肺動脈高壓在過去的二十年內有了長足的進步。隨著愈來愈多的研究被發表和討論，對於治療策 略與目標漸漸的有了相當程度的改變，也有更強的臨床證據支持。報告顯示在疾病一開始便積極的給予 治療，有助於改善長期預後。儘管在死亡率的好處尚未達到統計上的差異，接續性合併(sequential combination)治療或起始合併(upfront combination)治療大多能實質改善臨床上的惡化。為了達到更好的預 後，現在已經不再建議當臨床惡化才決定調整治療的強度，而是建議利用相關的治療標的(goals)或評估或 計分表來建立目標導向式(goal-oriented)的治療。

The knowledge of pulmonary arterial hypertension (PAH) has been substantially advanced in the past two decades. With the growing body of clinical evidence, treatment strategies and goals have been evolving considerably and have become more evidence-based. Early intervention with PAH-specific agents has been shown to improve long-term outcome. Besides, both sequential combination and upfront combination strategies are supported by many reports in terms of clinical worsening, though the mortality benefit has yet failed to reach statistical significance. To achieve better outcomes, the goal-oriented therapy according to a panel of treatment targets, rather than the clinical worsening oriented therapy, is more recommended.