Pulmonary veno-occlusive disease (PVOD) is a rare disorder classified as a subgroup of pulmonary arterial hypertension (PAH).
The term was coined in 1966; prior to this, the terms “isolated pulmonary venous sclerosis,” “obstructive disease of the pulmonary veins,” or the “venous form of primary pulmonary hypertension” had been used to describe the syndrome.
The pathologic hallmark of PVOD is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue, which may be loose and edematous or dense and sclerotic, the former probably reflecting an earlier stage in the development of the lesion than the latter.
Pulmonary veno-occlusive disease or Eisenmenger's syndrome, may cause chest pain. Chest pain from severe obstructive lesions results from increased myocardial oxygen demands from tachycardia and increased pressure work by the ventricle. Therefore, the pain is usually associated with exercise and is a typical anginal pain.
Pulmonary veno-occlusive disease (PVOD)
