ABSTRACT
Ebstein's anomaly is a malformation of the tricuspid valve characterized from the clinical view point by dysnea, evolutive cyanosis and fatigue. Is it characterized anatomically by a downward displacement of the tricuspid valve into the right ventricle due to anomalous attachment of the tricuspid leaflets. Because of the abnormally situated tricuspid orifice, a portion of the right ventricle lies between the atrioventricular ring and the origin of the valve, that is, an "atrialized" segment of the ventricle, and they have a functionally small ventricular chamber. Roentgenographic studies, usually demonstrate a characteristic enlarged heart. The globular-shaped heart on a chest X-ray may closely resemble the picture usually associated with a large pericardial effusion. The ecocardiographic study is more specific for the differential diagnosis, and usually eliminates one pathology but confirms the other. The patient that we are presenting is unusual, and, as far as we know, it is the only in the literature case in which there is a coexistence of the important pericardial effusion and the Ebstein's anomaly. We review the functional abnormalities of the right ventricle with Doppler and two-dimensional echocardiography in this patient.
Subject(s)
Ebstein Anomaly/complications , Pericardial Effusion/complications , Adult , Female , HumansABSTRACT
The aortopulmonary window is a rare congenital disease in which the aorta and pulmonary arteries are communicated by a defect of variable diameter. We review the clinical picture, diagnosis, associated lesions and treatment of four patients with this disease in a period between 1980 and 1990. Two patients had a distal and two a proximal type. There were associated lesions in three patients: Aortic arch interruption type A, patent ductus arteriosus, subaortic stenosis and a ventricular defect. All patients underwent surgical correction of a communication between 12 and 15 mm of diameter. We discuss diagnostic problems and treatment of this congenital disease.
