Scimitar syndrome is a rare congenital heart disease characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava associated to other cardiac and pulmonary anomalies.
The aim of surgical treatment is to connect the scimitar vein to the left atrium. Classic approaches include different types of baffling and reimplantation procedures, with or without graft interposition. A major complication of these techniques is thrombosis and pathway obstruction, which accounts for 25 to 50% of the cases. This can lead to pulmonary hypertension, complete obstruction of right pulmonary venous return and pneumonectomy.
In 2014, a new concept in scimitar syndrome repair was described. Lugones and coworkers used the in situ pericardium as an interposed autologous pathway between the scimitar vein and the left atrium in an effort to reduce the incidence of obstruction. Recently, a modification of the original technique was described to deal with those cases showing severe malrotation of the heart. With these two approaches, all variants of scimitar syndrome can be addressed. So far, results have been excellent in short and mid-term follow-up, with 0% mortality, absence of major complications and no evidence of pathway obstruction.
As scimitar syndrome is a rare disease, it has always been difficult to gather enough cases to draw conclusions. We built this website in order to collect worldwide experiences with this technique. The main objective of this study is to test the short, mid and long-term results of the use of these new surgical approaches in terms of mortality, related complications and patency of the reconstructed pathway. The secondary objective is to study a set of variables such as reproducibility and difficulty of the techniques, mean times of cardiopulmonary bypass and aortic crossclamping, mean hospital stay, need for reintervention and need for associated procedures, among others.
To join this international muticentric study and submit a new patient, just clicking below. Download a pdf with the instructions and the list of variables to be studied here.