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Introduction: Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with either staged repair (SR; initial palliation with subsequent complete repair) or primary repair (PR). Implications of these neonatal management strategies on late outcomes are not well understood. Thus, we aimed to assess neurodevelopmental (ND) outcomes and identify risk factors for ND concerns. Methods: From 2005-2017, sTOF patients who underwent neonatal intervention across 9 centers were identified. To assess ND, all living patients were sent age-appropriate parent-rating scales of executive function (Behavior Rating Inventory of Executive Function - BRIEF) and psychosocial symptoms (The Behavior Assessment System for Children, 3rd Edition - BASC-3). The results were compared by primary treatment strategy (SR vs. PR) using multivariable models. Patient and clinical factors were compared between those with normal and clinically significant abnormal results (T-score ≥60). Results: Of 572 patients in the initial cohort, surveys were sent to 511 survivors and returned from 144 (28%, 53% male, age 3.2-15.9 years). Up to 37% of respondents had a clinically concerning score on ≥ 1 scales, including 36% with ≥1 abnormal scale present on the BRIEF and 30% with an abnormal scale on the BASC. Across SR and PR strategies, there was no difference in ND outcomes (BRIEF adjusted odds ratio (AOR) 1.55 (0.57, 4.21, p=0.39); BASC AOR 1.13 (0.43, 2.99, p=0.81)). Factors associated with clinically significant executive dysfunction included genetic syndrome (p=0.001), need for invasive ventilatory support (p=0.003) and inotropic support (p=0.01) prior to the index procedure. Only genetic syndrome (p=0.026) was associated with clinically significant psychosocial problems. Conclusions: Late ND abnormalities, specifically executive dysfunction and psychosocial concerns, are common in sTOF patients. While initial treatment strategy does not appear to have impact, intrinsic patient characteristics and pre-procedure measures of clinical severity may predict late ND outcomes and help target increased surveillance for ND complications. Forthcoming analyses will explore existing ND service utilization and parental concerns in the context of these findings.

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.

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