We present the case of a 2.5 year-old child with unbalanced atrioventricular septal defect due to a small left ventricle (LV) (mitral annulus of 10mm and a 0.4 ratio in relation to the tricuspid annulus, LVDD: 17 mm, LV Vd2: 15 ml/m 2 and LV/RV long-axis ratio of 0.71); he had a favorable outcome after biventricular surgical repair. Normal LV development was observed three months after the operation (mitral annulus of 22 mm, with a 0.84 ratio in relation to the tricuspid annulus, and LVDD of 30 mm). Current parameters for utilization of the hypoplastic ventricle are discussed.

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Patient remained asymptomatic until 5 years ago, when he presented progressively increasing fatigue on stress. Paroxysmal nocturnal dyspnea occurred twice in the last six months. Heart murmur had been heard when he was 5. Physical examinationEupneic, blushed and normal pulses. Weight: 53 Kg, height: 162 cm, BP: 110/80 mm Hg, Cardiac Frequency: 80 bpm. Aorta was not felt at suprasternal notch.Chest examination showed mild impulses on the left sternal edge and ictus was palpable in the 5 th intercostal space on the midclavicular line. Heart sounds were normal and there was protomesosystolic murmur, + / + + intensity, in the mitral area, irradiating more to the left sternal edge than to the armpit.