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Pulmonary veno-occlusive disease

Dr Yuranga Weerakkody et al.

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

Epidemiology

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

It is rare but can potentially affects 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.

Associations

PVOD can be idiopathic or can complicate other conditions which include ²

Clinical presentation

Its presentation can be similar to other forms of pulmonary aterial hypertension.

Radiographic features

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

CT chest

Reported HRCT findings include^1-2,4

smooth interlobular septal thickening
regions of ground-glass opacity : can be
- diffuse multifocal ^1,4
- perihilar ⁴
- patchy ⁴
- centrilobular ^2,4
pleural effusions
enlarged central pulmonary arteries
normal calibre pulmonary veins
mosaic pattern of lung attenuation

Treatment and prognosis

PVOD generally carries a poor prognosis (some report a 72% mortality at one year from diagnosis ⁷). Lung transplantation is the treatment of choice ². It is omportant ot note that therapy commonly used to treat patients with primary pulmonary hypertension, such as calcium-channel blockers (CCBs) ¹, epoprostenol (PGI2)¹ or prostacylin ⁶ can lead to disastrous outcomes in patients with PVOD.