Dissecting hematomas of the pulmonary artery: rare and fatal catastrophies.

This article presents, to the author's best knowledge, the first reported case of a fatal pulmonary artery dissecting hematoma that was a long-term complication of a Waterston shunt procedure (aortopulmonary anastamosis). Pulmonary artery dissecting hematomas are rare and generally fatal, and they are usually not diagnosed until the autopsy is performed. They occur in young adults, are almost always associated with pulmonary hypertension, and often present with the sudden onset of excruciating lancet-like retrosternal pain. Risk factors for the development of dissecting hematomas, in addition to hypertension, include vascular turbulence, trauma, connective tissue disorders, syphilis, infection, previous surgery, and iatrogenic events (e.g., catheterization). The treatment is prompt medical reduction of pulmonary hypertension.