儿童肺动脉高压的药物治疗进展

Progress in pharmacotherapy of pulmonary arterial hypertension in children

摘要:

本文综述了儿童肺动脉高压（PAH）的药物治疗现状，并简要介绍了新的治疗靶点。前列环素类似物、内皮素受体拮抗剂及磷酸二酯酶5抑制剂等3类药物能显著改善PAH患儿的血流动力学、运动耐量并延长生存。随着这些药物在儿科的逐步应用，儿童PAH的治疗取得了明显进步，但仍需开展进一步的大样本随机对照试验，同时还必须努力研发新型药物。

Abstract:

This paper provides an overview of the current state of pharmacotherapy in children with pulmonary arterial hypertension (PAH) and a brief introduction to the potentially novel pharmacologic targets for PAH. Currently, 3 classes of drugs including prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors are approved for the treatment of PAH in children, which has led to improved hemodynamics, increased exercise capacity and prolonged survival. Despite these improvements, there is still a need to carry out well-designed, randomized, controlled studies with larger samples. In addition, novel drugs targeting other molecular pathways should be developed.