Multicenter prospective clinical study to evaluate children short-term neurodevelopmental outcome in congenital heart disease (children NEURO-HEART): study protocol.

BACKGROUND: Congenital 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. METHODS: Eligible 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). DISCUSSION: The 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 REGISTRATION: NCT02996630 , on 4th December 2016 (retrospectively registered).

Enfermedad de Kawasaki. Actualización y revisión de la casuística en nuestro centro en los últimos 10 años / Kawasaki disease. Update and review of the casuistic in our center int he last 10 years

La enfermedad de Kawasaki (EK) es una vasculitis sistémica, aguda y autolimitada, con complicaciones potentcialmente peligrosas, que afecta principalmente a lactantes y niños pequeños (edad< 5 años), aunque también se observa en adolescentes y adultos. Es la principal causa de cardiopatía adquirida en niños de los países desarrollados; su incidencia es de 15,1/100.000 niños de edad < 5 años/año, siendo similar en EE.UU. y Europa. Sin tratmiento, hasta 25% de lso casos desarrolla ectasias o aneurismas coronarios, con riesgo de infarto agudo de miocardio y muerte súbita. Revisamos las manifestaciones clíncias, los criterios diagnósticos, el diagnóstico diferencial, el tratamiento y los marcadores predictivos de no respuesta al tratamiento con inmunoglobulinas. Finalmente, comentaremos los datos de un estudio descriptivo retrospectivo de lso casos de EK diagnosticados en nuestro centro desde enero de 2002 hasta diciembre de 2012 (AU)

Kawasakii disease (KD) is a systemic vasculitis, acute, self-limited, with potentially dangerous complications, which potentially dangerous complications, which manly affects infants and young children (age<5 years), but also seen in adolescents and adults. The EK is the leading cause o acquired heart disease in children in developed countries and their impact on our environment is 15.1/100,000 children < 5 years/year, similar to the U.S. and European countries. Without treatment, up to 25% fo cases of KD develop coronary ectasia or aneurysms with risk of acute myocardial infarction and sudden death. In this work we will review clincial manifestations, diagnostic criteria, differential diagnsois, therapy and predictive markers of non response immunoglobulins. We also present data of a retrospective study of children diagnosed in our center from January 2002 to December 2012 (AU)