Primary pulmonary hypertension in a boy plexogenic pulmonary arteriopathy

Primary pulmonary hypertension (PPH) is a rare disease which, following diagnosis, in the majority of cases leads to death within 2.6 years (1). It usually affects young people, predominantly females. The aetiology of PPH is unknown, although familial disease accounts for roughly 10% of cases, which suggests a genetic predisposition (2). There are three pathohystologic types of PPH: plexogenic pulmonary arteriopathy, thromboembolic hypertension and venooclusive pulmonary hypertension. We present a boy who was treated for PPH diagnosed through a pulmonary biopsy, with pulmonary vasodilators, diuretics, intermittent infusion of PGE1 during the hypertension crises, antithrombotic agents and continuous oxygen supplements in the last year of life. He died six years after the diagnosis, due to right ventricular failure. The diagnosis was confirmed on autopsy. It was the plexogenic pulmonary arteriopathy type.