Pulmonary vein atresia with Shone's anomaly in an infant: a case report

We report a case of individual pulmonary vein atresia associated with multi ple levels of left heart obstruction, including aortic coarctation, valvula r aortic stenosis, and parachute mitral valves with stenosis, The diagnosis of pulmonary vein obstruction is likely to be missed in patients who also have other left heart obstructive diseases, since the latter usually domina tes the clinical presentation. We diagnosed the existence of individual pul monary vein atresia preoperatively via cardiac catheterization. The pulmona ry artery angiograms revealed back and forth motion of the dye with no visu alization of either a capillary or venous phase on the lesion side. The pul monary capillary wedge pressure was unevenly elevated and highest on the le sion side. The results were later confirmed by operation and autopsy. Thus, selective pulmonary artery catheterization and angiography remains a good diagnostic tool to rule out the existence of pulmonary vein obstruction in cases which have multiple levels of left heart obstruction.