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The aim of this work is to demonstrate the possibility to identify persistent right sinus venous valve and to differentiate it from other right sided heart pathology by two dimensional echocardiography. We report the echocardiographic findings observed in three out of 215 paediatric patients we examined for clinically suspected congenital heart disease in 12 months period. The first patient was a 24 hours old newborn with transient pulmonary hypertension; the second one was a 6 months old child with pulmonary atresia, severe right ventricular hypertrophy, atrial septal defect and patent ductus arteriosus; the third patient, aged 6 months, had atrial septal defect and mild pulmonary stenosis. In all three patients a particular linear structure was seen, as a membrane that crossed the right atrium from the orifice of the inferior vena cava towards the atrial septum. This structure was identified as persistent right sinus venosus valve according to its morphology, its position into the right atrium and its connection to the atrial septum. The incidence of this echocardiographic finding was 1.4% in our series. In the first case contrast echocardiography from the inferior vena cava demonstrated the deviation of blood flow caused by the persistent sinus valve, although no intra-atrial pressure gradient was shown at cardiac catheterization. In the second patient who died during the operative procedure for making a systemic-pulmonary anastomosis, post mortem examination showed a membranous structure connecting the orifice of the inferior vena cava to the atrial septum.(ABSTRACT TRUNCATED AT 250 WORDS)