Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Eur Respir Rev. 2017 Nov 15;26(146). doi: 10.1183/16000617.0092-2017. Print 2017 Dec 31.
Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from impaired right ventricular filling and/or reduced right ventricular flow output. This clinical picture results from an imbalance between the afterload imposed on the right ventricle and its adaptation capacity. Acute decompensated pulmonary hypertension is associated with a very poor prognosis in the short term. Despite its major impact on survival, its optimal management remains very challenging for specialised centres, without specific recommendations. Identification of trigger factors, optimisation of fluid volume and pharmacological support to improve right ventricular function and perfusion pressure are the main therapeutic areas to consider in order to improve clinical condition. At the same time, specific management of pulmonary hypertension according to the aetiology is mandatory to reduce right ventricular afterload. Over the past decade, the development of extracorporeal life support in refractory right heart failure combined with urgent transplantation has probably contributed to a significant improvement in survival for selected patients. However, there remains a considerable need for further research in this field.
慢性毛细血管前肺动脉高压导致的急性右心衰竭的特点是全身淤血的综合征迅速进展，这是由于右心室充盈受损和/或右心室流出量减少所致。这种临床表现源于右心室所承受的后负荷与其适应能力之间的失衡。急性失代偿性肺动脉高压与短期预后极差相关。尽管它对生存率有重大影响，但对于专门中心来说，其最佳管理仍然极具挑战性，而没有具体的建议。确定触发因素、优化血容量和使用药物支持来改善右心室功能和灌注压是改善临床状况的主要治疗领域。同时，根据病因进行特定的肺动脉高压管理对于降低右心室后负荷是必需的。在过去十年中，体外生命支持在难治性右心衰竭患者中的应用结合紧急移植可能对某些患者的生存率的显著提高做出了贡献。然而，该领域仍有大量的研究需求。
