Abstract
BackgroundCongenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. While advances in early diagnosis and postnatal management have increased survival in CHD children, worrying long-term outcomes, particularly neurodevelopmental disability, have emerged as a key prognostic factor in the counseling of these pregnancies.MethodsEligible participants are women presenting at 20 to < 37 weeks of gestation carrying a fetus with CHD. Maternal/neonatal recordings are performed at regular intervals, from the fetal period to 24 months of age, and include: placental and fetal hemodynamics, fetal brain magnetic resonance imaging (MRI), functional echocardiography, cerebral oxymetry, electroencephalography and serum neurological and cardiac biomarkers. Neurodevelopmental assessment is planned at 12 months of age using the ages and stages questionnaire (ASQ) and at 24 months of age with the Bayley-III test. Target recruitment is at least 150 cases classified in three groups according to three main severe CHD groups: transposition of great arteries (TGA), Tetralogy of Fallot (TOF) and Left Ventricular Outflow Tract Obstruction (LVOTO).DiscussionThe results of NEURO-HEART study will provide the most comprehensive knowledge until date of children’s neurologic prognosis in CHD and will have the potential for developing future clinical decisive tools and improving preventive strategies in CHD.Trial registrationNCT02996630, on 4th December 2016 (retrospectively registered).
Highlights
Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns
Up to 50% of children with CHD present deficits in at least one area of neurodevelopment [5, 6]. These findings seem to be more pronounced in CHD types associated with reduced oxygenated blood brain delivery, such as transposition of great arteries and left outflow tract obstruction; poor neurodevelopment has been reported in children with heart defects with normal cerebral oxygen delivery [6]
Most studies evaluating neurological abnormalities in children with CHD have focused on factors associated with surgical repair [7], as abnormal neurodevelopment was long believed to occur as a result of the procedures associated with open surgery, mainly extra-corporeal circulation
Summary
Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. More than 36,000 infants with CHD are born in Europe every year and approximately 3000 more die in utero either spontaneously through legal abortion or during neonatal life [2] Prognosis in these patients has significantly improved in recent years, with survival up to 95%, thanks to advances in diagnostic techniques and surgical management [3, 4]. Up to 50% of children with CHD present deficits in at least one area of neurodevelopment (learning ability, motor skills, language, etc.) [5, 6] These findings seem to be more pronounced in CHD types associated with reduced oxygenated blood brain delivery, such as transposition of great arteries and left outflow tract obstruction; poor neurodevelopment has been reported in children with heart defects with normal cerebral oxygen delivery [6]. Additional studies report that CHD fetuses have smaller head biometries and signs of brain sparing from the second trimester of pregnancy, regardless of the type of CHD, supporting the recent hypothesis of early onset appearance of noxa mechanisms that could lead to a poorer neurodevelopment later in life in these children [10, 11]
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