We present seven patients with the scimitar syndrome. The clinical and
anatomical spectrum is described. Two different types of scimitar vei
n were recognized. a) simple classic vein running from the middle of t
he right lung to the cardiophrenic angle (5 patients) and b) double ar
ched vein in the upper and lower lung zone, with ample drainage into t
he left atrium and inferior caval vein (2 patients). Four patients req
uired surgical-treatment. Indications, diagnostic procedures and surgi
cal management are discussed. In two patients, thrombosis and fibrosis
occurred in the scimitar vein that had been reimplantated in the left
atrium, necessitating pneumonectomy.