SCIMITAR SYNDROME. A POSSIBLE ROLE OF THE THORACOSCOPIC APPROACH.

Scimitar syndrome (SS) is a variety of congenital vascular abnormalities known as "partial anomalous pulmonary venous connections"(PAPVCs).In this anomaly, the venous return of a lung(more frequently the right one)drains into the inferior caval vein, the ipsilateral atrium, the coronary sinus, the azygos vein, portal vein or hepatic vein. SS is frequently associated with other anomalies:right lung hypoplasia(almost always present)with lobation defects, dextroposition of the heart,right pulmonaryartery hypoplasia(60%), sy stemic arterial blood supply to the lung(60%), atrial septal defect (ASD) (40%overall,80-90% in yhe infantile form), right posterior diaphragmatic hernia (15%)and horseshoe lung. The main clinical problem is the progressive development of pulmonary hypertension that may lead to heart failure. Definitive surgical treatment consists of rerouting the venous drainage. Many techinique have been proved but they all present technical difficulties and a high rate of postoperative complications such as stenosis of the scimitar vein. All medical and surgical measures aimed at controlling pulmonary hypertension can therefore be used to allow patient's grow and delay or avoidcardiac surgery. Among these,the closure of systemic arterial blood supply may constitute a valid and mininvasive opportunity. We report our initial experience in 4 pediatric cases. The first two cases were treated with percutaneous embolization and other two with thoracoscopic closure of the aberrant artery. In one case (treated with embolization), the patient dead for major cardiac malformation, while the other cases demonstrate hemodinamic stebility at follow-up.