Hybrid treatment and prognosis of Scimitar syndrome / 中华胸心血管外科杂志

ABSTRACT

Objective:To evaluated the clinical optimization of treatment strategies for the disease and risk factors for prognosis.Methods:The records of 34 patients with scimitar syndrome evaluated at our medical center between Jan 2013 and Dec 2018 were reviewed, including clinical data, treatment outcome and follow-up.Results:Scimitar syndrome was identified in 34 patients, 16 males and 18 females, aged (21.16±33.19) months. The mean length of follow-up after diagnosis was (50.30±26.99) months. Compared with adult form patients, patients presenting less than 1 year of age had a higher incidence of pulmonary hypertension and pulmonary veins stenosis. 8 patients didn’t require surgical treatment after interventional coil embolization of aortopulmonary collaterals or other interventional maneuvers in associated congenital heart diseases. 15 patients had scimitar vein surgery, 4 patients had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures( P=0.569), and tended to be more common in patients had preoperative obstruction of scimitar vein( P=0.009). Overall, 7(20.5%) of 34 died. High risk factors for death included age at diagnosis( P=0.000), obvious pulmonary hypertension( P=0.007) and pulmonary vein stenosis( P=0.014). Conclusion:Patients with pulmonary artery systolic pressure near normal and absence of congenital heart disease excluding atrial septal defect do not require surgery. Postoperative pulmonary vein obstruction is related with scimitar vein stenosis before surgery regardless of redirection technique. Age at diagnosis, obvious pulmonary hypertension and pulmonary vein stenosis are risk factors for death in patients with scimitar syndrome.