The author presents an overall account on the Syndrome of left heart hypoplasia. He emphasizes the etiopathogenesis of the disease, pathological variations, clinical manifestations and diagnostic possibilities of this complex cardiac defect. In the conclusion the author discusses ethical aspects of the hypoppaasic left heart Syndrome and its possible treatment under our conditions.

        Key words: inborn cardiac defects, extracardial anomalies, cyanotic cardiac defects, mitral stenosis, aortal atresia, aortal stenosis, hypoplasia of the aorta