PubMed · 17228014
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Christine H Attenhofer Jost, Heidi M Connolly, Joseph A Dearani, William D Edwards, Gordon K Danielson. 2007-01-16. Ebstein's anomaly.. https://doi.org/10.1161/circulationaha.106.619338
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Ebstein's anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier's technique for Ebstein's anomaly to restore the morphology of the right ventricle, and "one and half ventricle repair" can be a salvage procedure if the right ventricle is not functionally tolerant.
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An 50 year-old asymptomatic female was admitted to preoperative cardiovascular evaluation for noncardiac surgery. Two-dimensional transthoracic echocardiography demonstrated that left ventricular dimensions and ejection fraction were normal. The mitral valve was normal both in morphology and function. The attached margin of septal and anterior leaflet of the tricuspid valve was apically displaced. But right ventricle and right atrium dimensions were normal, and Doppler interrogation showed a slight tricuspid regurgitation. Two and three-dimensional transthoracic echocardiography showed prominent numerous trabeculations in the left ventricular apex. Colour flow Doppler studies confirmed the presence of blood flow within the trabeculations. Multi-plane three-dimensional transthoracic echocardiography showed a muscular band leading to left ventricular mid-caviter narrowing. Pulse wave Doppler echocardiography demonstrated that no intraventricular gradient existed. Ventricular noncompaction associated with Ebstein's malformation has been reported. But left ventricular noncompaction and mid-caviter narrowing associated with Ebstein's anomaly have not been reported so far.