CONGENITAL PULMONARY VENOLOBAR SYNDROME REVISITED

The term ''congenital pulmonary venolobar syndrome'' (CPVS) encompasse s a number of congenital abnormalities of the thorax that often occur in combination. Major components of CPVS include hypogenetic lung, par tial anomalous pulmonary venous return (this and the former are two of the most constantly occurring components), absence of a pulmonary art ery, pulmonary sequestration, systemic arterialization of the lung, ab sence of the inferior vena cava, and accessory diaphragm. Minor compon ents of CPVS include tracheal trifurcation, eventration and partial ab sence of the diaphragm, phrenic cyst, horseshoe lung, esophageal and g astric lung, anomalous superior vena cava, and absence of the left per icardium. Most patients with CPVS have no symptoms and require no ther apy; however, surgical intervention is often necessary in infants with severe symptoms. The authors review the imaging findings in 23 patien ts with CPVS and review the literature concerning the diagnosis and ma nagement of this complex syndrome in order to improve the understandin g of CPVS among radiologists and clinicians.