Timing and Mode of Death Following Treatment of Neonatal Symptomatic Tetralogy of Fallot

BACKGROUND: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, using a staged or primary repair strategy. Postprocedural mortality risk within this group is considerable. The mode of death and associated factors have been inadequately defined in early childhood mortality following treatment of sTOF. This study aims to describe the timing, cause, and associated risk factors of postprocedural death in infants with sTOF. METHODS: Neonates with sTOF who had an initial intervention from 2005 to 2017 at 9 centers of the Congenital Cardiac Research Collaborative were reviewed. Among nonsurvivors, details regarding timing, cause of death, and last follow-up were obtained. Patient characteristics between survivors and nonsurvivors were compared. RESULTS: Of 572 patients with sTOF (230 primary repair, 342 staged repair), 52 (9%) died at a median age of 82 (25th–75th%ile: 28–246) days. Death was perioperative (≤30days) in 52% of cases. Most deaths were cardiac (69%, n=36), with heart failure and sudden death being most common. Noncardiac causes included necrotizing enterocolitis (n=3), infectious (n=3), and intracranial hemorrhage (n=2). Risk factors for mortality included prematurity, low birth weight, genetic syndrome, race categorized as other, noncardiac anomaly, and ventilation before initial intervention (P<0.05 for all). Among patients discharged after definitive repair, right ventricular dysfunction was more common in nonsurvivors. Sudden death is a prominent known cause of death in this subgroup. CONCLUSIONS: When death occurs after interventions for sTOF, it frequently occurs early and relates to traditional risk factors. Sudden death is a prominent cause of mortality following discharge from definitive repair.