Wacker Julie, Beghetti Maurice
Pediatric Cardiology Unit, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, Rue Willy Donzé 6, 1211 Genève 14, Geneva, Switzerland.
Pulmonary Hypertension Program, Geneva University Hospitals, Geneva, Switzerland.
Eur J Pediatr. 2025 Apr 9;184(5):288. doi: 10.1007/s00431-025-06099-4.
Pediatric pulmonary hypertension differs from adult pulmonary hypertension in many ways, including multifactorial etiologies and comorbidities that can impact diagnosis, response to therapy, and outcome. The main etiologies of pediatric PH are idiopathic pulmonary arterial hypertension (PAH), PAH associated with congenital heart disease (PAH-CDH) and developmental lung disorders. Thorough diagnostic evaluation is necessary to properly classify pulmonary hypertension, find a potential treatable cause, and guide therapy. Diagnosis still relies on invasive hemodynamics that require sedation in most children. Management of pediatric pulmonary hypertension is mainly guided by small-scale studies, expert opinion, and extrapolation of adult data considering the paucity of trials in this population. The aim of this review is to provide an up-to-date summary of current knowledge on pediatric pulmonary hypertension, covering diagnosis to management, and to highlight the key takeaways from the pediatric task force of the 7th World Symposium on Pulmonary Hypertension, particularly regarding classification modifications, risk stratification, and management. What is known: • Pediatric pulmonary hypertension is a rare condition, with the main etiologies being idiopathic, associated with congenital heart disease and developmental lung disorders. • A risk-oriented treatment approach is recommended, with lower-risk mortality as the therapeutic target. Treatment should be escalated if the treatment response is unsatisfactory. What is new: • Classification of pulmonary arterial hypertension associated with congenital heart disease is expanded beyond the concept of a shunt. • Risk stratification is refined through the use of 25 validated risk factors.
小儿肺动脉高压在许多方面与成人肺动脉高压不同，包括多因素病因和合并症，这些都会影响诊断、治疗反应和预后。小儿肺动脉高压的主要病因是特发性肺动脉高压（PAH）、与先天性心脏病相关的PAH（PAH-CDH）以及发育性肺部疾病。进行全面的诊断评估对于正确分类肺动脉高压、找到潜在的可治疗病因以及指导治疗是必要的。诊断仍然依赖于侵入性血流动力学检查，而这在大多数儿童中都需要镇静。考虑到该人群中试验的匮乏，小儿肺动脉高压的管理主要以小规模研究、专家意见以及对成人数据的推断为指导。本综述的目的是提供关于小儿肺动脉高压当前知识的最新总结，涵盖从诊断到管理的各个方面，并突出第七届世界肺动脉高压研讨会小儿工作组的关键要点，特别是关于分类修改、风险分层和管理方面。已知内容：• 小儿肺动脉高压是一种罕见疾病，主要病因是特发性、与先天性心脏病相关以及发育性肺部疾病。• 推荐采用以风险为导向的治疗方法，以降低风险死亡率作为治疗目标。如果治疗反应不令人满意，应加强治疗。新内容：• 与先天性心脏病相关的肺动脉高压分类已扩展到分流概念之外。• 通过使用25个经过验证的风险因素对风险分层进行了细化。
