The case of 2-month old baby with pulmonary hypertension is described. In echocardiography examination the intracardiac congenital defect and persistent ductus arteriosus was excluded. In abdominal ultrasonography the anomalous artery leading through the diaphragm to the thorax was observed. In cardiac catheterisation the pulmonary hypertension was confirmed. In aortography additional artery originated from aorta and provided lower right lung lobe was shown. In the lung perfusion scan the lack of perfusion in the lower part of right lung lobe was seen. The closure of anomalous artery was successful performed during the interventional cardiology treatment. The temporary balloon obstruction of this artery did not increase the pressure in pulmonary circulation. In the next stage five coils diameter 8 mm to 3 mm were implanted and the leak through artery decreased. The controlled echo examination the next day and one week later showed the lower pressure in the right ventricle then before interventional procedure (from 100 mmHg to 60 mmHg). Twenty-one months follow up shows no signs of pulmonary hypertension. There is no shunt through anomalous artery .Baby is in good condition. The described case is such interesting because this type of defect, is so rare and closure of anomalous shunt during the interventional procedure was performed successfully. The diagnosis of pulmonary hypertension without coexisted congenital leak heart defect in the smalI child orders looking for anomalous lung perfusion especially nowadays when interventional (non surgical) treatment is available.

Keywords: Scimitar Syndrome, interventional treatment, coil