Anomalous Pulmonary Venous Drainage of Right Lung to Inferior Vena Cava (‘Scimitar Syndrome’): Clinical Spectrum in Older Patients and Role of Surgery

Abstract

Six new cases of the "scimitar syndrome" are described. The anatomical and haemodynamic features of these and other reported cases are reviewed. Within the spectrum of the disorder there is a group of patients in whom the bronchopulmonary manifestations are relatively unimportant. In five of our patients there was a left-to-right shunt exceeding 2:1 and the anomalous pulmonary venous connection was corrected surgically. The presence or absence of an associated atrial septal defect may be difficult to establish but influences the choice of surgical technique. When the atrial septum is intact, the anomalous vein should be reimplanted if possible into the back of the left atrium; otherwise a pericardial or teflon patch can be used to redirect the anomalous venous return through an existing or created atrial septal defect to the left atrium.