Cardiac transplantation in children with Down syndrome, Turner syndrome, and other chromosomal anomalies: A multi-institutional outcomes analysis

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Background

The purpose of this study was to describe the prevalence, characteristics, and outcomes in pediatric patients with chromosomal anomalies (CA) undergoing orthotopic heart transplantation (OHT).

Methods

A query of the database of the Pediatric Health Information System, a large administrative and billing database of 43 tertiary children’s hospitals, was performed for the Years 2004 to 2016. Pediatric patients who received OHT were analyzed based on presence and type of CA. CA analyzed included: Down syndrome (DS); Turner syndrome (TS)/gonadal dysgenesis; conditions due to anomaly of unspecified chromosome; autosomal deletion; microdeletion; and autosomal anomaly. Healthcare-associated charge analysis during hospitalization for OHT and survival after OHT were assessed.

Results

A total of 3,080 hospitalizations were identified in which OHTs were performed. Of these OHTs, 64 (2.1%) were performed in patients with a concomitant diagnosis of CA. The presence of CA did not confer a higher risk of in-hospital mortality after OHT (odds ratio 1.2 [0.5 to 3.2], p = 0.651). Differences in in-hospital mortality between different types of CA, including DS and TS, did not reach statistical significance. Survival at 1-year post-OHT was similar in patients with CA compared to those without CA (p = 0.248). Length of stay after OHT was longer in patients with CA: 76 (interquartile range [IQR] 76 to 142 days vs 49 [IQR 21 to 98] days) (p < 0.001), respectively. Overall adjusted hospital charges were significantly higher in the CA group: $1.2 million (IQR $740,000 to $2.2 million) vs $792,000 (IQR $425,000 to $1.5 million] (p < 0.001), respectively.

Conclusions

CA is present in ~2% of pediatric patients undergoing OHT. The presence of CA was not associated with increased mortality in pediatric patients undergoing OHT. Limitations of this study include the small number of patients available for analysis and a likely highly selective cohort of patients with CA.

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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