title: The association of fetal cerebrovascular resistance with early neurodevelopment in single ventricle congenital heart disease.
abstract: BACKGROUND
  Children with congenital heart disease are at risk for impaired neurodevelopment (ND). We investigated the association of fetal cerebrovascular resistance with ND in patients with single ventricle lesions.
  
  
  METHODS
  In the Single Ventricle Reconstruction (SVR) and Infant Single Ventricle trials, 14-month ND was assessed using the Bayley Scales of Infant Development II. We investigated associations between ND scores and fetal middle cerebral artery pulsatility index (MCA-PI) z-scores, a Doppler-derived estimate of cerebrovascular resistance in a subset of those infants.
  
  
  RESULTS
  Neurodevelopment assessments were performed at age 14.3 ± 1 months in 170 (74%) of 230 Infant Single Ventricle and 321 (58%) of 555 SVR subjects. Fetal echocardiographic data were available in 119 subjects, 72 (61%) of which had ND testing. Mean Psychomotor Development Index (PDI) (76 ± 20) and Mental Development Index (MDI) (89 ± 17) scores were lower than normative means (100 ± 15, P < .001). Mean MCA-PI z-score was -0.95 ± 1.52. Middle cerebral artery pulsatility index z-score correlated negatively with PDI (r = -0.27, P = .02) but was not associated with MDI. When MCA-PI z-score was added to a multivariable model controlling for factors identified in the SVR trial to predict PDI, the percentage of explained variation increased from 23% to 30%, and MCA-PI z-score remained an independent predictor (r = -3.864, P = .03). Middle cerebral artery pulsatility index z-score was not an independent predictor in a model adjusting for site.
  
  
  CONCLUSIONS
  Among fetuses with single ventricle anomalies, lower cerebrovascular resistance was associated with higher ND scores. This relationship is opposite to that observed with advanced intrauterine growth retardation and may represent a unique ability of these congenital heart disease fetuses to compensate for diminished cerebral oxygen delivery.
authors.affiliation: Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY 10032, USA. iib6@columbia.edu
authors.forename: Ismee A
authors.initials: IA
authors.lastname: Williams
authors.forename: Carlen
authors.initials: C
authors.lastname: Fifer
authors.forename: Edgar
authors.initials: E
authors.lastname: Jaeggi
authors.forename: Jami C
authors.initials: JC
authors.lastname: Levine
authors.forename: Erik C
authors.initials: EC
authors.lastname: Michelfelder
authors.forename: Anita L
authors.initials: AL
authors.lastname: Szwast
mesh_terms: D001921:Brain / Q000378:metabolism; D002560:Cerebrovascular Circulation / Q000502:physiology*; D002657:Child Development / Q000502:physiology*; D005260:Female; D005333:Fetus / Q000502:physiology*; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000503:physiopathology*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D020768:Middle Cerebral Artery / Q000503:physiopathology; D015999:Multivariate Analysis; D010100:Oxygen / Q000378:metabolism; D011673:Pulsatile Flow; D014655:Vascular Resistance; D014664:Vasodilation / Q000502:physiology
chemical_list: D010100:Oxygen
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