A rare congenital non-syndromic heart malformation characterized by an imperforate or absent mitral valve. In most cases there is a univentricular atrioventricular connection to a dominant right ventricle via a tricuspid valve and a hypoplastic left ventricle. Morphologic heterogeneity is considerable and hemodynamic picture and clinical manifestation depend on the type and severity of associated cardiovascular anomalies (such as ventricular septal defect or aortic atresia).
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 December 2023
Newly diagnosed with
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