SCIMITAR SYNDROME WITH ANATOMICAL OR FUNCTIONAL ABSENCE OF THE RIGHT PULMONARY-ARTERY - A STUDY OF 4 CASES

Background. - Scimitar syndrome is characterized by an anomalous pulmonary vein draining into the inferior vena cava and visible roentgenographically as a crescentic shadow of vascular density along the right border of the cardiac silhouette. The aim of this study is to report four cases associated with anatomical or functional absence of the right pulmonary artery. Case reports. - Diagnosis of scimitar syndrome had been made in 4 patients aged 3, 10, 23 and 33 years. Pulmonary scintigraphy and/or CT scan detected the scimitar syndrome and the type of pulmonary and systemic vascularization. In all cases, the vascularization of the right lung did not originate from the pulmonary artery trunk bur was supplied by systemic vessels arising from the abdominal aorta. The right pulmonary artery was vascularized a retro, against the stream, by the systemic vessels (two cases); alternatively, circulation into the right pulmonary artery resulted directly from the systemic vessels, this pulmonary artery being anatomically absent (two cases). Pulmonary arterial pressures were normal in all four cases and functional tolerance was good in three cases; repeated and severe hemoptysis required right pneumonectomy in the last patient. Conclusions. - These four cases are compared to six other similar cases in the literature. Elegant and non invasive methods are now disposable to detect such a syndrome and to assess the type of pulmonary vascularization.