Harch S, Whitford H, McLean C.
The Alfred Hospital, Melbourne, VIC, Australia.
Chest 2009;135:1462–9.
Editor’s note: Despite recent improvements in therapies for pulmonary arterial hypertension (PAH), a subset of patients remain unresponsive to treatment, and either undergo lung transplantation or die. If a diagnosis of PAH is made, but the subsequent PAH-targeted treatment is ineffective, the diagnosis has to be revisited. Clinical data suggest that PAH is difficult to distinguish from pulmonary veno-occlusive disease (PVOD), both upon presentation and by common investigation methods such as right-heart catheterization and pulmonary function tests. On this basis, the present authors hypothesized that treatment failure in patients with a PAH diagnosis may be due to misdiagnosed PVOD.