Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor?

Eur J Cardiothorac Surg. 2017 Sep 1;52(3):558-564. doi: 10.1093/ejcts/ezx119.

Abstract

Objectives: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass.

Methods: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence. Cognitive functions were assessed with the German 'Kognitiver Entwicklungstest für das Kindergartenalter' (KET-KID). Risk factors for impaired neuro-development were evaluated.

Results: Neuro-developmental assessment was completed in 95 patients (Norwood: n = 69; non-Norwood: n = 26). Median (interquartile range) IQ and KET-KID scores were in the normal range. Except for verbal KET-KID, scores did not differ between Norwood and non-Norwood patients (verbal IQ: 98 (86-105) vs 93 (85-102), P = 0.312; performance IQ: 91 (86-100) vs 96 (86-100), P = 0.932; full-scale IQ: 93 (86-101) vs 89 (84-98), P = 0.314; KET-KID verbal: 48 (17-72) vs 25 (2-54), P = 0.020; KET-KID non-verbal: 33 (18-62) vs 45 (15-54), P = 0.771; KET-KID global: 42 (14-65) vs 28 (6-63), P = 0.208). Full-scale IQ was below average (<85 points) in 14 (20%) Norwood and 9 (35%) non-Norwood cases (P = 0.181). Global KET-KID was below average (<16th percentile) in 19 (28%) and 10 (38%) patients (P = 0.326). Smaller head circumference z-score and complications before neonatal surgery were independently associated with lower scores.

Conclusions: Neuro-developmental outcome of preschool-aged Fontan patients was in the normal range. The Norwood procedure was not a risk factor for neuro-developmental impairment. Preoperative condition and patient-related factors were more important determinants than variables related to surgical palliation.

Keywords: Cardiopulmonary bypass; Neuro-developmental outcome; Univentricular heart disease.

MeSH terms

  • Child
  • Child, Preschool
  • Cognition / physiology
  • Cognition Disorders / epidemiology*
  • Cognition Disorders / etiology
  • Cognition Disorders / physiopathology
  • Developmental Disabilities / epidemiology*
  • Developmental Disabilities / etiology
  • Developmental Disabilities / physiopathology
  • Female
  • Germany / epidemiology
  • Humans
  • Hypoplastic Left Heart Syndrome / surgery*
  • Incidence
  • Male
  • Neuropsychological Tests
  • Norwood Procedures / adverse effects*
  • Risk Assessment*
  • Risk Factors
  • Treatment Outcome