RESUMEN
Congenital heart disease (CHD) is the most common neonatal congenital malformation. The variety and severity of clinical presentation depend on the cardiac structures involved and their functional impact. The management of newborns with CHD requires a multidisciplinary approach, in which the nutritional aspect plays an important role. An adequate caloric intake during either preand post-surgical period, in fact, improves the outcome of these patients. In addition, the failure to thrive of these children in childhood has been related to long-term cognitive delay (attention deficit disorders, aggressive behaviour and poor social and emotional development). To date, there is a lack of standardized feeding protocols and caloric goals about how to feed neonates with CHD, and current practice varies widely between centres. The latest American Society for Parenteral and Enteral Nutrition guidelines reiterate the importance of proteins, and recommend early start of enteral nutrition, also in the most severe heart diseases, such as univentricular forms. Necrotizing enterocolitis (NEC), the most frequent and feared complication of early feeding of these newborns, often represents an obstacle in spreading this practice. Furthermore, as demonstrated in premature infants, breastfeeding seems to reduce the incidence of NEC. That is why breastfeeding must be encouraged, even if it can be difficult for these mothers due to delivery complications, associated with infant disease. In addition, eating difficulties may persist even after discharge, because these patients require nutritional support through nasogastric tubes or percutaneous endoscopic gastrostomies.
Asunto(s)
Insuficiencia de Crecimiento/epidemiología , Cardiopatías Congénitas/complicaciones , Apoyo Nutricional/métodos , Lactancia Materna , Nutrición Enteral/métodos , Enterocolitis Necrotizante/epidemiología , Enterocolitis Necrotizante/etiología , Enterocolitis Necrotizante/prevención & control , Insuficiencia de Crecimiento/etiología , Insuficiencia de Crecimiento/terapia , Cardiopatías Congénitas/fisiopatología , Humanos , Recién Nacido , Recien Nacido Prematuro , Necesidades NutricionalesAsunto(s)
Proteínas de la Matriz Extracelular/genética , Inestabilidad de la Articulación/diagnóstico , Inestabilidad de la Articulación/genética , Mutación/genética , Índice de Severidad de la Enfermedad , Enfermedades Cutáneas Genéticas/diagnóstico , Enfermedades Cutáneas Genéticas/genética , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/genética , Arterias/anomalías , Femenino , Humanos , Lactante , Recién NacidoRESUMEN
OBJECTIVE: To study anti-Ro/La-negative congenital heart block (CHB). METHODS: Forty-five fetuses with CHB were evaluated by analysis of anti-Ro/La antibodies using sensitive laboratory methods. RESULTS: There were 9 cases of anti-Ro/La-negative CHB; 3 died (33.3%). Only 3 (33.3%) were complete in utero and 5 (55.5%) were unstable. No specific etiology was diagnosed. Six infants (66.6%) were given pacemakers. There were 36 cases of anti-Ro/La-positive CHB. All except 2 infants (94.4%) had complete atrioventricular block in utero. Ten died (27.8%), one (2.7%) developed severe dilated cardiomyopathy, and 26 (72.2%) were given pacemakers. CONCLUSION: Nine of the 45 consecutive CHB cases (20%) were anti-Ro/La-negative with no known cause. They were less stable and complete than the anti-Ro/La positive cases.
Asunto(s)
Anticuerpos Antinucleares/sangre , Bloqueo Atrioventricular/inmunología , Autoantígenos/inmunología , Complicaciones del Embarazo/inmunología , Ribonucleoproteínas/inmunología , Bloqueo Atrioventricular/congénito , Bloqueo Atrioventricular/mortalidad , Bradicardia/congénito , Bradicardia/inmunología , Bradicardia/mortalidad , Cardiomiopatía Dilatada/inmunología , Cardiomiopatía Dilatada/mortalidad , Femenino , Humanos , Recién Nacido , Masculino , Morbilidad , Embarazo , Diagnóstico Prenatal , Estudios Seroepidemiológicos , Antígeno SS-BRESUMEN
A newborn girl with atresia of the common pulmonary vein, presented immediately after birth with severe cyanosis and acidosis. The diagnosis of totally obstructed total pulmonary venous return was made by cross-sectional echocardiography. Subsequent cardiac catheterization failed to demonstrate the site of pulmonary venous return. Necropsy showed the pulmonary veins to be connected bilaterally to an atretic common pulmonary vein. There was no obvious alternative pathway for pulmonary venous return.