title: [Ebstein's "like" anomaly ventricular double inlet. A rare association].
abstract: OBJECTIVE
  The association of univentricular heart with double inlet and Ebstein's "like" anomaly of the common atrioventricular valve is extremely rare.
  
  
  METHODS
  Two hearts with this association are described with the segmental sequential system which determine the atrial situs, the types of atrioventricular and ventriculoarterial connections and associated anomalies.
  
  
  RESULTS
  Both hearts had atrial situs solitus, and a univentricular heart with common atrioventricular valve, a foramen primum and double outlet ventricle with normal crossed great arteries. In the fiefirst heart the four leaflets of the atrioventricular valve were displaced and fused to the ventricular walls, from the atrioventricular union roward the apex with atrialization of the inlet and trabecular zones and there was stenosis in the infundibulum and in the pulmonary valve. In the second heart the proximal segment of the atrioventricular valve was displaced and fused to the ventricular whith shot atrialization and the distal segment was dysplastic with fibromixoid nodules and tendinous cords short and thick; the pulmonary artery was dilate.
  
  
  CONCLUSIONS
  Both hearts are grouped in the atrioventricular univentricular connection in the segmental sequential system. The application of this method in the diagnosis of congenital heart disease demonstrates its usefulness. The associations of complex anomalies in these hearts show us the infinite spectrum of presentation of congenital heart disease which expands our knowledge of pediatric cardiology.
authors.affiliation: Departamento de Embriología, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México. Electronic address: munozembriologia1@gmail.com.
authors.forename: Luis
authors.initials: L
authors.lastname: Muñoz Castellanos
authors.affiliation: Escuela Superior de Medicina Instituto Politécnico Nacional, Ciudad de México, México.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Kuri Nivon
mesh_terms: D002102:Cadaver; D004437:Ebstein Anomaly / Q000473:pathology*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology*; D006801:Humans
keywords: Atrioventricular univentricular connection; Cardiopatía congénita compleja; Conexión auriculoventricular univentricular; Congenital heart disease; Corazón univentricular; Doble entrada ventricular con Ebstein «like»; Double inlet ventricle with Ebstein's “like” anomaly; Mexico; México; Univentricular heart