We present two symptomatic patients of five and six months old with unilateral agenesis of main pulmonary branch (right and left) without associated anomalies. The diagnostic was made with magnetic resonance imaging and two-dimensional echocardiographic with Doppler color flow. We think that the magnetic resonance, must be used with priority in this pathology for being a non invasive diagnostic method, that is able to diagnostic the basic cardiopathy and the intracardiac and extracardiac associated anomalies. His association with the Doppler-echocardiography allow to ignore the arteriography upon to get a accurate diagnostic.
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