TY - JOUR
T1 - Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus
AU - Congenital Cardiac Research Collaborative Investigators
AU - Maskatia, Shiraz A.
AU - Glatz, Andrew C.
AU - Goldstein, Bryan H.
AU - Qureshi, Athar M.
AU - Zampi, Jeffrey D.
AU - McCracken, Courtney E.
AU - Nicholson, George T.
AU - Meadows, Jeffery J.
AU - Shahanavaz, Shabana
AU - Law, Mark A.
AU - Batlivala, Sarosh P.
AU - Mascio, Christopher E.
AU - Chai, Paul J.
AU - Romano, Jennifer C.
AU - O'Byrne, Michael L.
AU - Ligon, Allen
AU - Beshish, Asaad G.
AU - Petit, Christopher J.
N1 - Publisher Copyright:
© 2024 Elsevier Inc.
PY - 2024/9
Y1 - 2024/9
N2 - Objective: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). Study design: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. Results: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P =.034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). Conclusions: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.
AB - Objective: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). Study design: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. Results: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P =.034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). Conclusions: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.
KW - cardiac surgery
KW - congenital heart disease
UR - http://www.scopus.com/inward/record.url?scp=85195867943&partnerID=8YFLogxK
U2 - 10.1016/j.jpeds.2024.114122
DO - 10.1016/j.jpeds.2024.114122
M3 - Article
C2 - 38815742
AN - SCOPUS:85195867943
SN - 0022-3476
VL - 272
JO - Journal of Pediatrics
JF - Journal of Pediatrics
M1 - 114122
ER -