Attenuated pulmonary vascular obstructive disease in double-outlet right ventricle and mitral stenosis

A young woman with double-outlet right ventricle, mitral stenosis, and high systemic pulmonary artery pressure is described. Pulmonary vascular morphometric analysis indicated considerably less pulmonary vascular obstructive disease than might otherwise have been expected. Total repair was performed and resulted in a marked fall in pulmonary artery pressure. Pulmonary vascular morphometry may be indicated in selected patients before palliative management is selected over complete repair.