Abstract

The most accurate treatment of obstructive pulmonary arterial hypertension (PAH) is the obstruction relief by surgery. Pulmonary endarterectomy is the treatment of choice of chronic thromboembolic pulmonary disease, the major cause of obstructive PAH. This procedure is associated with a low mortality rate in experienced centres and results in a dramatic improvement of hemodynamic parameters, functional status and survival rate. This surgery must be performed as early as possible in the history of this disease, preferentially before arteriolitis development in the non obstructed territories. The other obstructive PAH such as angiosarcomas, echinococcosis, arteritis, fibrosing mediastinitis can also be surgically treated by endarterectomy, tumorectomy or by pass according to their aetiology. By contrast lung transplantation is a palliative treatment indicated only after failure of medical treatment in an end-stage disease. Lung and heart-lung transplantation for PAH are associated with a 5 and 10 year actuarial survival rate of 50% and 35% respectively related to a higher postoperative mortality rate than for other organ transplantation and frequent development of graft obliterans bronchiolitis.

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