Infants less than or equal to 2.5 kg have increased mortality and worse motor neurodevelopmental outcomes at 2 years of age after Norwood-Sano palliation

Konstantin Averin; Lindsay Ryerson; Morteza Hajihosseini; Irina A Dinu; Darren H Freed; Gwen Bond; Ari R Joffe; De Villiers Jonker; Leonora Hendson; Charlene M T Robertson; Joseph Atallah

doi:10.1016/j.xjon.2023.03.007

Infants less than or equal to 2.5 kg have increased mortality and worse motor neurodevelopmental outcomes at 2 years of age after Norwood-Sano palliation

JTCVS Open. 2023 Mar 22:14:417-425. doi: 10.1016/j.xjon.2023.03.007. eCollection 2023 Jun.

Authors

Affiliations

¹ Division of Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
² Division of Critical Care, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
³ School of Public Health, University of Alberta, Edmonton, Alberta, Canada.
⁴ Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.
⁵ Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada.
⁶ Section of Neonatology, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
⁷ Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.

Abstract

Objectives: In infants with single-ventricle congenital heart disease, prematurity and low weight at the time of the Norwood operation are risk factors for mortality. Reports assessing outcomes (including neurodevelopment) post Norwood palliation in infants ≤2.5 kg are limited.

Methods: All infants who underwent a Norwood-Sano procedure between 2004 and 2019 were identified. Infants ≤2.5 kg at the time of the operation (cases) were matched 3:1 with infants >3.0 kg (comparisons) for year of surgery and cardiac diagnosis. Demographic and perioperative characteristics, survival, and functional and neurodevelopmental outcomes were compared.

Results: Twenty-seven cases (mean ± standard deviation: weight 2.2 ± 0.3 kg and age 15.6 ± 14.1 days at surgery) and 81 comparisons (3.5 ± 0.4 kg and age 10.9 ± 7.9 days at surgery) were identified. Post-Norwood, cases had a longer time to lactate ≤2 mmol/L (33.1 ± 27.5 vs 17.9 ± 12.2 hours, P < .001), longer duration of ventilation (30.5 ± 24.5 vs 18.6 ± 17.5 days, P = .005), greater need for dialysis (48.1% vs 19.8%, P = .007), and greater need for extracorporeal membrane oxygenation support (29.6% vs 12.3%, P = .004). Cases had significantly greater postoperative (in-hospital) (25.9% vs 1.2%, P < .001) and 2-year (59.2% vs 11.1%, P < .001) mortality. Neurodevelopmental assessment showed the following for cases versus comparisons, respectively: cognitive delay (18.2% vs 7.9%, P = .272), language delay (18.2% vs 11.1%, P = .505), and motor delay (27.3% vs 14.3%, P = .013).

Conclusions: Infants ≤2.5 kg at Norwood-Sano palliation have significantly increased postoperative morbidity and mortality up to 2-year follow-up. Neurodevelopmental motor outcomes were worse in these infants. Additional studies are warranted to assess the outcome of alternative medical and interventional treatment plans in this patient population.

Keywords: Norwood; congenital heart surgery; low birth weight; mortality; neurodevelopment; prematurity; single-ventricle congenital heart disease.