Acute decompensated pulmonary arterial hypertension (PAH) is characterised by rapid worsening of clinical signs of right heart failure (RHF) with subsequent congestion and systemic circulatory insufficiency that can lead to multisystem organ failure [1–3]. Short-term outcomes of acute decompensated RHF are very poor and it remains the primary cause of mortality in PAH [4, 5]. Intensive care of acute decompensated PAH is based on treatment of triggering factors, careful fluid management, and strategies to improve cardiac function and reduce right ventricular afterload [1]. However, this medical strategy is not always sufficient to restore a long-lasting balance between the afterload imposed on the right ventricle and its capacity for compensation. In case of refractory RHF despite maximal medical treatment, the use of mechanical support should now be considered in selected candidates for lung transplantation, or less commonly as a bridge to recovery in patients with a treatable cause of right-sided heart failure [1]. Veno-arterial extracorporeal membrane oxygenation (ECMO) is currently the most widely used strategy to support the right ventricle in PAH patients. This strategy, combined with changes in organ allocation rules to prioritise patients with a short-term life-threatening condition, should contribute to the improved survival of eligible patients with end-stage PAH [6]. However, long-term survival of patients admitted to the intensive care unit (ICU) for severe acute RHF management has not been studied extensively in the modern management era of mechanical support and high-priority lung transplantation.

急性失代偿性肺动脉高压 (PAH) 的特征是右心衰竭 (RHF) 的临床症状迅速恶化，随后出现充血和全身循环功能不全，可导致多系统器官衰竭[1-3]。急性失代偿性 RHF 的短期结果非常差，它仍然是 PAH 死亡的主要原因 [4, 5]。急性失代偿期 PAH 的重症监护基于触发因素的治疗、谨慎的液体管理以及改善心功能和降低右心室后负荷的策略 [1]。然而，这种医疗策略并不总是足以恢复强加于右心室的后负荷与其补偿能力之间的长期平衡。在经过最大限度的药物治疗后仍出现难治性 RHF 的情况下，现在应该考虑在选定的肺移植候选者中使用机械支持，或者不太常见地作为右侧心力衰竭可治疗原因患者康复的桥梁[1]。静脉-动脉体外膜肺氧合 (ECMO) 是目前最广泛使用的支持 PAH 患者右心室的策略。该策略与器官分配规则的变化相结合，以优先考虑短期危及生命的患者，应该有助于提高符合条件的终末期 PAH 患者的生存率 [6]。然而，在机械支持和高优先级肺移植的现代管理时代，重症监护病房 (ICU) 接受重症急性 RHF 管理的患者的长期生存率尚未得到广泛研究。