Abstract 13113: Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights From the Congenital Cardiac Research Collaborative

Introduction:Health-related quality of life (HRQOL) is impaired for patients with chronic illnesses and critical congenital heart disease (cCHD). For symptomatic neonates with tetralogy of Fallot who require neonatal intervention (sTOF), existing studies are limited but suggest early surgical management and ongoing disease burden negatively impact HRQOL. The current study aims to explore HRQOL in sTOF patients after primary repair (PR) vs. staged repair (SR; initial palliation with subsequent complete repair).Methods:The Pediatric Quality of Life Inventory (PedsQL) was used to assess HRQOL. Age-appropriate PedsQL modules and a parent survey were sent to all living sTOF patients with neonatal intervention from 2005-2017 across 9 centers. These were compared to established population data from children who are healthy, have chronic illness, and have cCHD. Differences in patient and clinical factors were compared between those with normal and low (>2 standard deviations below the mean) HRQOL scores.Results:Of 511 surveys sent, 143 were returned (28%). Of the respondents (53% male, age 3.2-15.9 years), PedsQL scores were lower than the healthy population (total score 75 vs 84, p<0.001) but similar to patients with chronic illnesses (74, p=0.64) and cCHD (76, p=0.72). Although only 7% and 2% of parents rated their child’s overall health and QOL as fair or poor, up to 27% of patients had PedsQL scores >2 SD below the healthy population mean. Factors associated with low HRQOL included longer duration of inhalational anesthetic exposure within 18 months of life (p=0.04) and any hospital complications after index procedure (p=0.01). There was no association between HRQOL and initial treatment strategy (PR vs SR, p=0.9).Conclusions:Lower HRQOL in sTOF is consistent with those with chronic illnesses and other forms of severe CHD. Initial treatment strategy was not associated with differences in HRQOL in sTOF patients so future comparison to other CHD groups, including non-cyanotic TOF patients, may help elucidate drivers of lower HRQOL. The discrepancy between parental description of QOL and standardized measurement of QOL is striking, warranting further investigation of parental expectations and potential impact on health resource utilization.