Introduction:The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease. Material and Methods:A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery. Objective: to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease. Results:We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I. Discussion and Conclusion: The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.

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Introduction:Complex congenital heart disease is a group of severe conditions. Prenatal diagnosis has implications on morbidity and mortality for most severe conditions. The purpose of this work was to evaluate the influence of prenatal diagnosis and distance of residence and birth place to a reference center, on immediate morbidity and early mortality of complex congenital heart disease. Material and Methods:Retrospective study of complex congenital heart disease patients of our Hospital, born between 2007 and 2012. Results: There were 126 patients born with complex congenital heart disease. In 95%, pregnancy was followed since the first trimester, with prenatal diagnosis in 42%. There was a statistically significant relation between birth place and prenatal diagnosis. Transposition of great arteries was the most frequent complex congenital heart disease (45.2%), followed by pulmonary atresia with ventricular septal defect (17.5%) and hypoplastic left ventricle (9.5%). Eighty-two patients (65.1%) had prostaglandin infusion and 38 (30.2%) were ventilated before an intervention. Surgery took place in the neonatal period in 73%. Actuarial survival rate at 30 days, 12 and 24 months was 85%, 80% and 75%, respectively. There was no statistically significant relation between prenatal diagnosis and mortality. Discussion: Most patients with complex congenital heart disease did not have prenatal diagnosis. All cases with prenatal diagnosis were born in a tertiary center. Prenatal diagnosis did not influence significantly neonatal mortality, as already described in other studies with heterogeneous complex heart disease. Conclusion: prenatal diagnosis of complex congenital heart disease allowed an adequate referral. Most patients with complex congenital heart disease weren't diagnosed prenatally. This data should be considered when planning prenatal diagnosis of congenital heart disease. Keywords: Heart Defects, Congenital; Prenatal Diagnosis. INTRODUÇÃOAs cardiopatias congénitas são as malformações congénitas major mais comuns e constituem o grupo com maior relevância clínica, com uma prevalência de 0,5% a 1% no período neonatal.1-3 Cerca de metade destas cardiopatias são graves e necessitam de intervenção no período neonatal ou nos primeiros anos de vida. 2As cardiopatias congénitas complexas (CCC) constituem um grupo de doenças graves e incluem na sua grande maioria uma alteração do arranjo segmentar, sendo exemplos o síndrome de coração esquerdo hipoplásico (SCEH), transposição das grandes artérias (TGA) e o coração univentricular. De entre as cardiopatias congénitas,

Percutaneous closure of atrial right-to-left shunt in selected patients with Ebstein's anomaly offers significant improvement, abolishing hypoxaemia and hyperviscosity and preventing paradoxical embolisation.

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