Rare cause of pulmonary hypertension - pulmonary tumour thrombotic microangiopathy.

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.