DOI: 10.1161/circ.148.suppl_1.12267 ISSN: 0009-7322

Abstract 12267: Timing and Mode of Death Following Treatment of Neonatal Symptomatic Tetralogy of Fallot

Sara M Trucco, Yun Zhang, Courtney McCracken, Andrew C Glatz, Christopher J Petit, Athar Mahmood Qureshi, Jeffrey Zampi, Shabana Shahanavaz, George Nicholson, Nathalie Abejero, Allen Ligon, Jay Patel, Christopher Mascio, Shiraz A Maskatia, Jennifer Romano, Michael L Obyrne, Kathryn Stack, Jeannette Wong-Siegel, Asaad Beshish, Mark Law, Kristal Hock, Sarosh P Batlivala, Sarah Speed, Jeffery J Meadows, Disha Goel, Hala Khan, Bryan H Goldstein
  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Introduction: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, using a staged (SR) or primary repair (PR) strategy. Independent of management, the risk of post-procedural mortality is considerable. Although risk factors for late mortality after TOF repair have been established, the modes of death and associated factors have not been defined in early childhood following treatment of sTOF.

Methods: Neonates with sTOF who had an initial intervention from 2005-2017 at 9 centers of the Congenital Cardiac Research Collaborative were reviewed. Among the patients who died, additional details related to timing and cause of death, and last clinical follow-up, were obtained and described. Comparisons of patient characteristics between survivors and non-survivors were performed.

Results: Of 572 sTOF patients (230 PR, 342 SR), there were 52 (9%) deaths at a median age of 82 (25 th - 75 th %ile: 28, 246) days . Timing of mortality included: inpatient post-palliation (n=9), interstage in SR pathway (n=11), inpatient post complete repair (CR) (SR, n=5; PR, n=11) and post-CR following hospital discharge (n=16). Death occurred within 30 days of palliation or repair in 52% of cases. Most deaths were due to cardiac etiologies, with only 6 including ≥1 non-cardiac cause of death (NEC (n=2), sepsis (n=1), CNS bleed (n=2), trauma (n=1)). Risk factors for mortality included presence of genetic syndrome, non-cardiac anomaly, prematurity, low birth weight, and need for ventilation prior to initial intervention (p<0.05 for all). Modes and timing of death in the 16 patients who died after CR discharge are shown (Figure). In these, RV dysfunction at last echo was present in 50%.

Conclusions: When death occurs after interventions for sTOF, it frequently occurs early, and relates to traditional risk factors. Amongst patients with childhood mortality following discharge from CR, sudden death and reintervention-related events predominate known causes.

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