《中国肺动脉高压诊断与治疗指南(2021版)》解读——聚焦先天性心脏病相关肺动脉高压

An overview of pulmonary arterial hypertension associated with congenital heart disease

文章是对《中国肺动脉高压诊断与治疗指南(2021版)》中先天性心脏病相关肺动脉高压(CHD-PAH)部分的解读,旨在推动CHD-PAH的规范诊疗,以改善患者预后。我国CHD-PAH患者基数大,疾病谱复杂。针对合并肺动脉高压的先天性心脏病,需要更加规范的手术前评估和手术适应证的判断。加强术后PAH患者随访管理,进行规范化治疗。艾森曼格综合征患者经过有效的药物治疗,也可改善生活质量,避免恶化,但对终末期肺血管疾病患者,心肺移植是最后的治疗手段。然而,目前还缺乏专门针对艾森曼格综合征患者的危险度分层和治疗管理策略。

Based on the 2021 guideline for the diagnosis and treatment of pulmonary hypertension in China,this interpretation aims to promote the standardized clinical practice forpatients with pulmonary arterial hypertension(PAH)associated with congenital heart disease(CHD)in order to improve prognosis.PAH associated with CHD is the most common type of PAH in China,representing a large and heterogeneous patient population.Patients with PAH due to nonrestrictive cardiacshunt should receive comprehensive evaluation and careful assessment of the risk for closingdefect because of the higher mortality in patients with PAH after defect correction.If the latter happens,receiving close follow-up and advanced PAH therapies are paramount.Advanced therapies also benefit patients with Eisenmenger syndrome.For endstage pulmonary vascular disease,the lung transplantation is the last resort.However,there are no risk assessment and treatment algorithm specifically for patients with Eisenmenger syndrome.