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 palliationCentral MessagePerspective
Konstantin Averin, MD, MSc,
Lindsay Ryerson, MD,
Morteza Hajihosseini, MSc, PhD,
Irina A. Dinu, PhD,
Darren H. Freed, MD, PhD,
Gwen Bond, RN, MN,
Ari R. Joffe, MD,
De Villiers Jonker, MD,
Leonora Hendson, MD, MSc,
Charlene M.T. Robertson, MD,
Joseph Atallah, MDCM, SM
Affiliations
Konstantin Averin, MD, MSc
Division of Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
Lindsay Ryerson, MD
Division of Critical Care, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
Morteza Hajihosseini, MSc, PhD
School of Public Health, University of Alberta, Edmonton, Alberta, Canada
Irina A. Dinu, PhD
School of Public Health, University of Alberta, Edmonton, Alberta, Canada
Darren H. Freed, MD, PhD
Department of Surgery, University of Alberta, Edmonton, Alberta, Canada
Division of Critical Care, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
De Villiers Jonker, MD
Department of Surgery, University of Alberta, Edmonton, Alberta, Canada
Leonora Hendson, MD, MSc
Section of Neonatology, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
Charlene M.T. Robertson, MD
Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
Joseph Atallah, MDCM, SM
Division of Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; Address for reprints: Joseph Atallah, MDCM, SM, Division of Cardiology, Department of Pediatrics, University of Alberta, 8440-112 St, WMC 4C1.19, Edmonton, Alberta, T6G 2B7, Canada.
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.
