RESUMO
The clinical findings of 2 patients are studied with an association of malformations not previously reported: double outlet right ventricle and total anomalous pulmonary venous connection to the innominate vein. The following features are worth while to be underlined: 1. Both patients showed a discordance between viscero-atrial situs and direction of the apex; 2. In both cases, particularly in case 2, there existed a very unusual spacial relationship between ventricles, the right ventricle being superior with respect to the left ventricular cavity; 3. From the embryological viewpoint, this ventricular arrangement is supposed to be due to an arrest of the development of the heart in the straight tube stage, there being, however, an upwards expansion of the atrioventricular canal and common atrium; 4. An accurate diagnosis of the common or divided nature of the atrioventricular valves has been impossible in these cases; 5. These combination of lesions constitutes a challenge to cardiologists and surgeons as far as the surgical indication is concerned. In that sense, the simple anastomosis of the common pulmonary vein to the left atrium, although is not a radical cure, may be a feasible and beneficial procedure.
