Background: Pulmonary hypertension in children is progressive with wide variability in prognosis. 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 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, together 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 on the basis of 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.

背景：儿童肺动脉高压是进行性的，预后差异很大。本文提供了一个循证的临床实践指南，以管理儿童进行性肺动脉高压，尽管最佳的治疗。方法：一个多学科小组确定了有关肺动脉高压患儿的管理的相关问题，尽管有最佳治疗，但仍有进展，对相关文献进行了系统回顾，并应用建议分级、评估、发展和评估方法来制定临床建议。结果：在审查了研究证据后，专家组在每个提出的问题中考虑了干预措施的可取（利益）和不可取（危害和负担）影响的平衡。我们还考虑了对主要结果的评估，以及所需资源、公平性、可接受性和可行性。针对经最佳治疗仍有进展的肺动脉高压患儿，提出了支持或反对介入策略的建议。结论：尽管患有肺动脉高压的儿童人数在不断增加，但对于那些尽管进行了最佳药物治疗，但病情仍在恶化的儿童，其治疗方法却明显缺乏经验证据。该小组在有限的经验证据的基础上，结合专家意见，制定并提供了支持或反对介入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，我们确定了未来研究的重要领域。