[Primary pulmonary arterial hypertension]

Primary pulmonary hypertension (PPH) currently remains an entity of which the precise mechanism, most likely vasospastic, is not completely explained. The diagnosis of PPH remains an exclusion diagnosis; it will be considered only after ruling out a secondary pulmonary hypertension. Non-invasive techniques such as sonography, cardiac Doppler should permit an earlier diagnosis and an easier monitoring of these patients whose prognosis remains poor in spite of the sporadic efficacy of a treatment with vasodilators. Heart-Lung transplant could be a future prospect; however, if the first reports seem encouraging, the follow-up is currently insufficient to appreciate the possible long-term advantage.