Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease is considered an uncommon variant of primary pulmonary hypertension (PPH) that preferentially affects the postcapillary pulmonary vasculature.

It is thought to account for ~5-10% of cases initially considered to be idiopathic PAH 2.

It is rare but can potentially affect all age groups with no geographic predilection.  The majority of affected patients are under 50 years of age 1. In the paediatric population, both sexes are equally affected. In the adult population, the ratio of male to female patients is approximately 2:1.

Its presentation can be similar to other forms of pulmonary arterial hypertension such as progressive dyspnea, cough, and also haemoptysis. Pulmonary capillary wedge pressure is usually normal due to patchy involvement.  

It is characterised by extensive occlusion of pulmonary veins by fibrous tissue in a patchy distribution. This may be loose and oedematous or dense and sclerotic 7.

Pulmonary veno-occlusive disease can be idiopathic (in most cases, the causes is not known) or can complicate other conditions which include 2:

In general, imaging features can be non-specific and a definitive diagnosis often requires a lung biopsy 1.

Reported HRCT chest findings include1-2,4:

Pulmonary veno-occlusive disease generally carries a poor prognosis (some report a 72% mortality at one year from diagnosis 1). Lung transplantation is the treatment of choice 2. It is important to note that vasodilators commonly used to treat patients with primary pulmonary hypertension, such as calcium-channel blockers (CCBs) 1, epoprostenol (PGI2) 1 or prostacyclin 6 can lead to disastrous outcomes (severe pulmonary oedema) in patients with pulmonary veno-occlusive disease.

It is thought to have been first described by Hora et al in 1934 1.

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Article information

rID: 16679
System: Chest
Tag: cases
Synonyms or Alternate Spellings:
  • Pulmonary veno occlusive disease
  • Pulmonary veno occlusive disease (PVOD)
  • Pulmonary veno-occlusive disease (PVOD)

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