Fatal acute pulmonary hypertension caused by pulmonary tumour thrombotic microangiopathy

Abstract

Pulmonary tumour thrombotic microangiopathy (PTTM) presents as acute pulmonary hypertension and sudden cardiovascular collapse in individuals with adenocarcinoma, usually of the stomach. The illness follows a fulminant course often before the adenocarcinoma has become clinically apparent. Frequently the diagnosis is made only at necropsy. The diagnosis can be made by pulmonary microvascular cytology, obtained through a wedged pulmonary artery catheter. No treatment has thus far been found to be of benefit. We present a typical case in which the diagnosis was not reached before death despite multiple investigations and review the literature.