of infections and can be at risk for opportunistic infections when the degree of T cell dysfunction is severe. Children undergoing cardiac surgery are at risk for infections in the post-operative period, prolonging hospital stay and increasing morbidity and mortality. The patient with DiGeorge syndrome may have higher rates of infection due to associated immune system dysfunction, however, this has not been previously reported from a large group of DiGeorge syndrome patients. Children's Healthcare of Atlanta follows over 200 patients with DiGeorge syndrome, with the majority having previously undergone cardiac surgery. This group of patients may benefit from more extensive antibiotic prophylaxis following surgery if they indeed have significantly higher rates of infection, but the true incidence needs to be determined.

Participating in This Clinical Trial

Inclusion Criteria

  • Children's Healthcare of Atlanta patients, Egleston DiGeorge Syndrome Cardiac Surgery 200 medical charts between Jan 1, 1998 and April 31, 2005 with cardiac surgery and DiGeorge Syndrome 200 medical charts between Jan 1, 1998 and April 31,2005 with cardiac surgery and no DiGeorge Syndrome Exclusion Criteria:

  • Those who do not meet inclusion criteria

Gender Eligibility: All

Minimum Age: N/A

Maximum Age: 15 Years

Are Healthy Volunteers Accepted: No

Investigator Details

  • Lead Sponsor
    • Children’s Healthcare of Atlanta
  • Provider of Information About this Clinical Study
    • Sponsor
  • Overall Official(s)
    • Kevin O Maher, MD, Principal Investigator, Children’s Healthcare of Atlanta, Sibley Heart Center Cardiology

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