Pulmonary venoocclusive disease

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  • Rare cause of pulmonary hypertension due primarily to occlusion of post-capillary venous radicles by fibrosis
  • Pulmonary arterial hypertension, pulmonary edema and normal wedge pressure
  • Poor prognosis


Differential for PAH

  • Thromboembilic disease
  • Lung disease
  • Idiopathic
  • Left to right shunt
  • Vasculitis