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Title: Rare cause of pulmonary hypertension - pulmonary tumour thrombotic microangiopathy.
Authors: O'Brien, Joseph M;Jones, Nicholas;Horrigan, Mark;Al-Kaisey, Ahmed M
Affiliation: Department of Cardiology, Austin Health, Heidelberg, Victoria, Australia
Issue Date: 9-Aug-2019
EDate: 2019-08-09
Citation: BMJ case reports 2019; 12(8): e225756
Abstract: Pulmonary tumour thrombotic microangiopathy (PTTA) is a rare but lethal cause of pulmonary hypertension (PHT). Its underlying mechanism is believed to be fibrocellular intimal proliferation and microthrombosis. It has been reported in association with gastric adenocarcinoma and breast, pancreatic and lung cancers. The diagnosis is often made on postmortem examination due to the absence of diagnostic criteria and its rare occurrence. We describe the case of a middle-aged man who presented with rapidly progressive PHT. He deteriorated into multiorgan failure despite aggressive medical therapy and died 4 weeks after his initial presentation. A postmortem examination confirmed the diagnosis of PTTA in addition to the finding of signet cell gastric adenocarcinoma. This case highlights the lethal nature of this rare condition, the ongoing challenges in making an antemortem diagnosis, and the importance of postmortem examination in determining the cause of death to provide closure for both, the treating physician and the family.
DOI: 10.1136/bcr-2018-225756
ORCID: 0000-0001-5174-1034
PubMed URL: 31401584
Type: Journal Article
Subjects: cancer - see oncology
gastric cancer
heart failure
Appears in Collections:Journal articles

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