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A case of a previously unreported anomaly is presented in which absence of the aortic valve cusps, mitral atresia, a normal left ventricle, and an intact ventricular septum were diagnosed by cross sectional echocardiography. The development of a normal left ventricle, rather than the hypoplastic ventricle usually associated with mitral atresia, is explained by filling of the ventricular cavity via the regurgitant aortic valve.

Absence of the aortic valve cusps with mitral atresia, normal left ventricle, and intact ventricular septum.