BACKGROUND Pulmonary hypertension in children often progresses despite optimal therapy. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy. METHODS A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach to develop clinical recommendations. RESULTS After reviewing the research evidence, the panel considered the balance of desirable (benefits) and undesirable (harms and burdens) effects of the interventions in each proposed question. Valuation of our main outcomes was also considered, along with resources required, equity, acceptability, and feasibility. Recommendations were developed for or against interventional strategies specific to children with pulmonary hypertension that has progressed despite optimal therapy. CONCLUSIONS Although there is a growing population of children with pulmonary hypertension, there is a striking lack of empirical evidence regarding management of those whose disease has progressed despite optimal pharmacotherapy. The panel formulated and provided the rationale for clinical recommendations for or against interventional strategies based on this limited empirical evidence, coupled with expert opinion, to aid clinicians in the management of these complex pediatric patients. In addition, we identified important areas for future research.

中文翻译：

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尽管接受了最佳治疗，但患有进行性肺动脉高压的儿童的介入策略。美国胸科学会官方临床实践指南。


背景 尽管进行了最佳治疗，但儿童肺动脉高压经常进展。本文件为尽管接受了最佳治疗但仍进行性肺动脉高压儿童的管理提供了循证临床实践指南。方法 一个多学科小组确定了有关尽管接受了最佳治疗但仍有进展的肺动脉高压患儿管理的相关问题，对相关文献进行了系统评价，并应用建议分级、评估、开发和评价 （GRADE） 方法来制定临床建议。结果 在审查了研究证据后，小组在每个拟议问题中考虑了干预措施的理想（收益）和不良（伤害和负担）效果的平衡。还考虑了对我们主要结果的评估，以及所需的资源、公平性、可接受性和可行性。针对尽管接受了最佳治疗但仍有进展的肺动脉高压患儿，制定了支持或反对干预策略的建议。结论 尽管肺动脉高压儿童的人数不断增加，但关于尽管最佳药物治疗后疾病进展的患者的管理，却明显缺乏经验证据。该小组根据这些有限的实证证据，结合专家意见，制定并提供了支持或反对干预策略的临床建议的基本原理，以帮助临床医生管理这些复杂的儿科患者。此外，我们还确定了未来研究的重要领域。