Progress in clinical diagnosis and treatment of scimitar syndrome / 国际儿科学杂志

ABSTRACT

Scimitar syndrome is a rare congenital cardiopulmonary anomaly,consisting of partial anomalous pulmonary venous return,right lung hypoplasia,and several associated defects.The condition generally has significant morbidity and mortality,but its etiology is poorly understood.Scimitar syndrome has a wide spectrum of clinical presentations ranging from severe respiratory difficulty and high mortality in the perinatal period to asymptomatic and incidental in adulthood.Its diagnosis is mainly dependent on angiocardiography and imging methods.The adult combined with scimitar syndrome are usually given symptomatic treatment.If severe symptoms appears,its primary treatment is surgery to correct anomalous drainage.In the article,the research progress of the pathological anatomy,familial scimitar ayndrome,the clinical diagnosis,clinical therapy and prognosis are reviewed.