A rare congenital heart malformation characterized by absence of the tricuspid valuvar annulus (absent right atrioventricular connection/junction) or an imperforate tricuspid valve leading to severe hypoplasia of right ventricle (functionally univentricular heart). The malformation is associated with normally related great arteries (70 to 80% of cases) or transposed great vessels an obligatory interatrial connection that is crucial for survival (patent oval foramen or atrial septal defect ostium secundum type) ventricular septal defect (VSD) pulmonary outflow obstruction (pulmonary atresia stenosis or hypoplasia) aortic coarctation and/or aortic arch interruption.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024
Newly diagnosed with
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