Isolated pulmonary vein stenosis in complex congenital heart disease, simulating cor triatriatum by cardiac catheterization and transoesophageal echocardiography.

Abstract

A case of localized stenosis of the lower left pulmonary vein, associated with perimembranous ventricular septal defect (VSD) and secundum atrial defect (ASD), a borderline sized mitral valve apparatus and left ventricle, is described. This final diagnosis was made at surgery, as the localized stenosis of the left pulmonary vein was misinterpreted as partial cor triatriatum by cardiac catheterization, cineangiocardiography and extensive echocardiographic studies. Surgical correction of all the defects was successful, but pulmonary vascular resistance was still increased 2 years after surgery.