Cardiac transplantation in children with Down syndrome, Turner syndrome, and other chromosomal anomalies: A multi-institutional outcomes analysis
Section snippets
Data source
Data for this study were obtained from the PHIS database, an administrative database that contains inpatient, emergency department, ambulatory surgery, and observation data from 48 not-for-profit, tertiary-care pediatric hospitals in the United States. These hospitals are affiliated with the Children’s Hospital Association (Overland Park, KS), a business alliance of children’s hospitals. Data quality and reliability are assured through a joint effort between the Children’s Hospital Association
Results
Between January 2004 and March 2016, 3,080 hospitalizations across 30 hospitals were identified in which an OHT was performed. A total of 64 (2.1%) OHTs were performed in patients with a concomitant diagnosis of CA. Table 1 demonstrates the demographic comparison between the CA and no-CA transplant recipients. Both gender and OHT within 24 hours of admission were statistically different between the patients with and without CA. Patients’ demographics by CA type are listed in Table 2. Although
Discussion
To our knowledge, incidence and outcomes of cardiac transplantation in pediatric patients with CA have not been previously described in the literature. Our data demonstrate that cardiac transplantation in individuals with CA appears to be a relatively rare phenomenon as only ~2% of patients who received OHT also had a diagnosis of CA. Furthermore, OHT patients with CA generally have similar rates of mortality when compared to patients without CA. Our data indicate a relatively higher risk of
Conclusions
In conclusion, cardiac transplantation in pediatric patients with CA is a rare phenomenon, accounting for ~2% of OHTs. Although the sample size of the patients at risk is small, the presence of CA does not appear to confer a significantly higher risk for mortality for a child undergoing OHT (OR 1.2 [0.5 to 3.2]; p = 0.651) and may not portend a worse long-term outcome in patients deemed to be acceptable transplant candidates. This study was not powered to detect statistical differences in
Disclosure statement
The authors have no conflicts of interest to disclose.
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