Farhat Nesrine, Lador Frederic, Beghetti Maurice
a Pediatric Cardiology Unit , University Children's Hospital of Geneva , Geneva , Switzerland.
b Pulmonary Hypertension Program , University Hospital of Geneva , Geneva , Switzerland.
Expert Rev Cardiovasc Ther. 2019 Mar;17(3):161-175. doi: 10.1080/14779072.2019.1576523. Epub 2019 Feb 12.
Pediatric pulmonary arterial hypertension (PAH) remains a rare and severe disease with a poor prognosis. PAH may be idiopathic, heritable or associated with systemic conditions in particular associated with congenital heart disease. Areas covered: A thorough and extensive diagnostic approach is required for a correct diagnosis. The outcome has improved over the last decade with a better diagnostic approach and with the initiation of new targeted therapies. However, there is still significant progress to achieve as there is still no cure for this devastating disease. Expert opinion: Adapted clinical studies to define the best therapeutic approach are needed. Even if the treatment approach is still mainly derived from adult data and expert consensus, several studies and registries are currently underway and should deliver important information in the next future. This review aims to give an overview of the current diagnosis and treatment strategies of PAH.
小儿肺动脉高压（PAH）仍然是一种罕见且严重的疾病，预后较差。PAH可能是特发性的、遗传性的或与全身性疾病相关，特别是与先天性心脏病相关。涵盖领域：正确诊断需要全面且广泛的诊断方法。在过去十年中，随着诊断方法的改进和新靶向治疗的开展，治疗结果有所改善。然而，由于这种毁灭性疾病仍然无法治愈，仍有显著的进展需要取得。专家意见：需要开展适应性临床研究以确定最佳治疗方法。即使治疗方法仍主要源自成人数据和专家共识，但目前正在进行多项研究和登记，未来应能提供重要信息。本综述旨在概述PAH的当前诊断和治疗策略。
