The "scimitar syndrome" is a complex malformation of the heart, lungs, and blood vessels. The pathology of this disease was described in detail by Park19 in 1912, but it was not until 1949 when Dotter et al.6 made the first clinical diagnosis of this anomaly. Since then clinical observations have been published with increasing frequency,1-27 indicating that the scimitar syndrome is not as rare as previously suspected.
The purpose of this paper is to discuss briefly the pathology, hemodynamics, and clinical characteristics of this disease and to report five additional cases—four of whom underwent corrective surgery.
The main feature of this malformation is the anomalous drainage of the pulmonaryveins into the inferior vena cava. In most cases the anomalous drainage is restricted to the lower lobe of the right lung. In some patients, however, the venous blood of the entire right lung is collected into a
SANGER PW, TAYLOR FH, ROBICSEK F. The "Scimitar Syndrome"Diagnosis and Treatment. Arch Surg. 1963;86(4):580-587. doi:10.1001/archsurg.1963.01310100064010