Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
National Heart and Lung Institute, Imperial College London, London, UK.
ESC Heart Fail. 2021 Jun;8(3):1901-1914. doi: 10.1002/ehf2.13263. Epub 2021 Mar 3.
Pulmonary arterial hypertension (PAH) is common amongst patients with congenital heart disease (CHD). It is a severe and complex condition that adversely affects quality of life and prognosis. While quality of life questionnaires are routinely used in clinical pulmonary hypertension practice, little is known on how to interpret their results and manage PAH-CHD patients with evidence of impaired health-related quality of life, especially those with advanced disease and palliative care needs.
We performed a systematic review of studies concerning palliative care for people with PAH-CHD, also reviewing the health-related quality of life literature pertaining to these patients. Of 330 papers identified through initial screening, 17 were selected for inclusion. Underutilization of advance care planning and palliative care resources was common. Where palliative care input was sought, this was frequently late in the course of the disease. No studies provided evidence-based clinical criteria for triggering referral to palliative care, a framework for providing tailored care in this patient group, or how to manage the risk of sudden cardiac death and implantable cardioverter defibrillators in advanced PAH-CHD. We synthesize this information into eight important areas, including the impact of PAH-CHD on quality of life, barriers to and benefits of palliative care involvement, advance care planning discussions, and end-of-life care issues in this complex patient group, and provide expert consensus on best practice in this field.
This paper presents the results of a systematic review and expert statements on the preferred palliative care strategy for patients with PAH-CHD.
肺动脉高压（PAH）在先天性心脏病（CHD）患者中很常见。这是一种严重且复杂的疾病，会对生活质量和预后产生不利影响。虽然生活质量问卷在临床肺动脉高压实践中经常使用，但对于如何解释其结果以及管理存在健康相关生活质量受损的 PAH-CHD 患者，尤其是那些患有晚期疾病和姑息治疗需求的患者，知之甚少。
我们对有关 PAH-CHD 姑息治疗的研究进行了系统评价，同时还回顾了与这些患者相关的健康相关生活质量文献。通过初步筛选确定了 330 篇论文，其中有 17 篇被选中纳入。姑息治疗资源的利用不足在 Advance care planning 中很常见。在寻求姑息治疗的地方，通常是在疾病的晚期。没有研究提供基于证据的临床标准来触发姑息治疗的转介、为该患者群体提供量身定制的护理的框架，或者如何管理晚期 PAH-CHD 中的心脏性猝死和植入式心脏复律除颤器的风险。我们将这些信息综合成八个重要领域，包括 PAH-CHD 对生活质量的影响、姑息治疗参与的障碍和益处、 Advance care planning 讨论，以及该复杂患者群体的临终关怀问题，并提供该领域最佳实践的专家共识。
本文介绍了一项系统评价的结果和专家对 PAH-CHD 患者首选姑息治疗策略的陈述。
