Pulmonary veno-occlusive disease

A 50-year-old female patient with a long history of Raynaud's phenomenon and rapidly deteriorating right-sided cardiac failure is presented. Pulmonary veno-occlusive disease was diagnosed from typical clinical and hemodynamic findings using a Swan-Ganz balloon catheter. The diagnosis was definitely confirmed at necropsy. There was no clinical, laboratory, or histologic evidence of a connective tissue disease.