Pulmonary arterial hypertension is a severe disease that has been ignored for decades. However, there has been growing interest from respirologists, cardiologists, and thoracic surgeons due to development of new therapies that have improved the outcome and quality of life of patients suffering from pulmonary arterial hypertension. Surgery has a major place among new therapies and consists of either transplanting the lungs in end-stage pulmonary arterial hypertension after failure or escape from pharmacotherapy or curing postembolic pulmonary hypertension by pulmonary endarterectomy. Other procedures, such as endarterectomy of angiosarcomas or Potts anastomoses as a palliative treatment of pulmonary arterial hypertension in children, are less common.