Extract
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure ≥25 mmHg, and can be associated with multiple conditions. The diagnostic strategy and treatment of PH is heavily reliant on accurately classifying patients [1]. We recently identified two patients presenting with rapidly fatal PH of unknown aetiology, who were subsequently diagnosed at post mortem with pulmonary tumour thrombotic microangiopathy (PTTM). This is a rare, albeit likely under-diagnosed cause of PH, characterised by a remodelling pulmonary vasculopathy rather than simple tumoural obstruction. At present, it is unclear where PTTM fits within the current classification system.
Abstract
PTTM is a rare, likely under-diagnosed cause of PH, which is related to, but distinct from PTE http://ow.ly/OQad5
Footnotes
Conflict of interest: Disclosures can be found alongside the online version of this article at erj.ersjournals.com
- Received January 13, 2015.
- Accepted June 2, 2015.
- Copyright ©ERS 2015