Congenital Pulmonary Venolobar Syndrome in Adults-Two Case Reports

Abstract

The congenital pulmonary venolobar syndrome (CPVS) is a rare congenital abnormality of the thorax that may appear singly or in combination. The common components of CPVS include hypogenetic lung, partial anomalous pulmonary venous return, absence of a pulmonary artery, pulmonary sequestration, systemic arterialization of the lung, absence of the inferior vena cava, and an accessory diaphragm. The rare components of CPVS include tracheal trifurcation, eventration of the diaphragm, partial absence of the diaphragm, horseshoe lung, esophageal and gastric lung, anomalous superior vena cava, and absence of the left pericardium. Herein, we present 2 young adult patients with CPVS. The first had a case of classic scimitar syndrome with right pulmonary venous drainage into the inferior vena cava. The other presented with partial anomalous drainage of the left pulmonary vein into the left branchiocephalic vein. A literature review is also included.