Key result
Pulmonary tumour thrombotic microangiopathy is a rare, likely under-diagnosed cause of rapidly fatal pulmonary hypertension characterized by a remodelling pulmonary vasculopathy.
Case Report (n=2)
Pulmonary tumour thrombotic microangiopathy (PTTM) is a rare, likely under-diagnosed cause of rapidly fatal pulmonary hypertension characterized by a remodeling pulmonary vasculopathy.
Alerts clinicians to PTTM in unexplained fatal PH; case reports leave open systematic diagnostic and therapeutic evaluation.
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. PTTM is a rare, likely under-diagnosed cause of PH, which is related to, but distinct from PTE
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Kumar et al. (2015) conducted a case report in Pulmonary hypertension (n=2). Pulmonary tumour thrombotic microangiopathy (PTTM) was evaluated. Pulmonary tumour thrombotic microangiopathy is a rare, likely under-diagnosed cause of rapidly fatal pulmonary hypertension characterized by a remodelling pulmonary vasculopathy.
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