Progressive primary pulmonary hypertension in a patient born at high altitude

Abstract

A girl, born at high altitude, subsequently developed pulmonary hypertension which did not improve on return to sea-level and proved fatal at the age of 19 years. The clinical and necropsy features were those of primary pulmonary hypertension, including a plexiform arteriopathy. There is a possibility of hypoxia in early life triggering progressive pulmonary vascular changes.