RESUMEN
Cutis laxa is a unusual disorder of the connective tissue. It may be hereditary or acquired and from the genetical viewpoint, can be either of the dominant or of the recessive type. The autosomic dominant type, less frequent, has a late beginning and the evolution is usually benign, and it seldom has cardiovascular anomalies. On the contrary, the recessive type usually has an early beginning having frequent cardiovascular anomalies, with the outcome of death early in infancy. We report two siblings with the recessive type of the illness, having peripheral arterial stenosis of pulmonary branches. We review the literature with special attention to the angiohemodynamic findings, as there are few bibliographic reports about this subject, as well as about the different hypothesis on the pathogenesis of this illness.
Asunto(s)
Enfermedades Cardiovasculares/etiología , Cutis Laxo/congénito , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/genética , Niño , Preescolar , Consanguinidad , Cutis Laxo/complicaciones , Cutis Laxo/diagnóstico , Cutis Laxo/genética , Humanos , Masculino , FenotipoAsunto(s)
Síndrome de Inmunodeficiencia Adquirida/complicaciones , Cardiomiopatía Dilatada/complicaciones , Infecciones del Sistema Respiratorio/complicaciones , Cardiomiopatía Dilatada/diagnóstico por imagen , Preescolar , Ecocardiografía , Resultado Fatal , Femenino , Humanos , Masculino , Radiografía Torácica , Infecciones del Sistema Respiratorio/microbiologíaAsunto(s)
Neumopericardio/diagnóstico por imagen , Humanos , Recién Nacido , Masculino , RadiografíaRESUMEN
Hypoplastic left heart syndrome, fundamentally aortic atresia with mitral hypoplasia or atresia, and related anomalies (critical aortic stenosis, mitral atresia and mitral stenosis) are the most important causes of severe left ventricular outflow and inflow tract obstruction in the newborn. We describe the anatomo-clinic forms of this entities and enumerate another less common causes of left ventricle obstruction in the neonate: mitral subvalvular abnormalities, cardiomyopathies and cardiac tumors.