ABSTRACT
Prior to the availability of the pulmonary arterial hypertension [PAH]-specific therapy, PAH was a dreadful disease with a very poor prognosis. Better understanding of the complex pathobiology of PAH has led to a major therapeutic evolution. International regulatory agencies have approved many specific drugs with different pharmacologic pathways and routes of administration. In the year 2013, two new drugs with great potentials in managing PAH have been added to the treatment options, macitentan and riociguat. Additional drugs are expected to come in the near future. A substantial body of evidence has confirmed the effectiveness of pulmonary arterial hypertension [PAH]-specific therapies in improving the patients' symptomatic status and slowing down the rate of clinical deterioration. The newer modern medications have significantly improved the survival of patients with PAH, it remains a non-curable and fatal disease. Lung transplantation [LT] remains the only therapeutic option for selected patients with advanced disease who continue to deteriorate despite optimal therapy