Analysis of diagnosis and treatment of scimitar syndrome in 13 cases / 中华胸心血管外科杂志

ABSTRACT

Objective:To investigate the clinical features, diagnosis, treatment and prognosis of Scimitar syndrome.Methods:A retrospective analysis of clinical data of 13 children with scimitar syndrome from January 2013 to November 2020, including clinical symptoms, chest X-ray, echocardiography, cardiac CT and cardiac catheterization prognosis and follow-up.Results:13 children with scimitar syndrome were diagnosed, including 7 girls and 6 boys with a average age of 17 months(21 days to 10 years).3 cases <5 kg in weight. Ten patients presented with the infantile form and 3 with the adult form of scimitar syndrome. 13 infantile form had lower respiratory tract infections, heart failure, and growth retardation, of which 8 cases were with severe pulmonary arterial hypertension. 3 adult form were diagnosed because of heart murmur. 12 cases had coexisting cardiac lesions, including 12 atrial septal defect, 2 patent ductus arteriosus, 1 right ventricular double outlet/ventricular septal defect, 4 right lung and right pulmonary artery dysplasia, 2 right lung dysplasia, 6 additional systemic arterial supply to the right lung. Ten patients had pulmonary venous drainage correction surgery, one patient only underwent right ventricular double-outlet correction, three patients died of severe pulmonary hypertension; one patient lost the opportunity of surgery due to obstructive pulmonary hypertension, and one patient was complicated by nervous system The disease gave up treatment. One corrected case was stenosed during discharge and 2 corrected children became stenotic during follow-ups.Conclusion:Found with the median or dextrocardial heart, recurrent respiratory infections, or unexplained pulmonary hypertension, the possibility of scimitar syndrome should be considered. The combination of echocardiography and cardiac CTA can confirm the diagnosis as soon as possible. The risk factors for mortality included infantile form and severe preoperative pulmonary hypertension. Long-term follow-up is still required after operation, and surgical intervention is required again if necessary.