Congenital Heart Disease
Trends in Pulmonary Valve Replacement in Children and Adults With Tetralogy of Fallot

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Operative correction of tetralogy of Fallot frequently results in pulmonary insufficiency and chronic volume overload, which have been linked to increased risk for adverse outcomes. No consensus recommendations for the timing of pulmonary valve replacement (PVR) exist. The aim of this study was to examine the pattern of PVR in the United States from 2004 to 2012. The Pediatric Health Information Systems database was used to perform an observational study of children and adults ≥10 years of age with diagnoses of tetralogy of Fallot who underwent PVR at 35 centers in the United States from 2004 and 2012, to assess the rate of PVR and the age at which is performed. Mixed-effects multivariate regression was used to account for patient-level covariates and center-level covariance. Additional analyses assessed for trends in cost, hospital length of stay (LOS), intensive care unit LOS, and in-hospital mortality over the study period. In total, 799 subjects at 35 centers underwent PVR over the study period. The number of PVRs performed per year increased significantly over the study period. There was significant between-center heterogeneity in age at PVR (p <0.001). Age at PVR, intensive care unit LOS, hospital LOS, and cost did not change over the study period. In conclusion, PVR in patients with tetralogy of Fallot is being performed more frequently, without an accompanying change in the age at PVR or other measurable outcomes. There is significant variability in the age at which PVR is performed among centers across the United States. This highlights the need for additional research guiding the optimal timing of PVR.

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Methods

The Pediatric Health Information Systems (PHIS) database is an administrative database that contains data from inpatient, emergency department, ambulatory surgery, and observation encounters from 43 not-for-profit, tertiary care pediatric hospitals in the United States, affiliated with the Children’s Hospital Association (Overland Park, Kansas).1 Data quality and reliability are assured through a joint effort between the Children’s Hospital Association and participating hospitals. The PHIS data

Results

From 2004 to 2012, a total of 799 subjects with TOF underwent PVR at 35 centers across the United States contributing data to the PHIS database (Table 1). Eight centers (10 subjects) did not meet the inclusion criteria and were excluded. Median age at PVR was 17 years (range 10 to 64). Most of the population was male (57%) and white (75%). A small minority (10%) carried diagnoses of genetic syndromes. Median hospital LOS was 4 days (range 1 to 126), with a median duration of ICU stay 2 days

Discussion

In this observational study from 35 children’s hospitals in the United States, the annual number of PVRs in children and adults with TOF increased significantly over 9 years. Although the age of subjects who underwent PVR did not change significantly, there was also significant heterogeneity among centers in the age at which patients underwent PVR. Older age was associated with increased ICU duration and cost. Presence of a genetic syndrome was associated with younger age at PVR, longer

Acknowledgment

The authors thank Zeinab Mohammad for assistance in data extraction and A. Russell Localio, PhD, for consultation during statistical analysis.

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Dr. O’Byrne receives support from the National Institutes of Health (NIH) (Grant T32 HL007915) and an Entelligence Young Investigator grant. Dr. Mercer-Rosa receives support from the NIH (Grant NIH 3U01HL098153-03S1). Dr. Kawut is supported by the NIH (Grant K24 HL103844).

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