Anales de medicina interna (Madrid, Spain : 1984)
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Primary pulmonary hypertension is defined as pulmonary arterial hypertension of unknown cause. It's a diagnosis made by exclusion; all recognizable causes of pulmonary hypertension must be ruled out before the diagnosis of primary pulmonary hypertension can be made. ⋯ Recently, with the advent first of heart-lung transplantation and then of single-lung transplantation the therapeutic options have increased considerably. We discuss the characteristics of this disease and we review the literature.